24 International Congress on Pediatrics

24th International Congress on Pediatrics &

10th Congress on Pediatrics Nursing

Oct 11- 15, 2012

Children’s Medical Center

Tehran- Iran

Organizer: Department of Pediatrics, Tehran University of Medical Sciences,

Tehran, IR Iran Growth & Development Research Center, Tehran University of

Medical Sciences, Tehran, IR Iran Children’s Medical Center, Pediatrics Center of Excellence, Tehran,

IR Iran

President Dr. Ali Rabbani

Tehran University of Medical Sciences

Growth & Development Research Center Tehran University of Medical Sciences

Department of Pediatrics Tehran University of Medical Sciences Pediatrics Center of Excellence,

Children’s Medical Center

Scientific Director: Gholam-Reza Walizadeh, MD Executive Director: Nima Rezaei, MD, PhD Coordinator of Scientific Program: Mahmoud Reza Ashrafi, MD

In colaboration with:

• TUMS International Relations Office • TUMS Public Relations Office • Iranian Pediatric Endocrinology Association • Iranian Pediatric Neurology Association • Students' Scientific Research Center, Tehran University of Medical Sciences

Organization Committee:

• Ashrafi, Mahmoud-Reza; MD

• Haghi- Ashtini, Mohammad Taghi; MD

• Pasalar, Parvin, PhD

• Rezaei, Nima, MD, PhD

• Shajari, Hamideh; MD

• Setodeh, Aria

• Shakiba, Marjan

• Tootoonchi, Parichehr, MD

• Ziaee, Vahid; MD

Executive Committee:

1- Ekrami, Sh 2- Rabiei, F. 3- Rahimi, S. 4- Rostami, Y 5- Seifkhani, A. 6- Karimi, R.; MD

7- Karimi, H. 8- Kianmehr, Sh. 9- Mirzaei, B. 10- Nekorazm, A 11- Yaghmaei, B; MD

1

Screening of children for the Marfan syndrome

Keyhan Sayadpour Zanjani, Babak Mahshidfar Tehran University of Medical Sciences Background: The Marfan syndrome is a multisystemic disease involving cardiac valves, aorta, eyes, musculoskletal system, etc. The incidence is one in 5-10 thousand people which is not low. Cardiovascular involvement can predict mortality as complications like aortic aneurysm and dissection are potentially fetal. Drug therapy (betha blockers, angiotensin-converting enzyme inhibitors, etc.) can prevent fatal complications but it should be started in early life. Therefore, the importance of early diagnosis of this syndrome can not be ignored. Methods: We incorporated screening of the Marfan syndrome in the general screening of school children by the help of the Health, Treatment and Medical Education Ministry. Suspected children are referred to a pediatric cardiologist for cardiac and skleteal examinations and if the diagnosis is likely, to an ophthalmologist for furthur examinations. If the diagnosis of Marfan syndrome is established, proper treatment and prevention of complications are issued. Our objective of introducing this project is attracting the attention of pediatricians. They can detect many patients and increase the efficacy of this project. We will discuss the features of this syndrome in children and the way pediatricians can help us in this project. Keywords: Marfan Syndrome, aortic Disease, screening

Heart failure among infants and children: Etiology and diagnosis

Bahram Mohebbi Tehran University of Medical Sciences The presentation of pediatric heart failure is diverse because of the numerous underlying cardiac etiologies and varying clinical settings. Heart failure is a clinical condition that results from impairment of the ventricle to fill with or eject blood. Heart failure is caused by ventricular pump dysfunction, or by overload of volume (preload) or pressure (afterload). Heart failure has been categorized either to describe its natural history after exposure to a risk factor for heart failure or to describe the severity of symptoms on a given day. Symptoms and physical findings in children with heart failure reflect the patient’s inability to adequately increase cardiac output (eg, exercise intolerance and easy fatigue) and/or pulmonary or systemic fluid overload (eg, shortness of breath at rest or with effort due to pulmonary interstitial edema or hepatomegaly). History: Symptoms of heart failure vary with the age of the patient as follows: • Infants: The most common symptoms are tachypnea and diaphoresis during feedings, easy fatigability, irritability, decreased volume of feedings, and poor weight gain. • Young children: In young children, the symptoms may be mistaken for common childhood illnesses such as gastroenteritis, reflux, asthma or even behavioral issues.

They include gastrointestinal symptoms (abdominal pain, nausea, vomiting, and poor appetite), failure to thrive, easy fatigability, and recurrent or chronic cough with wheezing. • Older children: Older children may present with exercise intolerance, anorexia, wheezing, dyspnea, edema, palpitations, chest pain, or syncope. Physical examination: Physical findings vary depending on the reduction of cardiac output, and degree of volume overload with signs of pulmonary and/or systemic venous congestion. Findings are suggestive of the underlying etiology for heart failure as demonstrated by the following examples: High blood pressure limited to upper extremities and/or feeble pulses in lower extremities are suggestive of aortic coarctation. The presence of a systolic murmur may be seen in patients with outflow obstruction in hypertropic cardiomyopathy or aortic stenosis, congenital heart defects with left to right shunting (eg, ventricular septal defects), or mitral regurgitation. Precordial examination may reveal a “thrill” in patients with shunt lesions, whereas those with a long-standing cardiomyopathy may have a “heave” with a laterally displaced point of maximal impulse. The diagnosis of heart failure in children is based on a combination of characteristic signs and physical findings of impaired cardiac output, respiratory distress, and poor growth .Laboratory studies and imaging studies generally are used to confirm the diagnosis of heart failure when there is clinical uncertainty, assess the severity of failure, and determine the underlying cause. In a child with congenital heart disease, an echocardiogram is useful to elucidate the following: cardiac anatomy, arterial and venous connections, presence and amount of shunting, presence and amount of valvular stenosis and regurgitation, atrial and ventricular sizes, diastolic function, estimation of right ventricular and pulmonary artery pressures. Keywords: heart failure, child, diagnosis

Contrast induced nephropathy (CIN) after administration of IODIXANOL

(VISIPAQUE) during cardiac catheterization in children with congenital heart disease

Saeed Abtahi, Elham Mohamadi rana Islamic azad university, Mashhad branch Background: Contrast induced nephropathy ( CIN ) is a common complication of percutaneous coronary intervention (PCI). The aim of this study was determination of the incidence of CIN after administration of Iodixanol during cardiac catheterization in children with congenital heart disease. Methods: All children age < 18 years that referred for cardiac catheterization were enrolled in this study. History and physical examination were taken before catheterization and 5 ml of serum sample was drawn for measuring of serum urea and creatinine. A total volume of iodixanol ( concentration 320mg/ml ) was 4-10 ml/kg . Serum sample for urea and creatinine was taken again 72 hours after catheterization, rising more than 25% compared to baseline serum creatinine was considered as CIN. Findings: 58 children enrolled at the study, 28 boys( 44.8%) and 32 girls( 55.2% ) with mean age of 64+/- 53.5 months. CIN ( increased of serum creatinine > 25% of

Cardiology & Pulmonology

2 Proceeding of the 24th International Congress of Pediatrics – Oct 2012Cardiology & Pulmonology

baseline ) was occurred in 17.2% of patients( 13.8% in cyanotic and 3.4% in acyanotic patients). Risk of CIN was not changed statistically related with age, sex, weight and dose of contrast media. Conclusion: Iodixanol seems to be safe in cardiac catheterization of children. Although the risk of CIN was not statistically related with age, sex, weight and dose of visipaque, there was significant relationship between risk of CIN in cyanotic congenital heart disease. Keywords: children catheterization Iodixanol, CIN

Evaluation of left ventricular function by

Doppler echocardiography and N- terminal Pro B-type Natriuretic peptide in children

with chronic kidney Roya Isa Tafreshi1, Nakysa Hooman2, Hasan Otukesh2, Azar Nikavar, Ebrahim Khamse2, Sudabe Hoseini 1Department of Pediatric Cardiology, 2Department of Pediatric Nephrology. 1, 2 Aliasgar Children’s Hospital, Tehran University of Medical Sciences and Health Services Background: Cardiovascular abnormalities have been considered as the major risk factor limiting the life expectancy of children with chronic kidney disease (CKD). Evaluation of N-terminal pro B-type natriuretic peptide (BNP) is a sensitive method for detecting left ventricular dysfunction (LVD). In patients with CKD, its use is confounded with concomitant volume overload and reduced renal excretion. Purpose: To assess early LVD with echocardiographic parameters and BNP level in children with CKD stages 2-4. Methods: A total of 105 patients (mean age 9.8 ± 4.4 years) and 100 aged matched control subjects were included in this study. Pulsed Doppler derived myocardial performance index (PWD-MPI) as a noninvasive index for assessing combined LV function was calculated. Systolic (sm) and early diastolic (Em) velocities of mitral annulus were measured by using tissue Doppler imaging (TDI) and E/Em ratio was calculated. Findings: The mean of GFR was 24±16 cc/min/1.73m2. The mean values of both PWD-MPI and TDI values of the patients were significantly different from those of the control subjects [PWD-MPI: 0.45±0.13 vs. 0.32 ±0.03; (p<0.003); Em: 10.8± 2.8 vs. 2 ± 12 (p<0.001); Sm: 6.8±1.9 vs. 7.2 ±1 (p<0.001), respectively]. High LV filling pressures (E/Em>10) were detected in a total of 28(26.7%) patients. The mean value of BNP (2239 ±720 pg/ml) was increased above reference range levels. Its level was correlated with severity of the renal impairment(r =-0.29). BNP was significantly correlated with high E/Em ratio (r=0.36). There was a negative correlation between BNP levels and LV Sm velocity as a marker of ventricular longitudinal systolic function(r= -0.47). Conclusions: Non-invasive diagnosis of LVD can be performed by measurement of PWD- MPI. Elevated level of BNP is related to LV dysfunction. BNP is a useful adjunct in early diagnosis of LVD in patients with CKD. Keywords: chronic kidney disease, ventricular dysfunction, natriuretic peptide, pediatrics

Evaluation of cardiovascular malformation in

diabetic mothers' newborns in Imam khomeini Hospital 1389-1387

Parvin Akbari-Asbagh, Milad Taghavi Department of Pediatrics, Valasr Hopital, Tehran University of Medical Sciences and Health Services Background: Many studies have been performed regarding the relationship between maternal diabetes and malformations in fetuses. It seems that cardiovascular malformations are the most common anomalies in infants of diabetic mothers. Concerning the high prevalence of diabetes mellitus in Iran further studies in this field seem to be necessary. The objective of this study was to focus on the relationship between different types of diabetes mellitus in pregnant women and various cardiovascular malformations occurring in their infants. Other aspects such as the effects of diabetes duration and control methods are also studied in this research. Methods: This is a retrospective cohort study in which 70 neonates admitted to Valiasr hospital between the years 1387 to 1389 are chosen and devided into two groups: one group consists of infants of diabetic mothers and the other group consists of infants of non-diabetic mothers. After collecting the data regarding each infant and his/her mother from their charts, statistical analysis was performed in order to compare the data from each group. Different variables such as gender, birth weight, gestational age, apgar score by the time of birth, clinical presentations, time and report of the echocardiography if it were performed and also maternal variables such as age, past medical and obstetric history and informations regarding diabetes of the mother were concerned in this study. Findings: According to this study there seems to be a higher prevalence of cardiovascular malformations in infants of diabetic mothers than those of nondiabetic mothers (p= 0.018). The most common anomalies seem to be PDA, hypertrophic cardiomyopathy and PFO. It is less probable that the type of maternal diabetes, its duration and control method could cause a great difference in the prevalence of CVMs. Conclusion: According to this study maternal diabetes has a major role in occurring some types of cardiovascular malformations in infants such as PDA, HCMP and PFO. Thus diagnostic prenatal and neonatal tests in infants and prenatal care in diabetic mothers seem to be necessary. Keywords: cardiovascular malformation, diabetic mother, newborn

Children interstitial lung disease:

presentation of the new case

SH Afsharpaiman Health Research Center, Baqiyatallah University of Medical Sciences Background: Classifications of interstitial (diffuse) lung disease in adults and children have undergone significant revision in recent years, with advances in our understanding of new entities and the biology and prognostic significance of certain histological patterns. ILD has been estimated to be 3.6 per 1 000 000 in a study in the United Kingdom and Ireland, and the incidence to be 1.32 per 1 000 000 in a recent study from Germany. Affected infants and children present with signs and symptoms of abnormal breathing, including tachypnea, crackles on examination, and diffuse infiltrates on chest radiography. Diagnosis is made by the summation of clinical, radiologic, and pathological findings, with some disorders having

3Proceeding of the 24th International Congress of Pediatrics – Oct 2012

Cardiology & Pulmonology

characteristic clinical, pulmonary function, or imaging findings lessening the need for biopsy. Treatment and prognosis are disease-specific, and can involve a combination of supportive and pharmacological care. Case presentation: we describe an 11-year-old boy who’s presented with a two-month history of dyspnea on exertion. The only positive history was near drowning in pool when he was 5. The physical examination was unremarkable. A chest radiography and HRCT revealed diffuse dense, bilateral, reticulonodular opacity. Lung biopsy shows severe inflammation, fibrosis and hemorrhage. The patient was started on prednisolone and discharged from hospital with better PFT.

Conclusion: Children’s interstitial lung diseases are rare diffuse lung diseases resulting from a variety of pathogenic processes that include genetic factors, association with systemic disease processes, and inflammatory or fibrosis responses to stimuli. Although some disease types overlap with those seen in adults, there are many causes unique to children, particularly those that occur in infancy. Diagnosis is made by the summation of clinical, radiologic, and pathologic findings, with some disorders having characteristic clinical, pulmonary function, or imaging findings, lessening the need for biopsy. Treatment and prognosis are disease-specific and can involve a combination of supportive and pharmacologic care. Keywords: children, interstitial lung disease, lung fibrosis

4

Effect of treatment in obese and non obese children with vitamin D deficiency

Fateme Saffari, Parisa Shahroodi Qazvin University of Medical Sciences Background: According to variation in vit-D metabolism in obese children and also increasing rate of obesity in recent years we decided to compare the response to vit-D therapy between obese (BMI >95th percentile for age) and non-obese (BMI = 5th–75th percentile for age) children. Methods: 133 non-obese and 69 obese children suffering from vit-D insufficiency and vit-D deficiency were treated with a single dose of 300000 unit vit-D intra-mascularly. We measured TSH, T4, ALP, P, Ca, PTH and 25-OH-vitD before treatment and 25-OH-vitD one month after and then they were compared for response to this treatment. Vitamin D deficiency was defined as serum 25(OH)D <20 ng/ml and insufficiency as 21–29 ng/ml respectively. Findings: Mean level of vit-D before treatment was 13.5 in obese and 14.5 in non-obese children and they were not significantly different. After treatment, mean level of vit-D became 29.6 in obese and 33 in non-obese children that was statistically different. Conclusion: Therapeutic response in obese children is less than non-obese children and it it seems that it is necessary that the obese children to be treated with higher doses or for longer term. Keywords: Children, obesity, vitamin D deficiency

Partial trisomy 7q and monosomy 13q in a Child with disorder of sex development:

phenotypic and genotypic findings Azadeh Shojaei, Farkhondeh Behjati, Pupak DerakhshandehPeykar, Maryam Razzaghy-Azar, Hasan Otukesh, Roxana Kariminejad, Mohammad-Ali Dovlati, Ali Rashidi-Nezhad, Javad Tavakkoly Bazzaz Tehran University of Medical Sciences Background: Terminal 7q duplication and terminal 13q deletion are two conditions with variable phenotypes including microcephaly, thumb a-/hypoplasia, cortical dysplasia, microphtalmia, intellectual disability and dysmorphic features. We describe a boy born to a mother with a reciprocal t(7;13) who combines both a terminal 7q33-qter duplication and terminal 13q33-qter deletion through the inheritance of a derivative chromosome 13 (der(13)). The patient presented with developmental delay, facial and non-facial dysmorphic features, hypertonia, genital abnormality and skeletal malformation but no thumb a-/hypoplasia or microphtalmia. Methods: Genomic DNA was extracted from peripheral blood. Cytogenetic investigation using GTG banding technique was performed on metaphase spreads of peripheral blood lymphocytes via standard procedures. Copy number variations in subtelomeric regions were studied using P036 SALSA MLPA kit. The Array-CGH was performed using the CytoChip ISCA 4x44K(v2.0) (BlueGnome, Cambridge, UK) microarray.

Findings: The patient’s karyotype showed an additional segment of 7q32-qter origin on the long arm of chromosome 13 at band 32 . His mother’s karyotype showed a reciprocal balanced translocation between long arms of chromosomes 7 and 13: 46,XX,t(7;13) (q32;q32) (figure 2B). Array CGH of the proband confirmed 22 Mb duplication of terminal end of 7q and pinpointed 7.7 Mb terminal deletion of 13q, consistent with transmission of maternal derivative chromosome 13 to her son. Final genotype of the proband is 46,XY, der(13)t (7;13) (q33;q33) mat.arr 7q33-q36.3(136570553-158291440) × 3mat,13q33.3-q34(106404835-114110721) × 1mat (figure 3). Conclusion: Knowing the exact breakpoints of his chromosomal aberrations using high resolution array CGH (aCGH) and comparison of his phenotypes with those of 24 and 59 previously published cases of 7q duplication and 13q deletion, respectively, allow us to further narrow the size of proposed critical regions for microcephaly, thumb a-/hypoplasia and hypo/hypertonia on chromosome 13. Keywords: array CGH, 7q duplication, 13q deletion, translocation, disorders of sex development

The investigation of 38 files from patients affected by Hyperammonemia

F Saeed Tehrani Sherkate Naft Hospital, Tehran

Methods: This investigation is a retrospectively reviewed of 38 files of medical records of patients affected with hyperammonemia who were treated at Metabolic Department of Children’s Medical Center Hospital in Tehran from year 2003-2009. Findings: The most important symptoms during admission included: 1. unconsciousness and somnolence in 20(%50) patients; 2. vomiting in 16(%40) patients; 3. convulsion in 14(%40) patients; 4. tachypnea and respiratory distress in 12(%30)patients; 5. recurrent apnea was present in 4(%10)patients who were intubated and connected to ventilator; 6.anorexia; 7.hypothermia; 8. Hyperventilation. Pharmacologic treatment: *31 Patients(%80) were treated only with pharmacologic drugs to decline blood ammonia level. 21 patients (%70) were treated with Sodium benzoate and 10 patients (%30) were treated with phenylbutyrate in combination with Sodium benzoate. Arginine 200mg/kg to 400mg/kg daily dose was administrated to 5 patients(%16). Citrulline 200 mg/kg daily dose was administrated to 1 patient(%3). Carnitine was administrated to all of the patients because Na benzoate and phenyl butyrate cause Carnitine depletion. Peritoneal dialysis: Peritoneal dialysis had been done in 7 patients (%20). These patients were very ill and had no response to pharmacologic drugs to decline blood ammonia level. Conclusion: 31 patients (%80) were treated only with pharmacologic drugs. Intravenous pharmacologic drugs with highest dose 500 mg/kg daily was more effective than low dose. In partial urea cycle enzyme deficiencies ammonia accumulation may be triggered by catabolic stress such as during an infection, illness, trauma, surgeries or after increasing of patient’s intake. In these patients an acute severe Hyperammonemia episode can occur after

Endocrinology and Metabolic Disorders


Endocrinology & Metabolic Disorders

newborn period to very late in life so we should always think to Hyperammonemia even after newborn period. Keywords: Hyperammonemia

Does congenital hypothyroidism have

different etiologies in Iran

Zohre Karamizadeh, Setillia Dalili1, Heidyeh Sanei-far, Hamdollah Karamifard, Hamid Mohammadi, Gholamhossein Amirhakimi Guilan University of Medical Sciences Background: To determine the prevalence of congenital hypothyroidism (CH), permanent and transient cH. Methods: From November 2006 to September 2007, 63031 newborns were screened by measuring serum TSH obtained by heel prick. The neonates who had a TSH≥5mU/L were recalled for measurement of serum T4, thyroid stimulating hormone (TSH) and TSH receptor blocking antibodies (TRBAb) in venous samples. In 43 primarily diagnosed as cases of CH, treatment was discontinued at age 2-3 years for 4 weeks and T4 and TSH were measured again. Permanent or transient was determined from the results of these tests and radiologic evaluation. Findings: The incidence of congenital hypothyroidism was found to be 1:1465 with a female to male ratio of 1.19: 1. The most common clinical findings were prolonged jaundice(73%), large anterior fontanel(56%) and wide posterior fontanel(55%). In 43 patients with CH, prevalence of permanent and transient form of the disorder was 53.6% and 46.4% respectively. Permanent cH was associated with higher initial TSH level than transient hypothyroidism (P<0.001). The most common etiology of permanent CH was dyshormonogenesis(57%). TRBAb was found in 6.8% of the total 43 cases. Conclusion: Congenital hypothyroidism in Iran may have different etiologies. Due to higher rate of transient CH than other similar researches, it is reasonable to follow these patients for a longer period to rule out the possibility of permanent hypothyroidism Keywords: Congenital Hypothyroidism, TSH Receptor, Dyshormonogenesis, Thyroid Dysgenesis

Congenital hypothyroidism: a review of the risk factors

S M Rezvany1, S Dalili1, A Dadashy1, A Medghalchi1, H Mohamadi2, H Dalili3, H Gholamnezhad1, M Mirzanejad1, A Amirhakimi2 1Guilan Health Center. 2Department of Pediatrics. 3Breastfeeding Research Center, Department of Pediatrics of Emam Khomeini Hospital. 1Guilan University of Medical Sciences, 2Shiraz University of Medical Sciences.3 Tehran University of Medical Sciences Background: Evaluation of the situation of Congenital Hypothyroidism(CH) in Guilan using the screening program and determine the correlation of congenital hypothyroidism with birth weight, gestational age and seasonality. Methods: During 2006 to 2010, in Guilan province, neonatal screening for CH by measurement of serum TSH level was performed in 3-5 days after birth. All neonates with TSH level ≥ 5mu/L were referred to endocrinologists

and serum TSH, T3 and T4 were measured. Based on public health data and profiles, total number of newborns, gestational age, route of delivery, birth season and birth weight in all screened neonates was reviewed and for ones with CH, their TSH measurements was also recorded. Findings: During 5 years, 119701 neonates were screened and CH was confirmed in 10.8% (221) of the referral cases (prevalence = 1:542). No significant statistical difference was seen between gender and birth season among patients with CH and the rest of the population. Interestingly, Low Birth Weight(LBW)(31% vs. 4.9%, P value< 0.01), postdate delivery(1.4% vs. 0.2%, P value< 0.01) and macrosomia were more prevalent in CH versus normal population (Odds ratio for post-date delivery was 6.9 and for LBW was 3.2). Rate of Normal Vaginal Delivery (NVD) was significantly higher in neonates with CH compared to normal population (39.2% vs. 29.2%, P value = 0.01). Keywords: LBW, postdate delivery, macrosomia, risk factors

The prevalence of iodine deficiency disorder

in two different populations in northern province of Iran; a comparison using

different indicators recommended by WHO

S Dalili1, S M Rezvani1, Z Mohtasham-Amiri2, A Dadashi1, S Hoseini1, H Gholami- Nezhad1, A Amirhakimi3 2Social & Preventive Medicine Department. 1,2 Guilan University of Medical Sciences. 3Shiraz University of Medical Sciences

Background: The objective of this study was comparison of the prevalence of Iodine Deficiency Disorder (IDD) in

neonates and school children using two different WHO indicators.

Methods: From 2006 to 2010, 119701 newborns were screened by measurement of serum TSH level by heel prick. Neonates who had blood TSH ≥ 5mIU/L were recalled for more evaluation. In the same period of time, urine iodine was measured in 1200 school-aged children. The severity of IDD was classified using WHO, UNICEF, ICCIDD criteria. Findings: Between 2006-2010 a total of 119701 neonates were screened in Guilan province and the total recall rate (neonates with TSH level ≥ 5mIU/L) was 1.8 %. The incidence rate of Congenital Hypothyroidism (CH) was 1/625. The median urine iodine level in school-aged children was 200-299 μg/L. Conclusion: Considering the WHO, UNICEF, ICCIDD criteria, Guilan province would be classified as a none-IDD endemic area. However, health care systems should pay attention to the iodine excess and the risk of iodine induced hyperthyroidism in this population.

Demographic evaluation o f type 1 diabetic patient referring to pediatric endocrine clinic

of Hamedan city Zahra Razavi, Leila Moradi Aramian Hamedan university of medical sciences Background: Type 1 diabetes mellitus is the most common endocrine disease in pediatrics. It has wide geographic

6 Proceeding of the 24th International Congress of Pediatrics – Oct 2012


variation in incidence and prevalence because of environmental factors interacting with genetically factors. Methods: This cross-sectional descriptive study has been considered in 150 patients with type 1 diabetes who were referred to pediatric endocrine clinic of Hamadan city. Different data such as age, sex, onset of incidence… were recorded in a questionnaire form and analyzed by spss software (v: 18). Findings: In this study the number of males and females were approximately equal (85 vs. 65). The peak incidence age was 8.47 years. The mean age of patients was 13.45 years and the mean of maternal age at delivery was 25.19 years. Most patients were living in urban area (81.3%). There were positive family history of T1DM in 16 patients of which 14 cases were present in siblings. 18 patients had thyroid dysfunction that 16 of them had hypothyroidism and a couple had hyperthyroidism. One of the patients was suffered from JRA. Patients BMI percentile at primary presentation of disease was also assayed. We had not any cases of T1DM with obesity. Conclusion: According to this study, T1DM prevalence was approximately equal in both genders and was most common in urban areas. Most of the patients were in age range of 10-14.9 years that were the same in both genders. Incidence of disease was lower in summer and higher in autumn. However, there was no significant difference between birth seasons. Keywords: Type 1 Diabetes Mellitus, pediatric, Hamedan

Pediatric metabolic bone diseases

Classification and an overview of clinical and radiological findings

M. Mearadji International Foundation for Pediatric Imaging Aid From practical point of view the metabolic bone disease should be categorized etiologically because of overlap of some endocrine bone diseases and skeletal dysplasia. The metabolic bone diseases affecting the bone formation and mineralization can be classified in four groups: 1: Disorders with insufficient mineralization organic matrix: This group includes different types of rickets following vitamin D abnormalities such as nutritional deficiency and other conditions (hepatic renal disease, vitamin dependent rickets type I and type II). Also mineral deficiency by prematurity, X-linked hypophosphatasia and oncogenic rickets are classified in this group.The radiological finding depends on severity and the type of rickets. In general the bones are demineralized. The bone physes reveal widening and irregularity, fraying and broadening of the metaphyses and long bone deformity following osteomalacia. Inherited hypophosphatasia is another entity characterized by deficient mineralization of cartilage and matrix (osteoid), however calcium and phosphate product is normal. The radiological finding depends on severities. The neonatal type the skull is non-mineralized with marked deficiency of extremities. On later age metaphyses show prominent lucent defects extending into the metaphyses from the growth plates. 2: Abnormalities of bone matrix formation.Osteogenesis imperfecta with decreased bone mineralisation due to insufficient matrix formation and quantative and qualitative defects in synthesis of type I collagen is found in this group. The radiological finding is heterogeneous and depends on severity in 5 different types and subtypes. In general osteoporosis, retarded calvarial

bone formation, rib and tubular bone fractures and collapsed vertebral bodies are characteristic findings with bowing deformities. Both Copper (Menkes disease) and now rarely observed vitamin C deficiency causing also osteoid deficiency. Clinically Menkes disease is characterized by kinky hair and neurodegenerative symptoms. The radiological changes are irregular distal metaphysic with metaphyseal fractures by minor trauma. The clinical symptoms of scurvy are muscle and joint pain, malaise and lethargy, spongy gums (bleeding) and capillary ruptures. Radiological findings of scurvy are diffuse demineralization, prominent zone of provisional calcification, metaphyseal fractures with spur formation and subperiostal hematomas. 3: Abnormalities of increased or decreased bone resorption: The major cause of increased bone resorption is hyperparathyroidism in children with renal failure. Radiolocigally they manifest themselves as renal osteodystrophy with subperiostal resorption along the phalanges, involving also the distal phalangeal tufts. Hyperphosphatasia is a rare autosomal recessive disease and also is a condition with increased bone turnover. It appears to be due to deficiency of osteoprotegen. Radiological signs are expanded and bowed diaphyses. There is marked demineralization with a heterogeneous pattern. Osteopetrosis is an example of decreased bone resorption with calcified cartilage. The several types of the disease are all caused by an abnormality of osteoclastic resorption usually due to heritable mutation. Depending on its type the radiological manifestation is different. Increased bone density, metaphyseal undermodeling and signs of fluctuating activity of sclerosering process and others are found. 4: Iatrogenic and toxic metabolic bone disorders. There are several iatrogenic and toxic disorders affecting the resorption of bone and calcified cartilage, including biphosphanate and prostaglandine or toxic effect by hypervitaminosis A and D and by heavy metal poisoning (lead). Also primary oxalosis should be classified in this category. Radiological changes in all these groups are different and characteristic. Increased bone density and opaque dense lines due to cyclic therapy will be found following biphosphanate treatment.Prostaglandins stimulate bone resorption with radiographic recognizable new bone formation.In chronic hypervitamin A hyperosteosis of the long bone and periosteal bone formation is the radiological finding.Hypervitaminosis D however shows an increase in width of provisional zone initially and cortical thickening later on. Lead lines due to excessive calcified cartilage is to be observed by lead poisoning.In oxalosis crystal deposit occurs in bone with formation of giant cell granulomatosis. Inflammatory response, osteosclerosis with greater cystic lesion will be developed. Based on above mentioned data the clinical and radiological findings of different metabolic diseases will be shown with special attention on characteristics of the radiological findings.

The diagnostic dilemmas of skeletal

dysplasia:classification, frequency and mode of inheritance of different type

(a clinical and radiological overview) M Mearadji International Foundation for Pediatric Imaging Aid There are more than 5000 known congenital malformation syndromes so far identified, about 400 involve skeleton, including 200 different type of skeletal dysplasia’s. Usually



skeletal dysplasia are a primary result of mutation genes that are expressed in chondro-osteus tissue, however there are also some other secondary dysplasia caused by abnormalities of extra-osteus factors with effects on the skeletal system as by metabolic errors as by hypophosphatemic rickets. The classification of skeletal dysplasia is principally based on radiological features of skeletal changes. Thanks to progress in molecular genetics especially in the last decade distinctions at the DNA level permit refinements in classification resulting in an increasing number of subtype of dysplasia. Some dysplasia with similar morphological findings with or without the same inheritance as well as their rare incidence are all reasons for diagnostic dilemma in this field. The first step in diagnosis of skeletal dysplasia is the responsibility of the pediatricians to observe a disproportionate skeletal development, unusual habitués with or without mental retardation as well as other clinical signs.The second step is more difficult and includes: Genetic counseling and interpretation of skeletal changes by radiological findings in affected patients and their families.The rare incidence of skeletal dysplasia on one hand and it’s diversities in morphology on other hand require some experience and therefore is recommended to be centralized. In this manner genetic clinicians and radiologists gain the ability and expertise in this field. Such a centralization exists in the Netherlands for many years as a study group (club) for skeletal dysplasia. As a member of this study group I would like to give an overview of lethal and non-lethal types of skeletal dysplasia and their genetic transmission.The importance of radiological approach and the specific findings of skeletal dysplasia will be demonstrated within scope of this meeting probably in 2 sessions. From each of the 22 categories classified by Lachman one or two cases will be shown including clinical findings, inheritance and characteristical radiological findings.

Determination of Arylsulfatase B activity on dried-blood filter paper samples in Iranian

MPS VI patients and controls

Sedigheh Shams1,2, Gabriel Civallero3, Roberto Giugliani3, Mohammad Taghi Haghi Ashtiani1, Leila Shafaghat6, Neda Rezaee6, Arya Setoodeh4, Ali Reza Tavasoli5 1Department of Pathology. 2Pediatric Urology Research Center. 3Medical Genetics Service, Clinical Hospital of Porto Alegre. 4 Department of Pediatrics Endocrinology. 5Department of Pediatric Neurology.1,2,4,5,6Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences. 3RS, Brazil

Background: Mucopolysaccharidosis VI (Maroteaux–Lamy syndrome, MPS VI) is an inherited lysosomal storage disease resulting from deficiency of the Nacetylgalactosamine 4-sulfatase (arylsulfatase B, ARSB). In the absence of this enzyme, glycosaminoglycans specially dermatan sulfate accumulate in the lysosomes of different organs. Laboratory diagnosis of MPS VI includes elevation of urinary glycosaminoglycan (uGAG), decreased activity of ARSB, and mutations in the ARSB gene. Recently treatment of MPS VI with enzyme replacement therapy became available, but early diagnosis of the disorder is critical for effectiveness of therapy. To further

validation of previous reported studies, here we present the evaluation of ARSB activity using dried blood on filter paper (DBFP) in MPS VI patients and healthy control groups. Methods: DBFP samples from 30 healthy individuals (1–30 years) and 6 cases of MPS VI (3-8 years) were used for the assay. Blood obtained after informed consent of the subjects or their parents. ARSB activity was determined by a fluorometric method after incubation of 3.0 mm disk of each DBFP for 20 hrs at 37 °C with appropriate dilution buffers and 4-methylumbelliferyl sulfate(4MU-sulfate) as substrates. Data reported as mean ± SD. Within-assay imprecision (CVs) was <10% and the between assay CVs was <15% respectively. Findings: The ARSB activities were 12.1 ± 5.2 µmol.l-1. h-1 ( min =7.4, max=29.2) in healthy individuals and 5.1 ± 1.0 µmol.l-1. h-1( min = 3.7, max= 6.4) in MPS VI patients. Our results showed a statistically significant difference of the enzyme activity between affected individuals and controls. Conclusion: Measurement of ARSB in DBFP can distinguish MPS VI patients from healthy controls easier, faster, and less expensive than assay in leukocyte or cultured fibroblasts. Transport of samples is easy, cheap, and suitable for newborn screening. Their incorporation in metabolic referent laboratories should be considered as a screening test, being important to confirm abnormal results with the routine leukocyte or fibroblast enzyme assays, or with molecular studies. More detail of assay and key points for correct diagnosis of ARSB deficiency will be discussed in the congress. Keywords: Mucopolysaccharidosis type VI, glycosaminoglycan, arylsulfatase B, dried blood filter paper samples

The effect of vitamin D on vitamin D level & calcium level in children & adolescence with

type one diabetes in Yazd provice Mahtab Ordooei, Seyed hadi Hoseini poor, Fatemeh Peyghambary, Nasrollah Bashar doost Shahid Sadoghi hospital, Yazd

Background: Vitamin D arrives to the body through cutaneous synthesis from ultraviolet rays and the food. Despite the sunshine in sunny countries, the prevalence of this vitamin deficiency in these areas is high. According to the prevalence of vitamin D deficiency, we decided to have a look on prescribing vitamin D effect on vitamin D level & calcium level in children and adolescents. Methods: 65 patients with type one diabetes who referred to Yazd Diabetes Clinic entered the study. For all patients a blood sample was obtained to check the level of vitamin D (25-OHD) & calcium and evaluated through the laboratory methods and the data was recorded in the check list. Then, the patients were given 50,000 IU of vitamin D3 every two weeks and the above experiment was repeated 3 months later and the results in patients were compared with their initial amount. The data were analyzed with Chi-square test and T-test and SPSS software Ver.17. Findings: The prevalence of patients with normal level of vitamin D (>30ng/dl), and deficiency of vitamin D (<30ng/dl) was 33.8% & 66.2% respectively. After administration of vitamin D, vitamin D levels significantly increased (p<0.001), a significant difference in calcium



level was not observed by increasing levels of vitamin D (P=0.887). Conclusion: The results of this study showed increasing vitamin D levels with no significant change in serum calcium levels after administration of vitamin D to children and adolescents with type one diabetes. This result can indicate the independent effect of calcium and vitamin D. Keywords: supplementation of vitamin D, vitamin D level, calcium level

A rare endocrine cause of hypertension in

children Farzaneh Abbasi, Bahar Ashjei, Hosein Shabani, Asieh Mosallanejad Growth and development research Center, Children Medical Center Hospital, Tehran University of Medical Sciences Background: Pheochromocytomas and paragangliomas are rare neoplasms in children. Tumors that arise from the adrenal medulla are termed pheochromocytomas, and those with extra-adrenal origins are called paragangliomas. Among hypertensive children, the incidence of surgically confirmed disease has ranged from 0.8 to 1.7 percent. Because of the uncommon nature of the disease, there have been few published reports of pheochromocytoma in children. Given the limited experiences with this disease in children and the similarity in clinical presentation, diagnosis, and treatment among children, the following report includes some data derived from a patient with pheochromocytoma. Case Presentation: A 13 year old boy, 8th child of the family referred to us with right upper quadrant abdominal pain, palpitation, pallor in morning, increased appetite, perspiration, headache and irritability for 1 month before the visit. In physical examamination, he had warm and wet skin, high pulse rate (HR=128/min) and high blood pressure (180/110mmHg), granulomatous lesions in sides of tongue, tenderness in deep palpation in right side of abdomen without organomegaly. A hypodense mass lesion with central necrosis in right adrenal was detected in abdominal CT scanning. In laboratory tests, positive finding was normetanephrine (urine) = 1219µg/24 hr (normal range <600). The pathologic finding approved pheochromocytoma. Conclusion: In children group, sustain hypertension can be a sign of pheochromocytoma after ruling out of other common causes of hypertension. Therefore, a pediatrician should be cautious about this rare disease. Keywords: hypertension, pheochromocytoma, normetanephrine

Results of congenital hypothyroidism screening program in the area under

coverage of Shahid Beheshti University of Medical sciences in 1390

F Rahimi Bairanvand, N Arjmand Kermani, Y Borna, A Golmohammadi Health Depuity, Shahid Beheshti University of Medical sciences Background: Neonatal screening programs (NSP) examined more than 150 million newborns untill 1999, and

about 42000 Congenital Hypothyroidism (CH) patients were diagnosed. Nowadays, almost all developed countries implement NSP for CH. Developing countries also implement these programs gradually. CH incidence is 1 in 3500-5000, 1 in 3000, 1 in 6600-7300 and 1 in 5700 live births in U.S., Europe, Sweden and Japan, respectively. Methods: All pregnant women or newly mothers living in the area under coverage of Shahid Beheshti Medical University were advised to take their 3-5 day's old newbornes to CH screening centers. A blood sample was taken from lateral parts of the newborn heel, using a Lancet and Guthrie paper. Thyroid Stimulating Hormone (TSH) level 5 to 9.9 mu/l led to repeated heel blood sample exam. If TSH level in first sample was ≥ 10 mu/l, or in repeated sample was ≥ 5 mu/l, a venous blood sample was taken for laboratory testing. Patients who had a venous TSH level ≥ 10 mu/l were considered as CH and were referred to a specialist for treatment. Treatment was begun urgently before referring, if venous TSH was ≥ 20 mu/l. Findings: Totally 123329 newborns were screened during one year. Heel blood sample TSH in 4385 (3.5%) and in 685 (0.5%) cases was 5-9.9 and ≥ 10 mu/l, respectively. 310 CH patients (158 boys and 152 girls) were diagnosed. The incidence rate was 1 in 398 screened newborns. Various towns reported different incidence rates ranging from 1 in 829 in eastern parts of Tehran (half of the university average) to 1 in 108 in Firoozkouh (about 4 times higher than university average). 134 patients (43.2%) were offspring of a Cesarian Section delivery and 27 cases (8.7%) were Low Birth Weight (LBW). Conclusion: The University overall incidence rate was similar to that of the whole country. There may be an association between incidence rate and mothers' nutrition, Iodine level in the area, delivery type and so on and further investigations and interventional programs are needed. Keywords: congenital hypothyroidism, neonatal screening program, Thyroid Stimulating Hormone

Is levothyroxine effective in improvement of migraine disability of girls with subclinical

hypothyroidism?

Mehrdad Mirouliaei, Razieh Fallah Department of Pediatrics, Shahid Sadoughi University of Medical Sciences Background: Migraine is the most frequent acute recurrent primary headache in childhood. Hypothyroidism may be an exacerbating factor for some primary headaches. The aim of this study was to evaluate effectiveness of levothyroxine in migraine disability of girls with subclinical hypothyroidism. Methods: In a quasi-experimental study, migraine disability of thirteen migraineurs girls with subclinical hypothyroidism whom were referred to pediatric neurology clinic of Shahid Sadoughi university of Medical Sciences,Yazd, Iran between January 2010 and February 2011 and treated with levothyroxine for two months, were evaluated by Pediatric Migraine Disability Assessment score (pedMIDAS). Findings: Mean age of patients was 9.3 ± 1.45 years. Headache migraine disability was significantly improved by treatment with a reduction of Pediatric Migraine Disability Assessment score from 42.1 ± 10.43 to 25.11 ± 8.3, P =0.03.



Conclusion: Levothyroxine was effective in reducing of migraine headaches disability in migraneurs girls with subclinical hypothyroidism. Keywords: headache, migraine, hypothyroidism, subclinical hypothyroidism

Maturity Onset Diabetes of the Young, Type

2 in an asymptomatic child Shahab Noorian, Fatemeh Sayarifard Elham Farhadi, Fabrizio Barbetti, Nima Rezaei Children Medical Center Hospital, Tehran University of Medical Sciences Background: Maturity onset diabetes of the young type 2 (MODY) is an inherited disorder, due to mutations in the glucokinase (GCK) gene, which lead to mild fasting hyperglycemia. Case Presentation: An healthy 8-year old boy with hyperglycemia is presented in whom the diagnosis of MODY2 is suspected. Genetic studies showed heterozygous inactivating GCK gene mutation in this patient. The same mutation was found in his father as well. The patient received some dietary advices without any medication. Conclusion: The identification of GCK mutation and diagnosis of MODY2 helps the clinicians to predict the disease course and patient prognosis and to exclude of other type of diabetes. Keywords: Maturity Onset Diabetes of the Young, GCK, fasting hyperglycemia, diabetes

Etiology of precocious puberty, a 10 year study in Endocrine Reserch Centre

(Firouzgar), Tehran Farzaneh Rohani Endocrine Research Centre (Firouzgar) Background: Precocious puberty as an early physical development and low final height might lead to psychosocial problems. The objective was to evaluate etiology and clinical features of precocious puberty in a cohort of Iranian children. Methods: In this case-series study, 44 girls and 8 boys with precocious puberty referred to Endocrine Reserch Centre (Firouzgar), Institute of Endocrinology and Metabolism (Hemmat Campus), were examined in a 10 year period of time. Findings: Mean age of girls and boys were 7.43±1.4 years and 5.8±2.1 years respectively. Most of the patients fell within the age category of 7-7.9 years old (40.9% for girls and 50% for boys). Patients, concerning etiology of precocious puberty were classified in three categories: 42.6% of patients had central precocious puberty (CPP) including idiopathic CPP (87.5%) and neurogenic CPP (12.5%). 23.3% of patients had peripheral precocious puberty (PPP) including congenital adrenal hyperplasia (CAH) (42.8%), ovarian cysts (28.4%), McCune-Albright syndrome (14.2%) and adrenal carcinoma (14.2%). 34.1% of girls and 25% of boys had normal variant puberty including premature thelarche (57%), premature adrenarche (38%) as well as premature menarche (4.7%l). Conclusion: The most common etiology of precocious puberty in girls was idiopathic central precocious puberty

and premature thelarche while in boys they were neurogenic central precocious puberty and CAH. Therefore, precocious puberty in girls is usually benign. In boys, CNS anomalies should first be considered in the differential diagnosis of CPP. Therefore brain Magnetic Resonance Imaging (MRI) is mandatory in all cases. Keywords: central precocious puberty, peripheral precocious puberty, premature thelarche, congenital adrenal hyperplasia

Incidence of Phenylketonuria in the area under coverage of Shahid Beheshti University

of Medical sciences in 1390 Nahid Arjmand, Faranak Rahimi, Jalil Daroozi Deputy of Health, Shahid Beheshti University of Medical Sciences Background: Phenylketonuria (PKU) is a metabolic genetic disorder due to a mutation in Phenylalanine Hydroxylase gene on 12th chromosome resulting in reduction of the enzyme’s activity and thus accumulating of phenylalanine in the body. The patient is normal at birth but untreatment can lead to mental retardation, seizures, and other serious medical problems. Early diagnosis and strict phenylalanine-restricted diet is crusial for prevention of severe and irreversible mental retardation. The disease prevalence in Iran is expected to be higher than that in the world because of high consanguineous marriage rate. Methods: During 1390, all 3-5 day old newborns living in the area under coverage of Shahid Beheshti Medical University were referred to screening centers where a blood sample was taken from lateral parts of the newborn's heel, using a Lancet and Guthrie paper. If phenylalanine level of this sample was ≥ 4 µIU/ml, a venous blood sample was taken for HPLC analysis. Suspicious patients who had a venous phenylalanine level ≥ 4 µIU/ml were referred to a specialist for further investigation and treatment. Findings: Totally 123323 newborns (84% of all live births) were screened during one year. Heel blood sample showed positive results for PKU in 49 newborns of which 23 cases (47%) had a venous phenylalanine level ≥ 4 µIU/ml. Finally, the disease was confirmed in 17 neonates (74%) and the incidence was calculated as 1 in 7254 screened newborns. Considering one affected fetus, diagnosed by prenatal diagnosis procedures in pregnant women who previously had a PKU child (leading to medical abortion), the incidence was 1 in 6851. Conclusion: The incidence of PKU in this area is higher than that in developed countries like US (1 in 10000 to 1 in 12000) but less than countries like Turkey (1 in 2500) or Ireland (1 in 4000) with high rates of consanguinity. More research is needed to detect other reasons of the difference between countries. Keywords: Phenylketonuria, neonatal screening, incidence

Clinical, Enzymatic, and Molecular Diagnosis

of Mucopolysaccharidoses (MPSs) in Iran

Yousef Shafeghati Sarem Cell Research Center and Medical Genetics Department

Mucopolysaccharidoses are a group of rare mostly autosomal recessive metabolic and genetic disorders.



Because of the high rate of consanguinity, they are not uncommon in our population. They are genetic disorders and now most of the responsible genes were determined, mapped, and cloned. Common mutations in different ethnic groups are being discovered. Clinical diagnosis is not difficult for experienced physicians, but for treatment and prevention measures in the future pregnancies precise biochemical or molecular diagnosis is mandatory. It is necessary to detect the type of MPS by measurement of responsible enzyme activity in the leukocytes and fibroblasts. Whenever we determined the exact type of the MPS by enzyme assay, we would be able to treat the patients with the different therapeutic options, like enzyme replacement therapy(ERT), bone marrow transplantation(BMT), stem cell transplantation(SCT), or even gene therapy in the near future. In the past 2 decades more than 240 families with 311 patients were referred for diagnosis and confirmation. After gathering necessary information regarding the probands and the families, and constructing detailed family trees, we evaluated the cases comprehensively by clinical and XR examination. If the phenotype of the patient was suggestive for one of MPSs, urine GAGs were positive, and XR exam showed Dysostosis Multiplex Congenita(DMC), then we arranged to investigate enzyme analysis for the suspected phenotype. We detected 64 families with type III MPS, followed by 56 families with type II, and 39 familes with type VI respectively. We also have seen 38 families with type I, and 37 families with type IV. At present, wild type or normal enzymes for MPSs type I, II, and VI are being produced by recombinant DNA technology. Enzymes for these types now are approved following extended clinical trials, are safe and efficient and alleviate many systemic signs and symptoms of these diseases. There are some difficulties regarding the marketing and high cost of the available enzymes in our population. If public health responsibilities and insurance companies support the families financially there is not any problem to use enzymes in our patients in Iran. Some of our patients have been treated by BMT, and the results would be released in the conference. Also we should consider other therapeutic modalities, like stem cell transplantation, or even gene therapy for the future. Now our priority is to do prenatal testing and detect affected fetuses by enzyme assay or molecular analysis on fetal cells after amniocentesis or CVS. We think that termination of the affected fetuses is logical option to control and prevent this type of very unfortunate disorders. Keywords: MPS, enzyme assay, enzyme replacement, gene therapy

Prevalence of vitamin D deficiency in children

with growth abnormalities Alvand Gharib1, Eisa Salehi2, Katayoon Bidad3, Alireza Moghaddam4, Heshmat Moayeri5 Amir Seyed Ali Mahbod1 1Naamdar Medical Diagnostic Lab. 2Immunology Department. 2,3,4,5Tehran University of Medical Sciences, 3Immunology, Asthma and Allergy Research Institute. 4Department of Pediatrics, Imam Khomeini Hospital. 5Department of Endocrinology, Imam Khomeini Hospital Vitamin D deficiency has recently emerged as a health concern all around the world. In children, it can lead to osteopenia, osteomalachia, rickets and also immune related diseases. The main objective of this investigation was to

determine the prevalence of Vitamin D deficiency among children with growth issues in two spring and winter seasons and its relevance to calcium and phosphorus levels. Methods: One-hundred and thirty four children aged 0 to 12 years old with growth problems were included in the study. Vitamin D, calcium and phosphorus levels were measured using 25-Hydroxy Vitamin D EIA Kit and Hitachi Boehringer Mannheim 717 Analyzer, respectively. Findings: A total of 86 subjects (64.17%) were Vitamin D deficient (25(OH) < 20ng/ml). Patients were divided into 4 age categories. The prevalence of Vitamin D deficiency was 35.3% in 0-3 year-old children, 64.5% in 3-6 year-olds, 75.0% in 6-9 year olds, and 66.0% in 9-12 year-olds. Among 31 two to twelve years-old-calcium-deficient subjects, 15 (43.6%) were also deficient in vitamin D. Calcium and Vitamin D levels were significantly (p <0.001) correlated. Phosphorus level was not related to Vitamin D level. Conclusion: High prevalence of vitamin D deficiency can be related to air pollution in Tehran and lack of exposure to sunlight, especially in winter. It is highly recommended to evaluate Vitamin D and Calcium levels in children with growth problems. Low or no intake of vitamin D supplements can lead to vitamin D deficiency as lower vitamin D deficiency prevalence was observed in neonates who took vitamin D supplements. Keywords: children, growth problems, viamin D deficiency, calcium, phosphorus

Therapy development for Neuronopathic Lysosomal Storage Disorders

Maryam Banikazemi Greater New York Metropolitan & Washington DC Metro area Lysosomal storage disorders (LSDs) constitute a group of recessive genetic diseases resulting from a specific enzyme deficiency that act to catabolize macromolecular substrates in the lumen of lysosomes. Deficient enzymes' activity leads to pathogenic accumulation and storage of the substrate for these enzymes. The consequent accumulation of undigested metabolites in lysosomes leads to multi-systemic dysfunction, including progressive neurologic deterioration, mental retardation, organomegaly, blindness, and early death. In last decades, significant advances in the treatment of non-neuronopathic forms of LSDs via enzyme replacement therapy (ERT) have been achieved. This approach while mostly improves visceral manifestations of LSDs, addressing the CNS aspects of these disorders remains a formidable challenge. Bone marrow transplantation (BMT) with hematopoietic stem cells has been successful in some forms of MPS with long term survival following successful engraftment. However, there is high rate of associated morbidity and mortality with BMT. In recent years, new approaches for targeting the Blood Brain Barrier have been under investigation. These approaches include use of peptide or pharmaceuticals as molecules that can bind to endogenous receptor-mediated transporters in the BBB, substrate reduction therapy, RNAi-mediated therapy of downstream targets, use of microfluidic or MEMS devices for in-vivo spatial and temporal controlled delivery of exogenous iomolecules. The hope is that these treatment regimens alone or in conjunction with ERT or BMT can offer desired results in CNS manifestation of LSDs. In addition, characterization



of pathophysiological processes, characterization and validation of diagnostic biomarkers, intermediate surrogate endpoints, and prognostic biomarkers has been the focus of many ongoing studies. Summary of advances in the development of new treatment for several neuronopathic lysosomal storage disorders will be presented. Keywords: CNS Therapy, Lysosomal Storage Disorders, neuronopathic Natural history of early onset lysosomal acid

lipase (LAL) deficiency (Wolman Disease) confirms a severe and rapid disease course

Maryam Banikazemi1, Christian J. Hendriksz2, Edmond Wraith2, Anil Dhawan3, Chester Whitley4, Maryam Banikazemi5, Martin Bialer6, Ornella Guardamagna7, Simon Jones2, Julian Raiman8, Stephen Cederbaum9, Maja DiRocco10, Jennifer Domm11, Gregory Enns12, David Finegold13, Amy Simonds14, Stephen Eckert14, Eugene Schneider14, and Anthony Quinn14

1New York Presbyterian Hospital, New York, NY, USA. 2 Birmingham Children's Hospital NHS Foundation Trust, Birmingham, United Kingdom. 3Central Manchester and Manchester Children’s NHS Trust, Manchester, United Kingdom. 4Kings College Hospital NHS Foundation Trust, London, United Kingdom. 5University of Minnesota, Minneapolis, MN, USA. 6North Shore Long Island Jewish Hospital, Manhasset, NY, USA. 7Department of Pediatrics, University of Turin, Turin, Italy. 8The Hospital for Sick Children, Toronto, ON, Canada. 9 UCLA, Los Angeles, CA, USA. 10 Istituto Giannina Gaslini-Ospedale Pediatrico, Genova, Italy. 11Vanderbilt Children's Hospital, Nashville, TN, USA. 12Stanford University School of Medicine, Stanford, CA, USA. 13 Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. Synageva BioPharma, Lexington, MA, USA Lysosomal Acid Lipase (LAL) Deficiency is a rare autosomal recessive disorder caused by mutations in the LIPA gene encoding lysosomal acid lipase, a key enzyme responsible for lysosomal processing of cholesteryl esters and triglycerides. Markedly reduced LAL activity results in the accumulation of lysosomal lipids in various tissues, causing malabsorption, hepatosplenomegaly, liver failure, cytopenias, and growth failure. The early onset form of LAL Deficiency, also known as Wolman disease, is a progressive disease which typically manifests in the first few months of life and leads to death usually before the child’s 1st birthday. Case reports have described the hallmark features of the disease. This is the first natural history study of early onset LAL Deficiency. Demographic and clinical information on 28 patients (16 males, 12 females) with LAL Deficiency have been collected utilizing clinical chart data abstractions and summarized to date. This report focuses on 19 patients who had Wolman disease defined by growth failure by 6 months of age. The median age (range) at first symptom, at diagnosis, and at death were 1.00 month (0.23–3.0), 2.17 months (1.05 - 7.07), and 3.44 months (1.45–37.37), respectively. Six of these patients had hematopoietic stem cell (n=5) or liver transplants. Excluding these 6 patients, the median age (range) of death was 2.95 months (1.45–5.72). Of the 6 transplanted patients, 5 died before 9 months of age, while the 6th, the liver transplant recipient, died at 37.37 months. Six out of 18 patients (33%) did not have adrenal calcifications. We conclude that early onset LAL Deficiency/Wolman disease is an inherited metabolic

disease with a rapidly progressive clinical course and near certain death during the first year of life.

New Advances in treatment of Hyperammonemia

Maryam Banikazemi Greater New York Metropolitan & Washington DC Metro Area

Metabolic disturbance characterized by an excess of ammonia in the blood (Hyperammonemia), is a dangerous condition that may lead to encephalopathy and death due to intracerebral hypertension and increased ammonia level. Hyperammonemia may be primary or secondary. Urea cycle defects (UCD), the principal mechanism of ammonia detoxification are the most common cause of primary hyperammonemia in children. The urea cycle is also regulated by NAG produced by N-acetylglutamate synthase (NAGS) function in mitochondria. Reduced activity or deficiency in any of urea cycle enzymes and/or NAG deficiency may results in excess ammonia accumulation. Secondary hyperammonemia in neonatal period can be caused by some of organic acidemias (e.g. Propionic acidemia, Methylmalonic acidemia) through inhibition of NAG synthesis. Several other conditions such as increased metabolism of protein, infection, impaired ammonia uptake and elimination by liver and increasingly certain drugs may cause hyperammonemia. The medical management of UCD used to be limited to supportive care; treatment of ICH, reduction of protein intake and increase in caloric intake, which in many patients were not sufficient and often patients would require hemodialysis. Introduction of compounds that promote alternate pathways for nitrogen excretion was a big breakthrough. Administration of nitrogen scavenger drugs (Sodium phenylacetate and sodium benzoate) allows excess nitrogen to be excreted through alternative pathway. In addition, intravenous administration of L-arginine hydrochloride and supplementation of N-acetylglutamate cofactor (carglumic acid) has changed management and outcome of hyperammonemia drastically. At present, in conjunction with a low protein diet and calorie supplementation, nitrogen scavenger drugs or their combination preparation (Ammonul) are indicated in the prevention and treatment of hyperammonemia. Also in conditions that hyper-ammonemia is due to the reduction of N-acetylglutamate, this cofactor should be provided.

Self-monitoring of blood glucose Shadab Salehpour Shahid Beheshti University of Medical Sciences Self-monitoring of blood glucose (SMBG) provides significant benefits to patients with diabetes and their healthcare providers. SMBG is increasingly recognized as an integral part of intensive therapy for all forms of diabetes. SMBG is helpful to patients with diabetes in four distinct ways. First, it allows patients and clinicians to detect high or low blood glucose levels, thereby facilitating therapeutic adjustments to achieve long-term A1c goals. Second, SMBG helps protect patients by allowing them to immediately confirm acute hypoglycemia or hyperglycemia. Third, the technology facilitates patient



education about diabetes and its management by giving patients more self-care responsibilities. Fourth, SMBG helps motivate people toward healthier behavior. Numerous trials have been carried out to determine the true impact of SMBG on glycemic control. Some, including randomized, controlled trials, have demonstrated the efficacy of SMBG. Among patients with type 1 diabetes, SMBG has been associated with improved health outcomes. Specifically, increasing frequency of SMBG was linearly correlated with reductions in HbA1c among type 1 patients. Among patients with type 2 diabetes, a higher frequency of SMBG was associated with better glycemic control among insulin-treated patients who were able to adjust their regimen. Other studies, however, have suggested that SMBG has not achieved its true potential impact as an aid to improving glycemic control. Here, we discuss the clinical utility of SMBG in type 1 and type 2 diabetes and look at the relationship between SMBG and improved overall glycemic control as measured by Hg A1c.

Endocrine and metabolic disorders in ß-

thalassemia major patients Fatemeh Saffari, Abolfazl Mahyar Qazvin University of Medical Sciences Background: Thalassemia is the most common hereditary anemia and beta thalassemia major is its most severe form. Endocrine abnormalities in thalassemia major are common

disturbing complications that need prompt management. The purpose of this study was to determine the endocrine disorders and bone mineral density in patients with major ß-thalassemia in Qazvin, Iran. Methods: In this cross-sectional study, 77 patients with ß- thalassemia major (15-36 years old) were enrolled. Physical examination, laboratory tests, bone radiography and bone density measurements were performed. Then, the data were analyzed. Findings: Forty patients were males. The mean age was 21.26±4.53 years old. The mean BMI was 20.15±2.79 kg/m2. Impaired puberty, short stature, hypothyroidism, diabetes mellitus, IGT, hypoparathyroidism, vitamin D deficiency and vitamin D insufficiency were observed in 46.8%, 33.8%, 18.2%, 16.9%, 13%, 7.8%, 45.5% and 24.7% of patients, respectively. Nearly 80% of patients had low bone mineral density. Bone mineral density was significantly associated with hypogonadism (p=0.001), short stature (p=0.026), hypoparathyroidism (p=0.031), hypothyroidism (p=0.048), diabetes mellitus (p=0.002) and vitamin D deficiency (p<0.001). Conclusion: Impaired puberty and short stature were the most common endocrine complications in our population. Low bone density (osteopenia, osteoporosis) is significantly different in ß-thalassemic patients with and without endocrine complications. Keywords: Major ß-thalassemia, bone mineral density, osteopenia, osteoporosis, puberty

13

The prevalence of infantile colic and some of its risk factors

Parichehr Tootoonchi Growth And Development Center, Children Medical Center, Tehran University of Medical Sciences Background: Infantile colic is defined as a severe crying started suddenly and may continue for hours. It is associated with flashing, perioral paleness, firmness of abdomen with legs drawn to the abdomen. Methods: We selected 5 health centers in south of Tehran randomly and interviewed with the mothers of 450 under 1 year old infants attended in them to determine the prevalence of infantile colic and some of its risk factors. Findings: The prevalence of infantile colic was 55.1% (248 cases). The age of onset was before 1 month in 72.1% and the mean age of abating of colic was 3.41 months. Infantile colic was more common in preterm infants. Furthermore, the colic was more prevalent in infants of 20-35 year old mothers than the infants of under 20 year old mothers. Besides, 99.6% of cases started supplemental foods before 6 months of age. The colic attack partially relieved by gas or stool passage in 84. 2% of cases, but with sucrose water just in 29.4%. Conclusion: Our results were very similar to other reports regards to the age of onset and abate, and the route of partial relief. Further community-based studies is recommended to determine the exact prevalence of infantile colic and its risk factors. Keywords: infantile colic, Tehran, prevalence

Effect of Pyridoxine in control of vomiting

due to gastroenteritis in children

F Jafari1, P Yousefi1, H Sheykholeslami1, M Rafeie2 1School of Medicine. 2Department of Statistics. 1,2, Arak University of Medical Sciences Background: Antiemetic drugs can be used when frequent vomiting due to gastroenteritis causes health problem. Study on the use of pyridoxine in the treatment of vomiting is not performed in Pediatrics. This study intends to review effect of intravenous pyridoxine in the control of vomiting due to gastroenteritis. Methods: This study is a single blind randomized clinical trial with placebo and single center. 147 Patients diagnosed with gastroenteritis and vomiting were hospitalized from August to December 2011 in Amirkabir hospital, were placed in case and control groups. In the control group intravenous fluid with distilled water (placebo) was used. In the case group pyridoxine was given along with intravenous fluid therapy. Since admission for three days, numbers of vomiting and other symptoms were collected in data form. Comparing of two groups was analyzed in SPSS software with non-parametric tests. Findings: Most children were males, age 6 months to 2 years and living in the city. There was no significant difference between two groups in the mean of vomiting frequency at various points and entire period of hospitalization (p>0.05). Also there was no significant

difference between two groups in the mean of duration of hospitalization (p=0.19). Conclusion: Based on this study Pyridoxine (Vitamin B6) was not effective in treating vomiting due to gastroenteritis in children, and did not reduce the duration of children hospitalization. Key words: Gastroenteritis, vomiting, Pyridoxine, Vitamin B6

Effect of toilet training in treatment of

functional constipation in Aliasghar Children Hospital

Tahereh Parvini, Shahrbanu Nakhaei Tehran University of Medical Sciences

Background: Toilet training is one of the developmental steps which is necessary to achieve by child. However, most children trained without problem and 3 percent experience complications during toilet training. One of the most frequent complications is functional constipation. Treatment of this problem includes dis-impaction and maintenance therapy with drugs or toilet training. We were to compare two methods of therapy in functional constipation, toilet training with medications and medications alone. Methods: In this intervention study(RCT), 3 to 12 year children with functional constipation whom referred to gastroenterology clinic of aliasghar children hospital from 2008 to 2009, were evaluated. They had no mental or physical disability and there was no organic diagnosis. Patients were divided in two groups: toilet training with medication and medication alone. Response to treatment was evaluated in three ways: comparison of two groups, number of patients with significant improvement, and patients with complete cure. Findings: From the total of 60 patients with functional constipation, 30 patients were in toilet training and medication group and 30 patients were in medication group. 41.66% were males and 58.34% were females. Mean age was 2.30± 1.858 years. According to repeated measures ANOVA test results there was no significant difference between two groups in mean of defecation intervals(p-value=0.103), but response to treatment according to defication intervals was significant in both groups. In addition there was no significant difference between two groups in the case of defecation per day(p-value = 0.795) but response to treatment according to defecation per day was significant in both groups. According to non parametric K-related samples (Cochran’s Q) test results there was significant difference between two groups in soiling (p-value < 0.001). Response to treatment according to soiling, enema usage and suppository usage was not significant in different months of study. Conclusion: In conclusion functional constipation is a hard to treat problem in children, and toilet training in addition to medications can improve patients health and can use as an effective therapy in constipated children. Key words: functional constipation, toilet training, behavior therapy

Gastroenterology

14 Proceeding of the 24th International Congress of Pediatrics – Oct 2012 Gastroentrology

Diagnosis and management of cyclic vomiting syndrome; Clinical report

Vajiheh Modaresi Social Security Organization, Shohada hospital, Yazd Background: The cyclic vomiting syndrome is defined as the episodes of severe nausea and vomiting lasting hours to days. The patient is healthy between the episodes and has no clinical finding. This disease is one of the rare periodic syndromes described mostly in children of school and pre school age. The differential diagnosis is difficult because many diseases may present with episodic vomiting. Since there are some serious problems which may mimic this syndrome, intense investigations is needed. Sometimes the disease is over-diagnosed in cases that are not evaluated according to standard medical approaches. Developing a national guideline for CVS to improve the recognition and treatment seems a necessity. Case Presentation: Here in, we try to give a clinical report from our cases who presented between 2010 and 2012 with recurrent episodes of nausea and vomiting separated by symptom free intervals and recommend that children with cyclic vomiting be evaluated for a possible metabolic or neurological disorder if any of the following conditions are met. 1. Presentation under age 2 years 2. Vomiting episodes associated with intercurrent illnesses, prior fasting, increased protein intake 3. Any neurological finding: ataxia, dystonia, or another gait disturbance; mental retardation; seizure disorder; or acute encephalopathy (including true lethargy, severe irritability, confusion, psychosis or rapidly changing/unstable mental status) 4. Laboratory metabolic findings: hypoglycemia, substantial anion gap metabolic acidosis, respiratory alkalosis, or hyperammonemia. Clinical features of cyclic vomiting syndrome (CVS) are also discussed along with the literature findings. Management involves a responsive, collaborative doctor-patients relationship and precise use of antiemetic agents to abort attacks, and also using suitable prophylactic agents with aim of good prevention. Although there are no published trials that evaluate the impact of lifestyle alterations on CVS attacks, most authors believe that lifestyle changes including avoidance from fasting, triggering foods(chocolate, cheese, monosodium glutamate and antigenic foods), excitement and excessive energy output and also maintaining good sleep would reduce episode frequency in children with CVS. Keywords: cyclic vomiting syndrome,CVS,recurrent vomiting

Poor gain weight in inflammatory diseases in

children Zohreh kavehmanesh Baqiyatallah Medical Sciences University

One of the most common problems facing in children with inflammatory diseases(IBD) is poor gain weight. The weight may be affected by nature of the inflammatory process itself, or may be contributed by several other factors such as corticoid therapy. Inflammation plays an important role in poor weight gain of IBD children. Up to 13% of patients with crohn disease and 3% of ulcerative colitis patients have height below third percentile at the time of diagnosis. Therefore, treatment that reduces inflammation will cause increase in growth velocity.

Unfortunately some patients continue growth failure despite of treatment. Malnutrition because of decreased food intake, malabsorption, maldigestion and increased energy consumption also influence poor gain weight in IBD. There may be an important role for poor weight gain and especially poor growth velocity with corticoid therapy in children during puberty. Glucocorticoids have effects on growth hormone secretion and action and also bone and collagen formation. Hypogonadism due to inflammatory process and malnutrition also causes some effects on growth process. Osteoporosis and osteomalacia are common bone diseases in IBD. All children with IBD should have regular monitoring of weight, height and pubertal assessment. To reduce effect of disease and treatment on bone formation, the patients should be annually monitored with bone densitometry and if z-score is 1 SD below the mean, calcium 1000-1300 mg and vit D 800-1000IU daily should be supplemented. These patients also benefit from nutritional counseling and reduction of corticoid treatment (if possible). We should avoid unnecessary diet restrictions. When children refuse to eat, we can support them by enteral tube feeding through the night. When the disease is not controlled with usual treatments, we can use immunomedulators such as infliximab especially in crohn disease. There are a few reports on growth hormone therapy in growth failure of IBD patients. Keywords: growth, IBD, crohn disease, ulcerative colitis

Amplification of non-structural genes (NS) segments 5, 8 of rotavirus as a major cause of

neonatal gastroenteritis by RT-PCR Elham Ahmadi1, Hoorieh Soleimanjahi1, Majid sadegizada 2, Ali Teimoori1 1 Department of Virology, Faculty of Medical Sciences 2 Department of Genetics, Faculty of Basic Sciences Tarbiat Modares University Background: Group A rotaviruses are responsible for the vast majority of acute gastroenteritis and the leading cause of severe dehydrating diarrhea in infants and is responsible each year for an estimated 600,000 to 870,000 childhood deaths. Therefore, diagnosis and treatment of this disease is important. Since the complete sequence of bovine rotavirus genome is not available, development of RT-PCR for detection and diagnosis of the Rota virus is needed . Methods: Rotavirus RNA was extracted from confluent monolayer of MA104 cells. The genome electrophenotyping has been applied using 10% polyacrylamide and stained with silver nitrate .The Full-length product of the genes coding for nsp1,2 were amplified by RT-PCR with specific primer. Findings: The PCR products of the genes were analyzed by gel electrophoresis then complete nucleotide sequence of each gene was determined from gel-purified PCR products and subjected to sequencing. Conclusion: Considering the importance of rotavirus infection in young children, methods used to determine the characteristics of them are constantly evolving. In order to manage of diseases and successful vaccination, data collection on Rota virus is necessary. Therefore, understanding of molecular and antigenic characteristics is useful and imperative. This study and other same works can provide the quality and quantity of investigation on Rota virus biology and evolution.


Gastroentrology

Keywords: Rotavirus, acute gastroenteritis, complete nucleotide sequence

Probiotics for the treatment of pediatric

Helicobacter Pylori infection: A randomized double blind clinical trial

Maryam Shoaran1, Ahmad Khodadad1, Fatemeh Farahmand1, Mehri Najafi1 Department of Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences Background: Helicobacter Pylori (H. Pylori) is recognized as a major etiological factor in the pathogenesis of gastritis and peptic ulcer disease. H.Pylori eradication has a failure rate of more than 30% in pediatric patients, particularly because of poor compliance, antibiotic resistance and occurrence of side-effects. This study was aimed to determine whether adding the probiotics to a standard anti-H. Pylori regimen could minimize the gastrointestinal side-effect prevalence and improve the eradication rate. Methods: Double-blind randomized placebo controlled study conducted at Children’s Medical Center in Tehran, Iran. Sixty six H. Pylori positive children were treated with a triple drug treatment protocol (omeprazole +amoxycillin +furazolidon) and randomly allocated to receive either probiotic or placebo. All patients underwent esophagogastroduodendoscopy. H.Pylori infection was diagnosed by either rapid urease test (RUT) or histology. H.Pylori status was assessed after 4-8 weeks of the completion of treatment with stool H. Pylori antigen test. The side effects of the treatment were determined in each group. Findings: Mean age of patients was 9.09 years (3‐14). 44 (65.7%) patients were boys(sex ratio 2:1). All 66 patients completed the course of treatment and follow-up. The rate of H. Pylori eradication was significantly higher in probiotic group (P=0.04). In probiotic supplemented children, there was a lower rate of nausea/vomiting (P=0.02) and diarrhea (P=0.04) during treatment. Conclusion: This study showed that probiotics have positive effects on the eradication of H.Pylori infection. Adjuvant therapy with probiotic is recommended in order to reduce the frequency of antibiotic induced side-effects during treatment with antibiotics. Keywords: children, probiotic, endoscopy, Helicobacter Pylori, eradication

Prevalence, serologic and histological

examination celiac disease in Irritable Bowel Syndrome patients under 20 years

Reza Kalvandi, Asghar Rahmani, Masoud Yasami, Mohammad reza Hafezi ahmadi, ali jafari heirdarloo, Mohammad rasool yasami Ilam University of Medical Sciences Background: Irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder and celiac disease (CD) is an enteropathy disease caused by sensitivity to gluten and autoimmune disorders. Both IBS and CD are having similar symptoms and mechanism of physiopathology. CD is common in children and adolescents. Studies have suggested the increasing rate of CD in patients with

irritable bowel syndrome. However, these studies have had contradictory evidence. The purpose of this study was investigation of celiac patients presenting with diarrhea-predominant irritable bowel syndrome in two groups over the following and down two years (2009-2011). Methods: In this cross-sectional study, between March 2010 and July 2012, IBS patients with diarrhea predominant clinical presentation in room II criteria, that were referred to Ilam gastroenterology clinics enrolled in the study. (anti-tTG) serology was checked to initially recognize possible CD cases. Patients with a positive test were offered for endoscopic duodenal biopsy to confirm the diagnosis of CD. Data collected and analyzed by SPSS 16. Findings: A total of 269 (129 males and 140 females) patients with IBS were included. The mean age of patients was 11.32. Number of individuals over ten years were 226 patients (101 males and 125 females) with a mean age of 16.4 years and 43 patients were under 10 years old (22 males and 21 females) with a mean age of 25.6 years. In upper 10 year group, twenty three patients (7 males,16 females) tested positive for anti-tTG antibodies, and duodenal biopsies confirmed the diagnosis in all of them. Also below 10 years, two patient (2 females) showed positive anti TTG and duodenal biopsies confirmed the diagnosis. Thus, Prevalence of celiac disease in all patients was %29.9 (10.1% over ten years and under 10 years, 4.65%). There was a significant relationship between sex and positive antibody titer (p≤0.05). Conclusion: CD is common in patients with presumed IBS at young ages under 20 years. CD can be considered as a differential diagnosis for IBS and routine testing for celiac disease may be indicated in all patients being evaluated for irritable bowel syndrome. Keywords: Irritable Bowel Syndrome, celiac Disease, antibodies, duodenum, diarrhea, adolescents

Prevalence of vitamin D deficiency and rickets in children with cholestatic liver

diseases Bahram Mohammadi, Mehri Najafi, Jamshid Mohammadi Yasooj University of Medical Sciences Background: Malabsorption of vitamin D and disturbance of calcium/phosphorous balance are complications of chronic liver diseases in children. This study was aimed to determine the prevalence of Vitamin D deficiency and rickets in children with cholestatic liver diseases. Methods: Forty eight children with established cholestatic liver disease who referred to gastrointestinal clinic of Children Medical Center (Tehran, Iran) between April 2010 and March 2011 were enrolled in a cross-sectional study. Laboratory analysis including calcium, phosphate, albumin, total and direct bilirubin, aminotransferases, alkalinephosphatase, prothrombin time (PT), parathyroid hormone, total protein determined by routine laboratory techniques. Findings: Mean age of participants was 299.1 ± 676.8 days (range 2-3600 days) whereas twenty one were females (43.8%) and 27 (56.3%) were males. Twenty two (45.8%) had evidences of rickets in X-ray evaluation. Three children with rickets and two with normal X-ray had vitamin D deficiency while ten in rickets group and 16 in normal group had vitamin D insufficiency.


Conclusion: The main underlying diseases were anatomical biliary atresia in cases with rickets and idiopathic in other group. Rickets and Vitamin D deficiency should be considered in chronic cholestatic children. Keywords: rickets, Vitamin D, chronic cholestasis, children

Hypertrophic pyloric stenosis or Cow's milk

protein allergy Shiva Rafati Shahed university Background: Cow’s milk protein allergy symptoms can develop in exclusively breastfed infants with an incidence rate of 0.5%.. Transfer of native bovine proteins into the breast milk is controversial. We report one case of Cow's milk protein allergy in an infant who presented with severe vomiting. Methods: Our patient was a baby who born by c/s. His weight was 3800g. His mother was 28 year old with no history of disease. He had severe crying after 15th day. He was exclusively breast fed. Urination and defecation were normal. With diagnosis of colic, he used several anticolic drugs with no improvement. He had vomiting that increased gradually. In 2 months he had severe and projectile vomitingx.His weight was 4000g. Antireflux drugs were used for him. With no improvement. Barium Swallow was performed and hypertrophic pyloric stenosis reported. He admitted in hospital for surgery. Dairy products were eliminated from maternal diet. After three days he was calm without vomiting and crying. He was discharged with good condition. Conclusion: Cow’s milk sensitivity or allergy can cause colic-like symptoms, eczema, wheezing, vomiting, diarrhea (including bloody diarrhea), constipation and functional bowel symptoms. This can lead to confusion and difficulty in managing pediatric surgical patients who also have Cow’s milk sensitivity or allergy. Excluding cow's milk from the mother's diet is the only cure. Keywords:projectile vomiting, cow's milk protein allergy, hyprtrophic pyloric stenosis

The effect of vitamin A on diarrheal disease in children

Mohammad Reza Sharif Pediatric department, Kashan university of medical sciences Background: Diarrheal disease are the most common health problems in developing countries and have an important role in malnutrition of children.The aim of this study was to evaluate the vitamin A in improvement of watery diarrhea in under 5 years old children. Methods:This study was performed on 200 under 5 year old children with acute watery diarrhea refered to Shahid Beheshti hospital in 2011. Patients were enrolled into two groups (vit A group and control group). Duration of diarrhea and the average of diarrheal episodes and hospital stay were recorded. Data were analyzed by the use of Chi-squared, Mann withnny, Kolmogorov-smirnov tests. Findings:This study showed that the average of duration of diarrhea was significantly lower in the vit A group (4.2 ±

1.36 vs. 6.23 ± 2.3 respectively, p<0.0001). Diarrhea frequency was significantly lower in the vit A group (6.08±3.18 vs. 8.68±5.33 respectively, p<0.0001); however, duration of hospital stay was significantly lower in the vit A group (4.45±1.4 vs. 6.3±2.16 respectively, p<0.0001). Conclusion: Vitamin A perception in therapeutic plan of diarrheal disease in children can to be decreased of diarrhea duration and severity. Keywords:diarrheal disease, Vitamin A, children

Approach to a child with chronic diarrhoea

Riccardo Troncone Department of Pediatrics & European Laboratory for the Investigation of Food-Induced Diseases, University Federico II, Naples Diarrhea is a common manifestation of many gastrointestinal diseases. It consists of a decrease in fecal consistency and occurs when there is an imbalance between the processes of water and ions intestinal absorption and secretion. This imbalance may be caused by either decreased absorption (osmotic diarrhea) or increased secretion (secretory diarrhea). Chronic diarrhea refers to the persistence of loose stools (with or without an increase in stool frequency) for at least two weeks. A wide range of conditions can cause chronic diarrhea and it is important to identify the underlying cause to ensure that a child receives the most appropriate treatment. A careful history and physical examination are the cornerstone of a successful diagnosis. A family history is always crucial in assessing the possibility of inherited conditions or disease. The most important data obtained from the history are age at the onset of diarrhea and whether it was abrupt or gradual in its appearance. It is important to think in terms of the child’s age when establishing a differential diagnosis as certain conditions first appear only at specific ages. If this approach is combined with establishing the diarrhea characteristics (secretory, osmotic, or mixed) and the presence or absence of malabsorption, the diagnostic possibilities are reduced and it is possible to select a relatively small number of tests to establish the diagnosis. Another important point in the approach to a child with chronic diarrhea is the growth and nutritional assessment. Weight for height is the simplest index of growth failure secondary to malnutrition. Sequential heights and weights, with measurement of head circumference, are critical for determining whether the disease has altered growth and weight gain. A common mistake is a failure to appreciate that the lack of progress on the growth chart may be due to feeding a diluted hypocaloric formula or clear liquids in an effort to reduce diarrhea. The character of the diarrhea (watery, presence of blood and mucus, presence or absence of undigested food particles) may aid in establishing its type, that could be secretory, inflammatory, osmotic, malabsorptive, or of a functional nature. If a child or a teenager has to get up in the night to defecate or becomes incontinent, this suggests an organic basis for diarrhea. These signs together with the presence of blood and/or mucus in the stools indicate inflammation in the intestine, and strongly suggest a diagnosis of IBD. On the contrary, indicators of a functional etiology are long duration of symptoms (> 12 months), lack of significant weight loss, absence of nocturnal diarrhea, and straining with defecation. The presence of vomiting indicates a


Gastroentrology

disturbance of intestinal or colonic motility. Abdominal distension could be secondary to true obstruction, pseudo-obstruction, malabsorption, or hypersecretion of liquids in the intestine. A review of any systemic or extraintestinal signs and symptoms is useful in the search for other diagnostic clues. The presence of fever is more typically seen in infectious or inflammatory processes. Arthralgia, arthritis, iritis, pyoderma gangrenosum, and hepatic disease are all indications of IBD. Recurrent respiratory tract infections could be an indication of cystic fibrosis or congenital and acquired immune deficiency. If the patient is a neonate with diarrhea in the first days of life, hospitalisation is mandatory for an accurate evaluation and therapy. It is important to note that some of these pathological conditions, starting in neonatal age, are related to genetic defects. Thus molecular analysis could be helpful in the diagnostic approach to these patients and whenever possible in genetic counselling for the family.

Prevalence of celiac autoantibody (anti tTG) in children with diabetes referred to Abuzar

hospital Majid Aminzadeh, Mehran Hakimzadeh, Mohammad Reza Fathi Ahwaz University of Medical Sciences

Background: Celiac disease (CD) is a chronic enteropathy caused by hypersensitivity to gluten. Most studies have shown more prevalence of CD in the patients with diabetes type 1 (DM1). The prevalence of CD among DM1 children is unknown in our area. The aim of this study was to survey the prevalence of celiac disease in diabetic children in Ahwaz. Methods: In a cross-sectional study, 226 children with diabetes mellitus type 1 referring to Abuzar children's hospital (endocrine department and clinic) from 2009 to 2011 were surveyed. The serum levels of Anti-tTG and IgA were measured by immunometric Enzyme Immunoassay and immunotorbidimetric respectively. Findings: Twenty eight of 226 patients (12.4%) with diabetes mellitus type 1 were serologically positive for celiac (cut off ≥ 20 U/ml). 21 of seropositive patients were females (18.4%) and 7 (6.2%) were males. Conclusion: Celiac disease in childhood diabetes (DM1) is common. Screening of celiac disease with serologic tests and confirming by biopsy is necessary, while early diagnosis can prevent the disease's complications. Keywords: Diabetes Type 1, Celiac disease, Screening

Infantile colic

Bahar Allahverdi Children Medical Center Hospital, Tehran University of Medical Science Infantile colic, is it a dilemma? Acute & unexpected abdominal pain is known as colic. Infantile colic is a syndrome characterized by self limited clusters of behaviors in the 1st 3 months of life presumed to be secondary to underlying GI disturbance. Colic is a painful condition and our understanding of its etiology, pathophysiology and treatment is far from complete. In 30% of cases persists into the 4th and 5th months of life. Crying is the core symptom and according to Wessel, episodes of crying and abdominal pain occurring more than

3 hours daily, more than 3 times a week and more than 3 weeks should be referred to as infantile colic. Crying has age-dependent and diurnal characteristics. Increased crying typically begins at about 2 weeks after birth, reaching a peak some time in the 2nd month and declines to baseline levels by about 4 months of age. This crying is not random but tends to cluster in the late afternoon & evening hours. Crying occurs in prolonged bouts that are resistant to soothing, even with feeding. Colicky infants clench their fists, flex their legs over abdomen, arch their backs & have an active & grimacing face “pain facies, hard and distended abdomen, regurgitation & flatulence. Most cases of colic occur in the absence of detectable disease in the infant or the parent. Organic diseases are found or associated with colic in about 5% of cases. Diverse etiologies had been traditionally proposed as causes of colic. Cow’s milk protein allergy, microfloral disturbance, gut neurotransmitter imbalance, reflux esophagitis intestinal nervous system immaturity should be in mind. Facing with an irritable, excessively crying infant, one should exclude important organic causes such as CNS abnormalities and increased intracranial pressure, Congenital glaucoma, “Shaken Baby” Syndrome, urinary tract infection. With a novel approach to infantile colic, different treatment options are being considered. Closely observing a colicky infant regarding his/her physical growth and development and some well known non-medical calming behaviours may suffice for most cases. Eliminating cow's milk protein from maternal diet, treatment of GERD and recently appreciated interventions with probiotics should be considered for each individual case with great caution. Keywords: colic, etiology, treatment

Study of factors affecting resolution of

urinary tract infection following treatment of constipation in Iranian children visited in

tertiary referral hospital

Farid Imanzadeh1, Ali-Akbar Sayyari2, Mostafa Sharifian3, Hazhir Javaherizadeh4, Parisa Aghasi5, Amir Imanzadeh6

1,2,3,5Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences.4Ahvaz Jundishapur

University of Medical Sciences. 6Children Medical Center Hospital,Tehran University Of Medical Sciences

The aim of this study was to evaluate effect of sex, age, number of urinary tract infection and duration of constipation on resolution of urinary tract infection following treatment of constipation in Iranian children referred to pediatric gastroenterology and pediatric nephrology clinics. Methods: This study was conducted in Mofid children’s hospital. Duration of study was from 1st October 2010 till 31 June 2011. In this study every child who visited due to urinary tract infection or constipation were asked about other condition. Data was anaylsed by SPSS ver 16.0(Chicago, IL,USA). Chi-square and t-test were used for comparison. Findings: In this study, 550 cases visited the hospital due to urinary tract infection or constipation. Finally 380 cases with constipation and UTI were detected. Among 220 cases with > 2 times of UTI, 90(40.90%) cases showed UTI resolution after treatment of constipation. Of 160 cases with <= 2 UTI, 85(53.12%) cases showed UTI


improvement following treatment of constipation (P=0.018, Chi-Square). In less than 1 yr, treatment of UTI after treatment of constipation was more effective than other age (p=0.000). This result showed that there was no significant difference among males and females. In the children with constipation duration less than 6 months, the urinary tract infection has resolved in most of the cases after treatment of constipation (p<0.001). Conclusion: UTI resolution after treatment of constipation was higher in children < 1yr. If patient had a history of more than 2 times of UTI, improvement of UTI following treatment of constipation will be decreased. Keywords: constipation, urinary tract infection, UTI, children

Concurrence of autoimmune hepatitis and Celiac disease in children of 6mo-18yr in

Children Medical Center in1390-1391

M Najafi Sani, M Sabaghian, N Sadjadei Children Medical Center, Tehran University of Medical Sciences Background: Celiac disease(CD) is an immune-mediated systemic disorder elicited by gluten and related prolamins in genetically susceptible individuals and characterized by the presence of a variable combination of gluten-dependent clinical manifestations, CD-specific antibodies, HLA-DQ2 or HLA-DQ8 haplotypes,and enteropathy. CD-specific antibodies comprise autoantibodies against TG2,endoplasmic antibodies (EMA) and antibodies against deamidated forms of gliadin peptides(DGP). In untreated CD, the characteristic abnormalities in the small bowel mucosa are villus atrophy, crypt hyperplasia and an increase density of inflammatory cells in the epithelium and lamina propria. Hepatic changes in CD are variable including: -Asymptomatic elevation of liver enzyme levels, -Nonalchoholic fatty liver disease, Autoimmune hepatobiliary disorders, Autoimmune hepatitis and cholangitis, Primary sclerosing cholangitis, Primary biliary cirrhosis. Autoimmune hepatitis (AIH) refers to a hepatic disorder not directly due to viral infection, inherited or acquired metabolic disorders or drug –induced liver injury, and whose laboratory features (circulating autoantibodies and hypergammaglobulinemia) suggest an immune etiology. There are associations between autoimmune disorders and in the same patient there may be more than one autoimmune disease. Methods: Our study was a case series study. In 64 patients with AIH, serum IgA level and TG2 antibody were measured, and if TG2 was elevated, 4 biopsies of duodenome were obtained and if the histology was compatible with CD, the patient was known as having CD and AIH. On the other hand in 32 patients with known CD, AST, ALT, ANA, ASMA and anti LKM1 were measured and if any of these markers was abnormal, a liver biopsy was done and if it was compatible with histologic findings of AIH, the patient was known as having AIH and CD. Findings: In our study 4 of 32 (12.5%) patient with CD had high levels of aminotransferases and biopsy of liver confirmed that they have AIH too, and 3 of 64 (4.7%) patients with AIH had high TG2 antibody with normal level of serum IgA and after upper GI endoscopy and examining the duodenal biopsies it was confirmed that they have CD too. Conclusion:Diagnosis of concurrence of CD and AIH is very important. Both of the two disease have a

lot of symptoms and complications. Nearly all of the complications of CD are preventable by a gluten-free diet .Control of AIH is possible with immunosupression. So it is very important too search for AIH in CD and CD in AIH, because treating the concurrent disease could prevent the complications. Keywords:celiac disease, autoimmune hepatitis, concurrence

Severe anemia initial presentation of

Helicobacter Pylori infection in adolescences Shahsanam Gheibi, Sasan Hejazi, Mehran Noroozi, Ahmad Jamei Khosroshahi Department of Pediatric, Urmia University of Medical Sciences Background: Helicobacter pylori is the most common infection worldwide and anemia is a widespread public health problem with major consequences for human health as well as social and economic development. Recent studies have suggested an association of Helicobacter pylori and iron-deficiency anemia. Case reports: We report four adolescences (two males, two females) with persistent severe iron-deficiency anemia associated H. Pylori gastritis. Age range were 13/87 years old and admitted with chief complaints of abdominal and chest pain, weakness, headache, respiratory distress. Range of hemoglobin in cases were 6.2mg/dl with resistent to iron therapy. After the diagnosis and therapy of H. pylori infection, clinical complaints, hemoglobin level and iron profile were being normal and their weight were increased. Conclusion: This study suggests that the screening for H. pylori should be extended to cover those adolescences with moderate or severe anemia especially with clinical manifestations of gastrointestinal tract. Keywords: Helicobacter pylori, anemia, iron deficiency, adolescence Diagnosis of Helicobacter pylori infection in intellectually disabled children using stool

antigen immunoassay Masoumeh Douraghi,Mahmoud Nateghi Rostami, Hossein Goudarzi, Zohreh Ghalavand School of Public Health, Tehran University of Medical Sciences Background: Several diagnostic methods are commonly used for diagnosis of Helicobacter pylori infection. The non-invasive tools are preferred for assessment of H.pylori infection particularly in children. To avoid the risk of endoscopy as an invasive procedure, we detected H.pylori infection by both H.pylori stool antigen test and serological IgG antibody against H.pylori in children with intellectual disabilities (IDs). The performance of H.pylori stool antigen test which is determinant of active infection was also evaluated using IgG serology as proxy gold standard. Methods: Eighty seven children with IDs who were permanent resident of long-stay institutes were included. Serum samples were obtained from blood and were stored at -20◦C. Stool samples were collected by assistance of caregivers. Examination of stool samples was performed by the Amplified IDEIA™ HpStAR™ (OXOID, UK). IgG toward H.pylori antigens were measured using ELISA method according to manufacturer’s instructions


Gastroentrology

(IMMUNOLAB GmbH, Germany). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated with 95% confidence intervals (CI). Findings: The H.pylori stool antigen test and anti-H.pylori IgG test revealed that 93.1% (81/87) and 85.1% (74/87) of children were positive for H.pylori infection. Fifteen children (17.24%) showed discordant results. Eleven (12.6%) children who defined positive by stool antigen test tested negative by serology. In addition, 4 children (4.6%) who were IgG seropositive, yielded negative results using stool antigen test. The monoclonal stool antigen test

showed 94.6% sensitivity (95% CI 86.4%-98.2%) whereas the specificity was 15.4% (95% CI 3.4%-43.7%). The PPV and NPV values were 86.4% and 33.3% respectively. Conclusion: H.pylori stool antigen test can be used as very sensitive and easy-to-perform tool for primary diagnosis of H. pylori infection in intellectual disabled children. This procedure does not require needle stick as needed for serology and eliminates the need for invasive methods. However, stool antigen test need to be validated in larger group of such children. Keywords: H.pylori stool antigen test, H.pylori IgG, Intellectual disability

20

Obesity and some related factors among students of elementary schools in Shahryar

city

Leili Salehi , Saeed Solki Alborz University of Medical Sciences Background: Nowadays, obesity among children is rapidly increasing as a public health issue. The early detection of overweight and obesity in children during the school years is an important prevention strategy. The purpose of this study was investigating prevalence of obesity and its relationship with anthropometric measures and lifestyle factors. Methods: This cross sectional study was conducted between October 2010 and June 2011. Samples of students were selected from eight elementary schools in shahryar city through multi-stage cluster sampling. A multi-section questionnaire was used which consisted of three sections including demographic and anthropometric data, the 24 recall and physical activity questionnaires. Findings: Totally, 325 students aged 6-12 years including 49.2% boys and 50.8% girls took part in the study. The mean age of participants was 8.53 ± 1.61. Results reveal that the mean of daily energy intake in obese children compare with healthy weight children was 2381/74± 308.95 versus1564.52± 160.567. Pearson correlation demonstrated a significant relationship between daily energy intake and body mass index in children. The multiple logistic regressions showed that the daily energy intake, duration of night sleep, meal during passive physical activity and parents’ body mass index were significantly related to obesity. While there was not any significant relationship in terms of age, sex, leisure time activities and birth order in this age group of participants. Conclusion: There are various risk factors associated with obesity and overweight in children. Health education programs should be designed based on these factors and implemented to help control of obesity and overweight in children. Keywords: students, obesity, shahryar Implementation of Selected Sections of PD856

By Private Elementary School Canteens

Dennis Glen Ramos, Kristine Marie B Abalos, Ralph Joseph S Cayanan, Cathryn Clare V Gapuz, Alyssa Noevi O Luzadas, Monica Joana C Orig, Zaila Mae L Pausa, Marc Vincent Charles Y Quiroz, Diana Scarlet J Ramos, LeddyGileanne G Ringor, Deanne Rose R Semon, Angelica R Sua, Leizl V Tolentino, Willie Jean L Villorante St.Louis University, School of Nursing, Philippines

Background: School canteens are the most accessible source of food for school children since it is located in their school premise hence most of the students buy their snacks and lunch there. This study aims to determine the implementation of the private school canteens to Presidential Decree 856, particularly on food handling, food preparation and solid waste management.The study was also aimed to determine if there is a significant

difference in the implementation of selected sections of PD 856 by private elementary school canteens along years of operation. Methods: The researchers made use of quantitative research approach specifically Structural Observation Method. The study was conducted October and November 2010 in the different private elementary school canteens located in Baguio City. Twenty–two schools consented to join the study hence total enumeration was done. A Yes or No type of evaluation tool based on PD 856 was used by the researchers. Frequency, percentage and mean were used in the study and to determine the significant difference, T test was utilized. Findings: The findings of the study revealed that private elementary school canteens fully implements selected sections of PD856. There is no significant difference in the implementation of selected sections of PD 856 by private elementary school canteens along years of operation. Conclusion: The researchers concluded that private elementary school canteens meet the terms of selected sections of PD 856. Despite the full implementation, there is a need to continuously do monitoring and surveillance. The years of operation do not influence the implementation if selected sections of PD856 of private elementary school canteens. Researchers recommend that health workers must continue monitoring and reinforcing the implementation of the sanitation code of the Philippines and to conduct similar study using the variables: types of food handlers, with permit, type of school and area setting Keywords: PD 856: Sanitation Code of the Philippines

Feeding Practices of Caregivers of Severely

Underweight Children

Patrick Arvin B Barcarse, RR A Dela Cruz, Charisse P Bucaoto, Afra Joy L Dulay, Joanne M Ferer, Jonesel L Garcia, Sherry Mae Rizza H Gonzales, Cindy Diane D Olas, Mae Faye C Picardel, Eloisa G Radam, Claude Lorraine C Soliven, Friencess M Transfiguration, Banderie F Uddin, Dennis Glen G Ramos, BSN, RN- Research Promoter Louis University, School Of Nursing, Philippines Background: Malnutrition remains a problem in today’s world. Research suggests that feeding practices play a critical role in the development of children’s taste preferences, eating habits, nutrition, and weight status. This study aims to know the extent of feeding practices of caregivers of severely underweight children residing in Baguio and aimed to know if there is significant difference on the feeding practices of caregivers along with gender, educational attainment and number of children in the family. Methods: This is a quantitative study wherein total enumeration was used. Forty one (41) caregivers of the identified severely underweight children were asked to answer a 25- item questionnaire. Data gathering was done from the month of October to November 2011. Collected data was then organized, tabulated and weighed mean, F-test and T-test was utilized in the study. Findings: The results of the study revealed that the feeding practices were practiced very satisfactorily (3.002).There is

Health & Nutrition


Health & Nutrition

no significant difference in the extent of feeding practices of caregivers according to gender (CV=0.06632 < TV=2.30600), number of children (CV=0.5475 < TV=3.885) and educational attainment (CV=0.125 < TV=2.807). Conclusion: The study concludes that caregivers of the severely underweight children of Baguio City used these feeding practices to help improve the weight of their children. Moreover, the study concludes that factors such as gender, education attainment and number of children are not intervening factors in the feeding practices of caregiver of severely underweight children. Therefore, Nutrition counseling should be conducted at regular intervals and to strengthen the responsible parenting movement seminars to spur awareness on the different feeding styles in handling children who are severely underweights. Keywords: feeding practices, severely underweight

Does breastfeeding reduce the likelihood of

adolescence obesity

Motahhareh Golestan, Moneyreh Modares-Mosadegh, Sedighah Akhavan karbasi Department of pediatric, Shahid Sadoughi University of Medical Sciences

Background:The global prevalence of obesity poses such a serious concern that the World Health Organization (WHO) has described it as a “global epidemic.” The increased prevalence of childhood obesity has resulted in an increased prevalence of the comorbidities associated with obesity. Recent literature suggests that the genesis of the problem occurs in the first years of life as feeding patterns are established. Breastfeeding is recognized as the ideal feeding for infants due to its potential for health maintenance and disease prevention. Among the multiple health benefits associated with breastfeeding, a protective effect from obesity has been recognized. Numerous studies and at least 5 meta-analyses and systematic reviews have examined the role of breastfeeding in relation to childhood or later obesity. Not all conclusions are in agreement with regard to the strength of the obesity protective effect of breastfeeding. Our study aimed to examine the relation of breast-feeding and the duration of breast-feeding in infancy, with the prevalence of overweight in pre-adolescence children aged 11-13 years in a population-based survey. Methods: In this cross-sectional study, data were collected on 800 adolescent(400 boys and 400 girls). Children's breast-feeding history and demographics were obtained in interviewer-administered questionnaires of a multistage cluster sample survey of 800 parents of children aged 11-13 years living in Yazd, Iran. Height and weight were measured in the children and BMI determined .Overweight and obesity were defined as body mass index 85th-95th and ≥95th age and sex-specific percentile of the 2000 Centers for Disease Control reference values. Findings: One hundred and four (13.0%) of 800 children were overweight (male 17%, female 8.8%) and 6.5% were obese (male 8.8%, female 4.3%) A total of 783 (97.9%) of the children had been breast fed. There was a markedly lower overweight prevalence among breast-fed than non breast-fed children. Controlling for age and sex, children breast fed for at least 24 months were substantially less likely to be overweight than children breast fed for less than 12 months (OR 0.56, 95% CI 0.31-0.9). A longer

overall duration of breast-feeding was associated significantly with the decreasing prevalence of overweight. Conclusion: This sample of Iranian children in Yazd shows high rates of overweight at young ages. The duration of breast-feeding is inversely related with the prevalence of overweight in pre-adolescent children. Keywords: body mass index, breast-feeding, overweight, epidemiology, pre-adolescent

Assessing the effect of lifestyle modification

intervention in elementary school obese Children

Fatemeh Esfarjani, Marjan Khalafi, Fatemeh Mohammadi, Roshanak Roustaee, Asiyeh Mansour Research Institute, Faculty of Nutrition sciences & Food industries Background: Obesity has increased all over the world and becomes a major health problem. The main causes are genetic, cultural, economical and social status or unhealthy life style. The purpose of study was to assess the effect of lifestyle modification intervention in obese children (2010). Methods: This study was a field trial and was accomplished in a random sample of 7 year-old obese children (n=156) who were selected from 3 area of health centers in Tehran. Children were randomly divided to case (n=70) and control (n=86). The study was designed in four stages. Anthropometric indices and biochemical tests were measured according to standard procedure. Second and third phases consisted of 12 sessions of group training for 6 months for parents in the intervention group. There was no training program and follow up sessions for intervention group in the fourth stage. At the end of every four stages, a questionnaires on demographic characteristics, lifestyle and food frequency (FFQ) was completed by interviewing with children's mothers. Data were analyzed by Spss. Findings: Among 156 children, the majority of them were the first child in their family and were born in winter or fall. 40% of them had the history of breastfeeding for more than 12 months. 44% of children had the history of obesity in their family. Evaluation of anthropometric indices in obese children showed that although weight and height increased significantly in both groups, weight increased more slowly in intervention group. The largest decline in waist and hip circumferences was also seen during 2nd and 3nd stages. Among biochemical indices, only blood triglyceride and cholesterol decreased significantly (p<0.05). Evaluation of food group consumption showed that not only utilization of milk, dairy and nuts food group increased significantly in intervention group but also consumption of breads and cereals, butter, cream, mayonnaise, sugar and confectionery ingredients significantly decreased(p<0.05). Use of oil and fat food group decreased dramatically in case group and this reduction was significant (p<0.05). Watching TV and playing computer games decreased significantly in case group specially during 2nd and 3nd stages. Conclusion: Overall, if obesity interventions are done in longer periods with including all family and school members, weight control and lifestyle modification would be more efficient. It is obvious that Government policy and organization support are also effective ways to control children obesity in order to have a healthy society in the future. Keywords: Obese children, intervention, lifestyle


Health & Nutrition

Docosahexaenoic Acid in Breast Milk Reflects Maternal Fish Intake in Iranian Mothers

Beheshteh Olang, Majid Hajifaraji, Mohamed Atiya Ali1, Sophie Hellstrand, Mohammad Palesh, Ebrahim Azadnyia, Zinat Kamali, Birgitta Strandvik, Agneta Yngve Karolinska Institutet, Stockholm, Sweden

Background:To estimate essential fatty acid (FA) and long-chain polyunsaturated fatty acid (LCPUFA) concentrations in early breast milk (BM) in relation to habitual fish intake. Methods: BM was collected within 72-hours after delivery from consecutively included mothers, 60 in Guilan (coastal) and 60 in Kermanshah (inland) provinces. Mothers were interviewed to complete a food frequency questionnaire. The FA composition was measured with gas chromatography. Findings: Mothers in the coastal area had higher intake of fish/seafood. Consumption of saturated fat was higher in Kermanshah and olive intake was higher in Guilan. High fish/seafood intake was associated with higher docosahexaenoic acid (DHA) and lower arachi-donic acid (AA)/DHA ratio in BM. There were no differences in linoleic and α-linolenic acid concentrations in BM between the provinces. N-3 FA and DHA concentration were significantly higher in Guilan than Kermanshah, but total n-6 FAs and AA did not differ and were high in both provinces. The ratios of total n-6/n-3 and AA/DHA in BM of mothers from Guilan were significantly lower than those in Kermanshah. Conclusion:The LCPUFA status in BM in two Iranian provinces was generally good and DHA was higher and the AA/DHA was significantly lower in mothers with high fish intake. Keywords: essential Fatty Acids, Docosahexaenoic Acid, Arachidonic Acid, Linoleic Acid, Alpha-Linolenic Acid

Underweight or overweight? Which is the weight disorder of Iranian children? the

CASPIAN-III Study Roya Kelishadi1, Mohammad Esmaeil Motlagh2, Ramin Heshmat3, Mostafa Ghorbani4, Gelayol Ardalan5 1Faculty of Medicine & Child Growth and Development Research Center, 1Isfahan University of Medical Sciences. 2Pediatrics Department, Ahvaz Jundishapur University of Medical Sciences & Bureau of Population, Family, and School Health, Ministry of Health and Medical Education. 3Endocrinology& Metabolism Research Institute, Tehran University of Medical Sciences. 4School of Public Health, Tehran University of Medical Sciences. 5School Health Office, Ministry of Health and Medical Education This study aimed to determine the prevalence of weight disorders and cardiometabolic risk factors among Iranian children, as a nationally-representative sample of pediatric in the Middle East and North Africa (MENA). Methods: This national study was part of the third study of the school-based surveillance system entitled CASPIAN-III, was representative of the Iranian students aged 10 to 18 years, living in 23 provinces in Iran. The prevalence of different cardiometabolic risk factors and components of the metabolic syndrome was studied.

Findings: The study participants consisted of 5738 students (2875 girls) with mean age of 2.4 years living in 23 provinces in Iran. 17.4% of participants were 14.7 underweight and 17.7% were overweight or obese. Based on the criteria of the International Diabetes Federation for the pediatric age group, 24.2% of participants had one risk factor, 8.0% had two, 2.1% had three, 0.3% had all the four components of metabolic syndrome. Low HDL-C was the most common component (43.2% among the overweight/obese vs. 34.9% of the normal-weight participants); whereas high blood pressure was the least common component. The prevalence of MetS was 15.4% in the overweight/obese participants, the corresponding figure was 1.8% for the normal-weight students, and 2.5% in the whole population studied. Conclusion: These findings provide evidence-based data on the considerable prevalence of double burden of nutritional disorders as well as the components of metabolic syndrome in Iranian children. The current health programs and educational curriculum focuses on prevention and management of underweight, whereas overweight and its complications should also been considered as a priority. keywords: underweight. Overweight, children, cardiometabolic risk factors, Iran

A longitudinal study of growth curve in

children under 2 years referring to health- therapeutic centers in Amol city

S J Seyedi1, M Hajiahmadi1, R Rezaei2, S Saataz2, S Molokzadeh2, M Hadizadeh3 1Medical Faculty, Babol University of Medical Sciences. 2Nursing Faculty, Mazandaran University of Medical Sciences, Amol. 3Complex of Health & Treatment, Mazandaran University of Medical Sciences, Noor Background: Based on existing researches nearly 43% children under 5 years old don't growthed and nearly 9% have low–weight. The World Health Organization (WHO) released two sets of child growth standards as an international tool for growth assessment. Therefore, this study did search in order to assess the different situations of growth in children under 2 years. Methods: The present descriptive-analytic study has been performed in longitudinal an approach. Among children referred to 14 urban health- therapeutic centers, 280 healthy children were selected using census method, so as to have at least six times referral during a total of two years. Data were collected from household records using a researcher-made questionnaire in which validity and reliability had been previously proven. Data were analyzed. Findings: From a total of 280 participants, 133 cases (47.5%) were males and 147 (52.5%) females. Generally during the referral months, all the three growth parameters including weight, height and head circumference had statistically meaningful difference with children's gender. But growth rate did not show meaningful difference with mothers' age, type of delivery, birth order and breast feeding. The results also revealed that growth different aspects such as weight, height and head circumference were normal in 73.9% of children. Conclusion: Based on findings of the present study, it seems that a constant reference growth curve is not optimum for the whole country. Hence, more specific growth curves can be designed based on different


Health & Nutrition

geographic areas and diverse ethnicity as well as a guideline impart to health-therapeutic centers for being revised and inserted necessary changes every few years. Keywords: growth curve, referral, health-therapeutic centers

Implications of the new WHO Child Growth

Standards in Birjand-Iran

Kokab Namakin, Gholamreza Sharifzadeh, Mahmood Zardast, Zynab khoshmohabat, Mahsa Saboori Birjand University of Medical Science

Background: Diagnosis of malnutrition and estimate of under nutrition among 0-2 year old children of Birjand city-Iran by using WHO growth standard and the National Center for Health statistics (NCHS) references. Methods: This cross–sectional descriptive study conducted on 822 children under 2 years of age by mean z scores for weight-for-age and Height-for-age. Prevalence, underweight, stunting and wasting were calculated by the new WHO growth standards and then compared with NCHS references. Findings: Based on NCHS references, the mean Height for age scores were lower and mean Weight for age scores were almost similar. Prevalence of underweight, stunting and wasting by using WHO standards were 5.2%, 8.2% and 1.9% respectively compared with 6.5%, 37.3% and 1% based on the NCHS references. Conclusions: In compared with NCHS references, WHO standards resulted different mean z score for Height for age and the prevalence of stunting in Birjand city children. Keywords: WHO Growth standards, underweight, stunting, wasting, Birjand, children

The effect of outpatient training practitioners based on strategy of integrated management

of child illness

A Mohsenzadeh Lorestan University of Medical Sciences Background: Current training is mainly in teaching hospital and practitioners are not involved with the clinical follow-up and treatment of patients. Strategy of integrated management of child illness (IMCI) determines the logic approach with an ill child. The aim of this study was to evaluate the impact of outpatient training based on the IMCI strategy on medical internship capacity. Methods: The study subjects had consecutive 2 year internship in Pediatrics department. A pretest was done then the intervention group underwent the intervention. Practitioners visit the 20 ill children every day. In the intervention group, outpatient training was given based on strategy of IMCI including how you are doing with the sick child, signs of immediate threat, assessment, classification, diagnosis, differential diagnosis, treatment and follow-up consultation with the child's mother. All subjects were evaluated with an observation list. Findings: Study results showed that the mean scores of the control group (17/1 )compared to the control group (13/3) had a significantly increased. Statistical analysis showed significant difference between the scores before and after training based on strategy of IMCI . Conclusion: This study showed that outpatient training based on strategy of IMCI increased capabilities in pediatric interns. Keywords: outpatient training, practitioners, strategy of IMCI

24

Effect of vitamin E on chemotherapy-induced mucositis and neutropenia in leukemic

children undergoing bone marrow transplantation

Farzad Shidfar1, Zohre Choreishi1, Masoud Iravani2, Ali Esfahani2, Ardeshir Ghavamzadeh2 1Department of Nutrition, Faculty of Health Science., 2Hematology, Oncology and Bone Marrow Transplantation Research Center, Dr Shariati Hospital. 1,2 Tehran University of Medical Sciences Background: This study was conducted to investigate the effect of oral vitamin E on mucositis and neutropenia in patients with leukemia. Methods: This was a randomized double-blind placebo controlled clinical trial of 60 patients with leukemia (acute lymphoblastic leukemia, acute myelogenous leukemia and chronic myelogenous leukemia) who were consecutive recipients of allogenic bone marrow transplantation (BMT), randomly assigned to receive 400 mg vitamin E twice daily (supplemented group) or placebo (control). The incidence and severity of mucositis and the mean duration of neutropenia were compared. Findings: The mean duration of neutropenia and the incidence of the mucositis between the two groups was the same (P=1.0). The difference between the placebo group and mucositis grade 1 (P=0.31), grade 2 (P=0.25), grade 3 (P=0.93) and grade 4 (P=0.32) was not statistically significant. Moreover the variables of age, sex, BMI and underlying disease had no effect. Conclusion: In this study supplementation with oral vitamin E had no effect on mucositis and neutropenia in patients with leukemia who were recipients of allogenic BMT. More interventional trials are warranted. Keywords: bone marrow transplantation, leukemia, mucositis, neutropenia, vitamin E

Mucoepidermoid carcinoma of the left bronchus in a nine years old boy: A case

report Ali Ghafouri, Abolfazl Shojaiefard, Setareh Mamishi, Mohsen Raisi, Mostafa Ranjbar, Shaheen Talebi Surgery Ward, Dr Shariati Hospital,Tehran University of Medical Sciences Background: Mucoepidermoid carcinoma of the tracheo-bronchial tree is rarely seen in children, and most of them are metastatic. Of the primary variants of mucoepidermoid carcinoma about 10% are malignant. Case report: We herein present a 9-year-old boy with productive cough and recurrent episodes of pneumonia with several medical admissions and hospitalization which initially was thought to be due to more common causes like infectious diseases, foreign body aspiration or cystic fibrosis, however because of progressive symptoms of airways obstruction we performed a thorough and comprehensive workup that eventually leads to the diagnosis after surgery. The report of the histopathology proved it was mucoepidermoid carcinoma of the tracheobronchial tree. Although this rare

tumor is more common in adults than children, however it has more favorable prognosis in children than adults. In the adult population, the overall mortality is slightly less than 30%. The choice of surgical resection is provided by maximum preservation of pulmonary parenchyma. Well-confined local growth and minimal metastatic potential of childhood mucoepidermoid carcinoma makes this purpose accessible by local resection, however the patient in this report required pnumonectomy, because of the extensive damaged and diseased lung. A thorough search in the literature showed that The 31 reported cases of tracheo-bronchial mucoepidermoid carcinoma in pediatric groups were free of tumor or recurrence after surgery with a mean follow-up period of 5.8 years (range, 0.7–21 years). Based on the documented articles and reports it appears that tracheo-bronchial mucoepidermoid carcinoma can be successfully treated by surgical resection in children and adolescents. Keywords: Mucoepidermoid carcinoma, Tracheobronchial tree

Study of in vitro chromosomal sensitivity to gamma irradiation in lymphocytes of

children with acute lymphoblastic leukemia Majid Mahmoodi1, Asghar Ramyar2, Asghar Aghamohammadi3, Hossein Mozdarani4, Cirus Azimi1, Nima Rezaei3, Zahra Safari1, Hassan Abolhassani3 1Cancer Research Center, Cancer Institute. 2Department of Hematology and Oncology of Children's Medical Center. 3Research Center for Immunodeficiencies, Children's Medical Center. 4Department of Medical Genetics, School of Medical Sciences. 1,2,3Tehran University of Medical Sciences. 4Tarbiat Modares University Background: Acute lymphoblastic leukemia (ALL) constitutes a heterogeneous group of diseases characterized by abnormal proliferation and accumulation of immature lymphoblasts arrested at various differentiation stages. Increasing evidence suggests that chromosomal defects are present in these patients. The aim of this study was to investigate the chromosomal radiosensitivity in a group of children with ALL. Methods: To analyze chromosomal radiosensitivity of ALL patients, lymphocytes of 20 patients were cultured followed by exposure to γ-ray irradiation to detect the chromosomal aberrations as an indicator of radiosensitivity. Cells were scored for the number of aberrations (chromatid breaks, chromatid gaps, chromosome breaks, chromosome gaps and chromatid exchanges). Results were compared with healthy children and ataxia telangiectasia (AT) patients as positive controls. Findings: On average, number of aberrations in ALL children was significantly higher than that in healthy controls. We found 65% of children with ALL appeared to be susceptible to in vitro irradiation. Chromosomal radiosensitivity of 35% patients was not different from healthy donors. Children with AT showed the highest degree of lymphocyte radiosensitivity. The results also indicate that there is a good correlation between the two

Hematology & Oncology



assays, G2 and G0, using the same blood sample for both assays. Conclusion: According to the results, we concluded that most of children with ALL are sensitive to ionizing radiation and therefore should be protected from unnecessary diagnostic and therapeutic procedures using ionizing radiation. Key words: acute lymphoblastic leukemia, chromosomal sensitivity, gamma irradiation

Evaluation of cardiac iron deposition of thalassemia major patients using t2 MRI &

comparision with serum ferritin Nasrin Habibian Islamic azad university, Tehran medical branch Background: Thalassemia is an inherited type of anemia. Today with the screening and identification of premarital thalassemia gene carriers and carriers in premarital stage, the number of new cases of thalassemia major is coming down, but about 18000 patients in Iran are still suffering from thalassemia major. Becouse these people need frequent blood transfusions, complicated iron deposition in their bodies occurs. For this reason, regular use of of drugs such as desferal in these patients is essential. One way of measuring iron overload is measuring serum ferritin. Methods: Therefore, in this study we investigated iron deposition in thalassemia major patients using cardiac MRI at ages 5-40 and compared it with serum ferritin. 36 patients with thalassemia major were examined in this study. Findings: In the next stage, t2 MRI for assessment of iron deposition was performed in all people and their heart rate was studied. In addition, ferritin levels were determined in all patients with iron deposition in the heart and were compared with t2MRI. Finally, data were analyzed by using SPSS software. Conclusion: According to Chi-Square test, a significant correlation between serum ferritin levels and average annual blood transfusions in patients with cardiac iron deposions did not exist (P.<0.05) and MRI is better and more noninvasive method for determining iron deposition in these patients. Keywords: thalassemia major, T2 MRI, serum ferritin

Humoral immunity state in survivors of pediatric acute lymphoblastic leukemia:

results from a single institute in Iran Gholamreza Bahoush, Ebrahim Kalantar, Ahmadreza Shamshiri, Soudabeh Hoseiny Tehran University of Medical Sciences Background: Since there are very few studies on the immunodeficiency state of the Iranian survivors of pediatric Acute Lymphoblastic Leukemia (ALL), we conducted this study to determine the prevalence of humoral defects in children with ALL at least one year after completion of chemotherapy. Methods: In this study, antibody titers against vaccine-preventable infectious diseases including mumps, rubeola, rubella, tetanus and diphtheria toxoids, pertussis and poliovirus were measured in 28 survivor of childhood ALL and 29 normal children. Also, immunoglobulins titers for all participants were evaluated.

Findings: In spite of normal serum immunoglobulin levels in all participants, the percentage of children with ALL who had protective titers was markedly lower than that anticipated for immunized controls (P < 0.001). The rate of protective titers for mumps, rubeola, rubella, tetanus and diphtheria toxoids, pertussis and poliovirus were 7.1, 50, 25, 35.7, 10.7, 21.4, and 10.7 percent in patients and 93.1, 93.1, 100, 96.6, 86.2, 82.8, and 100 percent in controls, respectively. Conclusion: The prevalence of humoral immune defects was high among the survivors of pediatric ALL. It appears that these survivors are at risk of developing these bacterial and viral infections and therefore have to be re-vaccinated as required. Keywords: humoral immunity, pediatric ALL survivors, vaccine-preventable infectious diseases, Iran Report of a case of rhabdomyosarcoma with

skin metastasis Shahla Ansari, Saeed yousofian, Ghasem Miri-Aliabad Tehran University of Medical Sciences and Health Services Rhabdomyosarcomas are the most common soft tissue sarcoma in adult and children that accompany with skeletal muscle differentiation. Skin methastasis of rhabdomyosarcomas are unusual and have only been sporadically reported in literature. In this paper we present a case of skin metastasis of rhabdomyosarcoma in a 8-year-old girl that has treated with chemotherapy. Keywords: Rhabdomyosarcom, skin methastasis, chemotherapy, children

Requirements for building up a Pediatric bone marrow transplantation(BMT) Unit,

special point of views for developing countries Mahshid Mehdizadeh Shahid Beheshti University of Medical Sciences Bone marrow transplantation is now the only curative treatment for many hematologic, metabolic and immunologic diseases in children. General agreement exists that the number of BMT units in our country is not sufficient and the demand for new pediatric BMT beds is increasing. When a BMT unit build up project is starting we should consider important differences in pediatric BMT like underlying diseases, donors, early complications(e.g. organ toxicities, viral infections), late complications(growth impairment, dental damage, social integration) and late effects (fertility, secondary maligancies). The minimal requairments for a new BMT unit are collection facilities, Stem cell processing facilities, transfusion service, hygiene Coordinator, accredited HLA testing lab, diagnostic labs for infection surveillance (esp. Viral), chimerism testing and immunologic reconstitution. Single rooms with air filtration and personal shower/toilet and easy access to ICU are neccesary for any pediatric BMT unit. A permanant BMT team including program director (pediatric hemato-onkology/HSCT), senior physicians, specialized nurses, data manager and coordinator are the least persons needed and pediatric psychologist, teachers, pharmacologist, nutritionist and pediatric physical therapist will improve the team. A quality management system to define responsibilities and describe all essential transplant



procedures as well as ensuring continuous education of all staff members on all relevant transplant issues is also neseccary. So we emphasize that children and adolescents have different demands before, during and after HSCT than adult patients undergoing BMT and need specific care by experienced staff. Therefore, we should look for a generous sponsor to make a "center of excellence" but during this time renovating existing units for children based on above informations is principal. Keywords: bone marrow transplantation, pediatric, unit

Evaluation of iron deficiency anemia in

admitted patients of Children's Hospital of Khorramabad in 2008-2011 years

Nader Mohsenzadeh, Azam Mohsenzadeh Lorestan University of Medical Sciences Background: Iron-deficiency anemia is the most common hematological disease during infancy and childhood. Approximately 1%-3% of children 1 to 2 years have iron deficiency and 3% are anemic. Center for Disease Control and the American Academy of Pediatrics advices screening for iron deficiency anemia at risk population including infants 9-12 months, high-risk toddler infants and teenager Girls. The aim of this study was to assess iron deficiency anemia in patients admitted to children's Hospital of Khorramabad in 2008-2011 years. Methods: This cross-sectional study was conducted. All of the patients were studied. If there were CBC Hb<12 g / dl and mcv <80 children were chosen as microcytic hypo-chromic anemia. Samples from the patients were collected. If serum iron was lower than normal, TIBC was higher than normal, ferritin levels were less than normal, and saturated precedent was less than 10%, anemia was diagnosed. Hemoglobin electrophoresis is done for suspected cases to have Thalassemia. Data were collected by questionnaire. All data were statistically analyzed by Spss. Findings: Of 120 patients who were anemic 35/5% were females and 64/5% were males. In terms of age, 52% were between 6 months to a year, 26% aged 2 to 5 years, 10% aged 6 to 10 years and 12% aged 11 to 14 years. Cause of admission included (35%) pneumonia, 25/5% gastroenteritis, 17% febrile convulsion, 6% bronchiolitis, 4% croup, 3/3% of urinary tract infections, 2/2% parasitic diseases (Giardia .....), 2% sepsis and 1% of idiopathic thrombocytopenic and the rest were other causes. 2/2% had thalassemia minor. Keywords: anemia, iron deficiency, thalassemia minor

Clinical view on molecular aspects of HbH

alpha thalassemia

H Bagherian, S Zeinali, MS Fallah Pasture Institute of Iran

Background: Hemoglobinopathies are the most prevalent genetic disorders in the world. In Iran thalassemia prevention has been in effect since 1997. The program focuses mostly on beta-thalassemia. Affected fetuses are aborted with a fear of birth of beta-thalassemia major. There are controversies regarding the clinical severity of alpha-thalassemia. In this regard the question is that what form of HbH disease requires prenatal diagnosis and clinical attention.

Methods: Patients with alpha-thal were referred to for molecular analysis. Techniques such as ARMS PCR, DNA sequencing and MLPA were used to determine the underlying mutation. Findings: Alpha-thalassemia can have different forms like –a/aa, aT a/a a (mild) or - -/aa, - a/- a, - aT /a a, - a/ aT a,, aT a/aT a (severe), - - /- a, - -/aT a (HbH) and - -/- - (Hydrops fetalis). Our 10 year experience shows that deletional types of HbH (- - /- a) has milder clinical outcome compared with a non-deletional (- -/aT a). We have found that not all forms of non-deletional cause severe HbH. Conclusion: Percent of alpha-thalassemia carriers is much less than that of beta-thal. We have found that more than 30% of population of Tehran are carrier of different forms of alpha-thalassemia but among transfusion dependent thal patients (TDTP) very few are affected with HbH diseas compared with beta-thal which has lower carrier rate but higher percent of TDTP. In our recent paper published in NEJM we gave results of our Iranian experience and think that our clinicians have to have a better understanding on the genetic basis of HbH disease. We think clinicians working with patients suffering with anemia have to have a clear understanding of the genetic basis of such anemia and perform research to provide better understanding of the HbH status in Iran. Keywords: HbH disease, alpha thalassemia molecular

Primry Ewing’s sarcoma of the rib: a case

report

Fatemeh Samiee Rad, Fatemeh Talebi Qazvin University of Medical Sciences

Background: Although the costal lesions in the children and adolescents are rare entities but many of them clinically are important and need for comprehensive considering due to neoplastic nature of them. According to previous data in this locus malignant neoplasms are more than popular in comparing with benign kinds. In these age groups, the Ewing’s sarcoma is the most common primary malignant tumor of the chest wall bone. Ewing's sarcoma is an aggressive malignancy and has male predominant. In this paper, we reported a rare new case of Ewing’s sarcoma. Case presentation: A 12-year-old boy was referred in August 2011 to the surgical clinic of the Rajaee Hospital, with a chief complaint of painful swelling in the middle part of the 5 th rib in right side since 5 months ago. The clinical examination revealed a tender mass in previously described location association with fever and malaise. Laboratory investigations shown raise of ESR and LDH level. Plain films displayed a tumor with aggressive nature, with osteolysis, periosteal reaction, periosteal reaction and a soft tissue mass. On MRI studies, the tumor was extended to soft tissue(marrow tissue and vascular channels ). The lesion and associated involved bony and soft tissue were excised. Macroscopically a soft whitish-gray tissue is observed. Cortical destruction associated with a soft tissue component were found. On histopathology examination, there were syncytial sheets of mitotically active small round cells that separated by richly vascularized network. The tumoral cells can not produce osteoid or chondroid matrix. PAS staining shown many amount of cytoplasmic glycogen. On IHC studies, neoplastic cells demonstrated CD99 immunoreactivity. Patient underwent combined therapy including neoadjuvant chemotherapy and radiation



therapy. There were not any findings of disease recurrence for eight months. Conclusion: Although Ewing’s sarcoma of the rib is a rare, with aggresive and malignant nature, in this location it has a favorable outcome compared with other part of body. The main differential diagnosis are osteomyelitis and osteosarcoma. This fact leads to conspicuous delay in treatment. Therefore, for better patient management we need to fast, actual and precise diagnosis and treatment. Keywords: Ewing’s sarcoma, rib

Evaluation of therapeutic effect of

pamidronate on osteoporosis in β-thalassemia major patients

Majid Naderi1, Shaban Alizadeh2, Akbar Dorgalaleh2 1 Department of Pediatrics, Zahedan University of Medical Science. 2Hematology Department, Allied Medical School, Tehran University of Medical Sciences Background: Osteoporosis is a disorder with low bone mass density, architectural deterioration, enhanced bone fragility and increased fracture risk. Patients with β-thalassemia are susceptible to osteoporosis due to several factors include delay in sexual maturation, presence of hypothyroidism, accelerated hemopoiesis and direct iron toxicity on osteoblasts. This study aimed to evaluate the therapeutic effect of pamidronate on osteoporosis in patients with β-thalassemia major. Methods: This 12 month clinical-trial study conducted on 40 women with β-thalassemia major affected by osteoporosis. Twenty patients received Pamidronate monthly as a case group and a similar number of thalassemic patients as a control group without receiving Pamidronate. Finally analyzed of data were done by SPSS software. Findings: With calculation of mean of Z score, BMD lumbar spine and hip in the case and control groups it was revealed there was statistically significant different between these two groups(P<0.05). Also comparison ALP between the case and control groups revealed statistically significant different (P<0.05). Conclusion: According to our study, Pamidronate is an effective treatment to enhance density of bone in patients with β-thalassemia major. Keywords: osteoporosis, Pamidronate, β-thalassemia major

Evaluation of liver and kidney function in

favism patients Bijan Varmaghani, Shaban Alizadeh, Akbar Dorgalaleh, Zahra Kashani Khatib Hematology Department, Allied Medical School, Tehran University of Medical Sciences Background: G6PD deficiency is the most common red cell enzymopathy that is associated with favism as a hemolytic crisis induced by ingestion of fava beans. The common features of favism include anemia, jaundice and hemolysis that the latest in long period seem to affect liver and kidney as two main organs involved in hemolytic crises. Thus the aim of this study was to evaluate kidney and liver function tests in favism patients in the most common area of favism in Iran with high rate of fava bean cultivation.

Methods: This cross-sectional study was performed on 55 patients with favism and 60 non favism patients as control group. Initially after confirming of G6PD deficiency, kidney and liver function tests were performed on patients and healthy individuals. Finally comparison between two groups was done by SPSS software (t-test). Findings: Analysis of obtained results revealed that liver function tests, AST, ALT and ALP in patients' group were statistically significantly higher than control group (P<0.05). In other hand no statistically significant difference were observed in the two groups in kidney function tests, BUN and Creatinin (P>0.05). Conclusion: It is recommended that in favism patients, periodically check up tests for liver function should be considered. Keywords: favism, G6PD deficiency, liver function tests, kidney function tests

Peripheral neuropathy in β-thalassemia major

patients Shaban Alizadeh1, Majid Naderi2, Akbar Dorgalaleh1 1Hematology Department, Allied Medical School, Tehran University of Medical Sciences. 2 Department of Pediatrics, Zahedan University of Medical Science Background: β–thalassemia major is a lifelong transfusion dependent disease. Thalassemic patients suffer from skeletal changes, hepatosplenomegaly, and neuropathy resulted from anemia, iron overload or chelation therapy. This study aimed to determined the prevalence of peripheral neuropathy in patients with β–thalassemia major and also evaluate the effect of other factors such as ferritin level, DFO dose, sex, splenectomy and age on the incidence of this disorder. Methods: This cross-sectional study conducted on 65 patients with β–thalassemia major who had received regular transfusion and DFO chelation therapy at least for ten years. Both sensory and motor conduction studies in the upper and lower limbs of 65 thalassemic patients were done with electromyography/nerve conduction studies. Finally obtained results were analyzed by SPSS software. Findings: From total number of 65 patients, 43(66.15%) persons suffered from a peripheral neuropathy and there was statistically significant relationship between aging and peripheral neuropathy (P<0.001) but there was not any statistically significant relationship with gender, serum ferritin level and splenectomy. Conclusion: According to obtained results, the incidence of peripheral neuropathy in patients with β–thalassemia major can be reduced by regular DFO chelation therapy. Keywords: β–thalassemia major, peripheral neuropathy, DFO Prevalence of Vitamin D deficiency in patients

with ITP admitted in general ward in Ali Asghar Hospital, Tehran, Iran

Zohreh Kalbassi, Hossein Shiri General ward, Ali Asghar hospital, Tehran University of Medical Sciences In past, vitamin D has been thought only to have an effect on calcium metabolism and bone. Recent works have shown that it effect on many other cells and tissues. Different studies have found an effect of vitamin D on



infectious diseases such as TB and autoimmune diseases like MS, IBD, Rheumatoid arthritis, Type I diabetes. The goal of this study was evaluation the prevalence of hypovitaminosis D in ITP patients and influence of its treatment on the prognosis of that. Methods: This was a prospective cross-sectional study, done 2008-2011 in general ward Ali-Asghar hospital. In the patients with ITP who admitted, serum VD level was measured. Patients levels less than 30 ng/ml were treated by IV prescription of sufficient amount of vitamin D. IVIG level was prescribed as well when needed. Findings: There were 50 patients in this study. Mean age was 41/38 (SD=49.16) months. 46% of patients were

females and 54% were males. Mean vitamin D level was 23.99±17.01. Severe deficiency was considered in 12% of the patients. 36% of them had moderated and 26% had mild VD deficiency. As a whole 74% of them had vitamin D deficiency. Mean platelete level before treatment was 12800 (SD=7748.60) which reached 17120 (SD=1102660.03) after treatment with IVIG and it was significant (P<0.0001). Conclusion: This study demonstrated that VD deficiency is very common and its treatment my improve the outcome of ITP and further research is needed in this aspect. Keywords: ITP, vitamin D, Thrombocytopenia, vitamin D deficiency

29

Immunological phenotypes in CVID Klaus Warnatz Division of Rheumatology and Clinical Immunology, Centre of Chronic Immunodeficiency, University Freiburg-Medical Center, Freiburg, Germany Common variable immunodeficiency is a very heterogeneous immunodeficiency disorder. Due to its variable prognosis a better dissection of CVID is important not only for research but also for clinical management. CVID can be dissected on the basis of clinical, genetic and immunological information. Currently the clinical presentation determines treatment decisions, but the definition of predictive parameters preempting the manifestation of serious complications is highly important. Therefore several groups have suggested immunological phenotyping based on characterization of circulating B- and T cells. Thus over 75 percent of CVID patients present with reduced switched memory B cells predictive of a reduced vaccination response and secondary complications. Inflammatory CVID is associated with a complex dysregulation of the immune system including the expansion of CD21low B cells, decrease of naive CD4 and regulatory T cells. While immune phenotyping has not been included into the diagnostic criteria of CVID, there is ample evidence that it helps to dissect subgroups of patients for research into pathogenesis and more importantly for clinical management.

Approach to a child with food allergy Riccardo Troncone Department of Pediatrics & European Laboratory for the Investigation of Food-Induced Diseases, University Federico II, Naples, Italy Food allergy (FA) is defined as an abnormal immunological reaction to dietary proteins that causes an adverse clinical reaction. The prevalence appears to be increasing although reliable, population-based data are limited. FA in children present with a wide spectrum of clinical manifestations, including anaphylaxis, urticaria, angioedema, atopic dermatitis and gastrointestinal symptoms (such as vomiting, diarrhoea and failure to thrive). Gastrointestinal syndromes are becoming increasingly recognised in young infants. Food protein-induced proctocolitis also occurs in the first few months of life but does not result in systemic illness. Infants appear healthy and present with normally formed, blood-streaked stools. Infants with this disorder are sensitive to cow’s milk or soy protein and most are exclusively breastfed. The hematochezia usually resolves within 72 hours of eliminating the allergen including that in the maternal diet for breast-fed infants and the food are generally tolerated by one year of age. Other gastrointestinal disorders such as eosinophilic oesophagitis (EoE), have also been shown to be associated with FA. EoE was first reported in the late 1980s but has gained increasing interest over the last decade. The epidemiology of EoE remains unknown but the incidence in both the pediatric and adult populations

may be increasing. It remains unclear whether the apparent increasing incidence of EoE is real or related to increased awareness and improved detection. However, it is likely that this is a true increase with a corresponding increased burden of eosinophil-related disease such as eczema over the last decade. The characteristic endoscopic appearances, a histological picture with massive accumulation of eosinophils in the esophageal epithelium, normal pH monitoring results, and the ineffectiveness of antireflux treatment separate EoE from gastroesophageal reflux disease. Correct diagnosis of FA is crucial to ensure appropriate patient care. The essential criterion is a clear response to elimination diet, and other diagnostic tests are secondary to this. Oral food challenge play a crucial role in the diagnosis of FA, but it is largely underutilized even by allergy specialists. Potential responsible factors contributing to the lack of a correct diagnostic work-up in the vast majority of cases in our study population could be numerous and include lack of training on the procedure, increased reliability on screening methods (skin testing, in vitro tests), extensive time needed, fear of risk, and suboptimal fee reimbursement. The issue is pressing because a large number of suspected FA diagnoses could be incorrect leading to unnecessary elimination diets, economic and social costs.

Clinical challenges in CVID Klaus Warnatz Division of Rheumatology and Clinical Immunology, Centre of Chronic Immunodeficiency, University Freiburg-Medical Center, Freiburg, Germany The heterogeneity of the clinical presentation of Common variable immunodeficiency (CVID) is associated with a clear variance of prognosis. Therefore, the clinical presentation determines treatment decisions. Several large cohort surveys have demonstrated that CVID patients manifesting only with infection have a better prognosis than patients having any additional complication like inflammatory disease, granulomatous disease, lymphoproliferation or autoimmunity. The most relevant prognostic factors are inflammatory lung, liver and gut disease and lymphoma. Clinical trials need to develop predictive parameter of these complications for allowing preemptive interventions. Optimal treatment for interstitial lung disease, gut disease and liver disease has not been sufficiently addressed and require a better understanding of the underlying pathogenesis. For some of our patients potentially stem cell transplantation needs to be considered to cure underlying combined immunodeficiency.

Approach to a child with asthma Mostafa Moin Immunology, Asthma & Allergy Research Institute (IAARI), Tehran University of Medical Sciences, Thean, Iran Asthma is a complex and common chronic inflammatory disorder of the airways that has multiple genetic, environmental and lifestyle related risk factors and triggers regarding etiology, pathogenesis, clinical manifestations

Clinical Immunology, Allergy and Immunodeficiencies


Immunology and Allergy

and response to treatment. Diagnosis and treatment of asthma due to the high and increasing prevalence & burden (in Iran and globally), obscure natural history, chronicity, acute exacerbations, unpredictability and variable clinical manifestations remain significant and challenging. A high index of suspicion and a full history is a prerequisite for asthma diagnosis. Paraclinical workups and tests only support clinical impression and objective evidence for therapeutic interventions. The goals of therapy are developed by the physician, the family and the child as a team. For managing asthma, the severity assessed to initiate therapy accordingly and the control of signs and symptoms assessed periodically to adjust therapy to prevent impairment of the quality of life of patients and risks of future asthma attacks. A stepwise approach is used to prescribe medications to control asthma and at the same time attention to co-morbidities as allergic rhinitis, sinusitis and gastro-esophageal reflux diseases, patient adherence to medication, inhaler technique, and environmental control measures including allergen and tobacco smoke avoidance are regularly assessed. Educating children with asthma and their families on the disease and proper treatment is emphasized in the management of asthma. In patients with persistent asthma both long-term control medication to control symptoms and quick-relief medication to prevent exacerbations are needed. Inhaled corticosteroids are the preferred long-term control medication across all age groups. Other medications as theoplylline, cromoglycate, Ipratropium and newer classes of medications including leukotriene modifiers, long-acting beta-adrenergic agonists, and anti-IgE are also recommended in a stepwise approach to control asthma according to the international guidelines for the diagnosis and management of asthma. In this article the key & Practical points in diagnosis, treatment and prevention of pediatric asthma will be addressed.

Approach to a child with congenital neutropenia

Nima Rezaei Research Center for Immunodeficiencies, Children's Medical Center and Molecular Immunology Research Center, Tehran University of Medical Sciences, Thean, Iran Neutropenia, as the most common phagocyte defects, is a reduction of the absolute neutrophil. Congenital neutropenia could be associated with variety of primary immunodeficiency diseases (PIDs), including oculocutaneous hypopigmentation, pancreatic insufficiency, combined immunodeficiency, metabolic disease, congenital heart disease, and bone marrow aplasia/infiltration. These disorders consist of several genetic diseases ranging from isolated form of neutropenia such as severe congenital neutropenia and cyclic neutropenia to complex inherited disorders associating neutropenia. Chediak-Higashi syndrome, Griscelli syndrome type 2, Hermansky-Pudlak syndrome type 2, and p14 deficiency are a group of PIDs with oculocutaneous hypopigmentation that could be associated with neutropenia, while Shwachman-Diamond syndrome and WHIM syndrome could be associated with exocrine pancreatic insufficiency and warts respectively. Immunoglobulin class switch recombination deficiency due to CD40 ligand mutation is usually characterized by hypogammaglobulinemia and increased or normal IgM level but sometimes present with neutropenia. Cartilage

hair hypoplasia, glycogen storage disease Ib, Barth syndrome, dyskeratosis congenital, and Cohen syndrome are some other diseases which could be associated with neutropenia. Keywords: Congenital Neutropenia, infection

Primary hypogammaglobulinemia in children: The outcome and predictors

A Ozen, S Baris, E Karakoc-Aydiner, C Ozdemir, NN Bahceciler, IB Barlan Department of Pediatric Allergy and Immunology, Marmara University, Istanbul, Turkey We evaluated 131 children (M = 88, F = 43) with hypogammaglobulinemia. Data was analyzed mainly for delineating predictor factors for outcome.The distance from the lower limit of normal (− 2SD) for any single measurement of immunoglobulins (Ig) was calculated and transformed into Ig scores. Mean age and duration of follow-up were 5.06 ± 4.05 and 3.7 ± 3.03 years, respectively. The diagnoses were 22 CVID, 16 IgA deficiency, 33 transient hypogammaglobulinemia of childhood (THC), 3 selective IgM deficiency and 57 unclassified hypogammaglobulinemia (UCH). Low IgA scores (<−0.124) at presentation were indicative of subsequent development of IgA deficiency or CVID, whereas low IgM score (<−0.038) pointed towards more severe and persistent phenotypes. Combination of low IgM score between 2 and 5 years, impaired antibody response and low B cell counts enabled us to predict persistence of hypogammaglobulinemia beyond 5 years specificity = 90.5% and PPV = 94.9%) and chronic lung disease (sensitivity = 90.4% and specificity = 68.3%). The set of criteria including low IgM scores, impaired antibody response and low B cell counts provided ahigh predictive value in detecting those with persistent hypogammaglobulinemia.

Immunomodulatory effects of probiotics in the management of lung diseases

Esmaeil Mortaz Division of Pharmacology, Utrecht Institute for Pharmaceutical Sciences, Faculty of Science, Utrecht University, Utrecht, The Netherlands Chronic Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Disease (NRITLD), Masih Daneshvari Hospital, Shahid Beheshti University of Medical Sciences, Thean, Iran Common physiology and pathology of the respiratory and gastrointestinal tracts has been shown. Structurally, the respiratory and gastrointestinal systems show many similarities such as highly vascular systems, surface area and epithelial barrier cells and an overlying mucus layer which protects from bacterial pathogens and foreign antigens. This similarity could be due to a shared common embryonic origin. This similarity between the two organs may underlie why dysfunction in one organ may induce illness in the other. For example, smoking is a major risk factor for COPD and IBD and increases the risk of developing Crohn’s disease threefold. Thus, the respiratory and gastrointestinal systems could have similar etiology and therapeutic approaches. Probiotics have shown promising results in regulating innate and inflammatory



immune responses. Probiotics have been defined as ‘live micro-organisms which, when administered in adequate amounts, confer health benefits on the host’ Commonly used probiotics include lactic acid bacteria, particularly Lactobacillus, Bifidobacterium and Saccharomyces and these are often used to supplement dietary products to provide a health benefit to the host. It has been suggested that probiotics confer benefit as a therapeutic approach in diseases involving the gastrointestinal tract (e.g., infections, inflammatory bowel disease, and colon cancer). In this respect, the role of probiotics as immunomodulatory agents and activators of host defense pathways may represent an important factor for their therapeutic use. There is increasing evidence that orally delivered probiotics are able to regulate immune responses outside the gastrointestinal tract including the respiratory system.

Immunologic markers in patients with with β-Thalassemia

Akefeh Ahmadiafshar, Amir Ghadi pasha, Abdolreza Esmailzadeh, Kambiz Davari, Noureddin Mousavinasab Zanjan University of Medical Sciences, Zanjan, Iran Background: Several studies demonstrated some alterations in immune system of β-thalassemia major patients. The aim of this study was to assess the immunologic markers of these patients in comparison with control group. Methods: Immunologic markers including CD8, CD4 [T-lymphocyte],CD19 [B-lymphocyte], and CD56 [NK cell] were assessed in thirty patients with β-thalassemia major (18 male and 12 female; under 18 years) and similar age and sex matched healthy controls. All patients had no infectious, malignant or chronic diseases. Statistical analysis performed by SPSS (v.15) software. Findings: We did not found any abnormality in cellular and humoral system. However, mean CD56 level in thalassemia group were significantly lower than control group (6.54±2.87% vs. 9.13±4.01%; p=0.006). Mean CD4 in thalassemia patients with spelenectomy was significantly lower than patients without splenectomy (31.8±6.55% vs. 40.3±9.2%; p=0.02). Conclusion: NK cell marker in the patients with β-thalassemia major is lower than healthy individuals, that may be responsible for defects in innate immune system. Keywords: β-thalassemia , immunologic markers

Molecular basis of autosomal recessive chronic granulomatous disease in Iran

Shahram Teimourian Genetic Department, Tehran University of Medical Sciences, Thean, Iran Background: Chronic granulomatous disease (CGD) is a rare inherited condition resulting from mutations in the genes that encode the proteins of the NADPH oxidase enzyme in phagocytes, rendering these cells incapable of killing invading pathogens. Methods: Patients subtypes are determined by neutrophil functional assays and immunoblotting. Although defects in the X-chromosome-linked gp91-phox component account for the majority of CGD patients in the world, in Iran there are many CGD patients suffering from the autosomal recessive forms of the disease. Most of these patients show

impairment in the synthesis of the 47-kDa cytosolic component p47-phox of the oxidase. The second causative factor of autosomal recessive CGD is deficiency of the 22-kDa component (p22-phox) of the oxidase. Another rare form of the disease is due to mutations in the NCF2 gene encoding the 67-kDa component (p67-phox) of the oxidase. Findings: Mutation analysis showed a novel homozygous splice site mutation, c.intron4+1G>T, in CYBA. A novel mutation in NCF2: a gross homozygous deletion of exon 1 and 2, causing p.Met1_Lys58 deletion in p67-phox. We also found a previously published homozygous nonsense mutation, c.196C>T, causing p.Arg66X.33 in p67-phox. Conclusion: Our data show that CGD in Iran is predominantly due to mutations in p47-phox, while the number of mutations in p22-phox is roughly equal to that in gp91-phox. These data indicate that the genetics of CGD are ethnically variable, and this should be considered in approaching families with CGD. Keywords: CGD, molecular diagnosis

Mendelian susceptibility to mycobacterial disease: A single center experience

Seyed Alireza Mahdaviani Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran MSMD is a rare congenital syndrome characterized by the occurrence of severe, often disseminated clinical disease, caused by weakly virulent mycobacterial species, such as BCG vaccines and nontuberculous environmental mycobacteria, in otherwise healthy individuals. Understandably, patients with MSMD are also susceptible to the more virulent species, Mycobacterium tuberculosis. Severe disease caused by nontyphoidal and, to a lesser extent, typhoidal Salmonella serotypes is also common, observed in nearly half of the reported cases, including patients who did not have any mycobacterial disease before the diagnosis of salmonellosis, or even at last follow-up. Mutations have been identified in the following autosomal genes involved in the IL-12/IL-23/IFN-γ axis: IFNGR1 and IFNGR2 (encoding IFN-γR1 and IFN-γR2, respectively), STAT1 (encoding Stat-1), IL12P40 (encoding the IL-12p40 subunit), IL12RB1 (encoding the IL-12Rβ1 chain) and TYK2 (encoding tyrosine kinase 2). In addition an X-linked genetic trait has been identified affecting NEMO, encoding nuclear factor-κB-essential modulator (NEMO). Mutation in the IL12RB1 gene encoding the IL12Rb1 chain is the most common genetic etiology of MSMD. We reported 3 cases of such patients. The first is two years old child suffering from complete IFN-G receptor 2 deficiency and disseminated BCG infection. The other, a 28-year-old adult, suffers from IL- 12p40 deficiency and presented with disseminated BCG infection followed by recurrent episodes of systemic salmonellosis. The third patient is a 33-year-old man was admitted in hospital due to fever, generalized lymphadenopathy, and hepatosplenomegaly and past history of anti-tuberculosis treatment in the previous 3 years. A sputum sample was smear-positive for acid-fast bacilli, and polymerase chain reaction was positive for Mycobacterium tuberculosis complex. Evaluation of the patient’s immune system revealed IL-12Rβ1 deficiency. T his is the first IL-12



receptor-deficient patient presenting with disseminated TB in adulthood, without any previous relevant medical history.

A study of atopy frequency in patients with anticonvulsant drug hypersensitivity and

control group Zahra Chavoshzadeh, Shima Ataei, Hasan Tonkaboni, Fatemeh Abdolah Gorji, Maryam Moshiri Mofid Hospital, Thean, Iran Adverse drug reactions are frequent and occur in 10-20% of hospitalized patients and approximately 7% of general population. Antibiotics and antiepileptic are the most frequently responsible drugs. As we know atopy defined as the genetic predisposition to IgE response to antigen is not normally associated with higher risk of drug hypersensitivity. We proposed this study for evaluation of atopy in anticonvulsant drug reaction. Methods: In this case-control study, there were 100 pediatric patients who had early or late allergic reaction to anticonvulsant drugs during the latest 3 years and 100 control patient who used anticonvulsant drug without any drug reaction. The tool of study was questionnaire which was designed according to the ISSAC questionnaire for atopic dermatitis, asthma and allergic rhinitis. SPSS software was applied for analyzing data. Findings: 35% from total 100 patients who had experienced adverse anticonvulsant drug reaction including TEN, SJS and DRESS had history of atopy and 16% from total 100 control group had atopy (P-value=0.003). There was family history of atopy in 38% case and in 18% control groups (P-value=0.003). Conclusion: With respect to the high atopy rate in our pediatric patients who had reaction to anticonvulsant drugs and their families, we suggest to do this study in bigger sample for finding significant association between atopy and anticonvulsant drug reaction. Keywords: atopy, adverse drug reaction

Serum level of zinc in asthmatic patients; A case-control study

Ghamartaj Khanbabaee1, Ali Omidian1, Nima Rezaei2 Mofid Children Hospital, Shahid Beheshti Medical University, Thean, Iran Children’s Medical Center, Tehran University of Medical Sciences, Thean, Iran Background: Zinc deficiency could lead to a variety of defects in growth and the immune system, while it seems to be associated with increased rate of asthmatic attacks in children. Methods: This study was performed to assess the serum zinc level in 100 pediatric asthmatic patients in comparison with a control group. Findings: Mean serum level of zinc in the asthmatic patients was 70.5±22.6 µg/dL, which was significantly lower than 80.9±16.9 µg/dL in the control group (p<0.001). Forty two asthmatic patients (42%) had hypozincemia, while this rate was 12% in healthy children (p<0.001). There was a significant association between the zinc level and severity of asthma (p<0.001). However, no significant association was detected between the serum level of zinc

and other factors, including control and treatment of the disease. Conclusion: As of high rate of hypozincemia in the asthmatic children, evaluation of serum zinc level in asthmatic children could be suggested, while zinc substitution in the diet of those with zinc deficiency could be recommended. Keywords: asthma, zinc, children TNF-alpha single nucleotide polymorphisms

in atopic dermatitis Nasrin Behniafard1 , Mohammad Gharagozlou1, Elham Farhadi2,3, Mojdeh Khaledi4, Soheila Sotoudeh1, Behzad Darabi1, Seid Mohammad Fathi1, Zahra Gholizadeh Moghaddam1, Mahdi Mahmoudi5, Asghar Aghamohammadi1,6, Ali Akbar Amirzargar2, Nima Rezaei1,2,6 1Pediatrics Center of Excellence, Children's Medical Center. 2Molecular Immunology Research Center; and Department of Immunology, School of Medicine 3Hematology Department, School of Allied Medical Science. 4Growth and Development Research Center 5Rheumatology Research Center 6Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center. 1,2,3,4,5,6Tehran University of Medical Sciences, Tehran, Iran Tumor necrosis factor-alpha (TNF-α) could be considered as potential biomarkers in atopic dermatitis (AD), while their levels could be influenced by cytokine single gene polymorphisms (SNP). This study was performed in 89 pediatric patients with AD and 137 controls to assess polymorphisms of TNF-α gene at positions -308 and -238, using polymerase chain reaction with sequence-specific primers method. The highest positive allelic association that makes the patients susceptible for AD was seen for TNF-α -238/G (p<0.001) and TNF-α -308/G (p=0.003). The GG genotypes at TNF-α -238 and TNF-α -308 were both significantly higher in the patients with AD compared to the controls (p<0.01). The GG haplotype at TNF-α (-308,-238) was seen in 92.7% of the patients, which was significantly higher than the controls (p<0.001), while the haplotypic negative association for AD was seen in TNF-α (-308, -238) AG and GA (p<0.01). This study showed that AG genotype of TNF-α -308, associated with high production of cytokines, was significantly decreased in the patients with AD, while the low-producing GG genotype was over-expressed in the atopic patients, which could lead to low production of TNF-α in the atopic patients. Gene polymorphisms of tumor necrosis factor

and interleukin-1 gene complex in allergic rhinitis

Rasoul Nasiri, Ali Akbar Amirzargar, Masoud Movahedi, Nima Rezaei, Armin Hirbod Mobarakeh Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Background: Allergic rhinitis is a complex polygenic disorder of the upper respiratory airway. As proinflammatory cytokine, including tumor necrosis factor



(TNF) and interleukin-1 (IL1) super family, seem to have a role in development of allergic rhinitis, this study we performed to evaluate the associations of several variants of TNF and IL1 genes. Methods: Ninety eight patients with allergic rhinitis were enrolled in the study. Genotyping was done for two TNF-Α promoter variants (rs1800629 and rs361525), one variant in promoter region of IL1α (rs1800587), two SNPs in IL1β gene (rs16944 and rs1143634), one in IL1 receptor (rs2234650) and IL1RA (rs315952), using PCR sequence-specific-primers method. Findings: The patients homozygous for the T allele of rs16944 in IL1 β had an 8.1-fold risk of having allergic rhinitis than those with the C allele. In TNF-Α, there was also a significant relationship between two SNPs of rs1800629 and rs361525 and having allergic rhinitis. Except rs1800587, in IL1α, and rs315952 in IL1RA, there were significant difference between control group and patient group for all other SNPs. Some SNPs were associated with the nature of sensitivity and severity of disease. Conclusion: We found that genetic variants in TNF-Α and IL-1 gene complex not only were associated with the risk of developing allergic rhinitis, but also affected the course of disease and its severity. Keywords: allergic rhinitis, genetic susceptibility, single nucleotide polymorphism, proinflammatory cytokines, Tumor necrosis factor, Interleukin 1 family

Decreased circulating CD4⁺ ⁺ ⁺CD25 Foxp3 T cells in patients with chronic autoimmune

and chronic idiopathic urticaria

Saba Arshi, Delara Babaie, Mohammad Nabavi, Majid Tebianian, Babak Ghalehbaghi, Farhad Jalali, Alireza Ahmadvand, Reza Gholami Rasoul-e-Akram Hospital, Tehran University of Medical Sciences, Thean, Iran Patients with chronic ordinary urticaria (CU) are divided into two groups: 40-50% has chronic autoimmune urticaria (CAU) and the remainders have chronic idiopathic urticaria (CIU). CD4+CD25+ regulatory T (Treg) cells play critical roles in maintaining peripheral tolerance and preventing autoimmunity, but the characteristics of Treg cells have not yet been defined in CU. To identify whether CD4+CD25+ Foxp3+ T cells play an important immunoregulatory role in the etiology of CU, we determined the frequencies of circulating CD4+CD25+ FOXP3+ T cells, and serum level of IL-10, TGFβ, and IL-17 in CU patients compared with healthy control subjects. Peripheral blood mononuclear cells (PBMCs) were obtained from CU patients and healthy controls. The frequency of CD4+CD25+ T cells in PBMCs and expression levels of forkhead box P3 (FOXP3) was detected by flow cytometery. The serum level of IL-10, TGFβ and IL-17 were measured by ELISA. A decreased percentage of Treg cells was observed in CU patients compared to control subjects. It was also detected in CAU as well as CIU patients compared to their respective control. No significant difference between the serum levels of IL-10, TGFβ, and IL-17 in CU patients and control subjects. Our data demonstrates that the frequency of CD4+CD25+ FOXP3+ T cells in PBMCs was decreased in CU patients. Further studies are needed to clarify the exact role of Treg cells in the pathogenesis of CU and identify factors regulating their function in CU.

Keywords: Treg; chronic urticaria; IL-10; IL-17; TGFβ

Diffuse candidia esophagitis in a patient with agammaglobulinemia

Tooba Momen, Sepideh Darougar Shiraz University of Medical Sciences, Shiraz, Iran Infections with opportunistic organisms including fungal infections are very rare in agammaglobulinemic states. This report illustrates a 12 y/o boy with agammaglobulinemia and diffuse candidia esophagitis. The case is a 12 year old boy who referred to our center with recurrent episodes of pneumonia. He did not have tonsils and palpable lymph nodes in physical exam. He had 3 healthy sisters and a history of 2 sibling deaths (both male) due to encephalitis in one and also poliomyelitis and pneumonia in the other one. So the patient evaluated for immune deficiencies. He had undetectable lmmunoglobulins (IgG<1.95g/L; IgM<0.24g/L; IgA<0.36g/L; IgE<0.1IU/ml) and absence of antibody responses to tetanus and hepatitis B antigens. In flowcytometry CD19 and CD20 B cells were both absent (0%) with normal CD3, CD4, CD8, CD4/CD8 T cells. The patient was diagnosed as agammaglobulinemia and intravenous immunoglobulin was started. On admission, antibiotic therapy was initiated for pneumonia and pleural effusion but his fever continued. Meanwhile, he had also developed severe thrush in the mouth. He had also prolonged diarrhea which lasted for 3 weeks. Stool exam was negative for cryptosporidiosis. An upper GI endoscopy showed diffuse whitish exudate in total surface of esophagus with a pathology report of candida esophagitis. Amphotericin was started for him and after that his gastrointestinal symptoms(including diarrhea, thrush ) and also fever were subsided. Conclusion: Humoral immunedeficiencies are more prone to encapsulated organisms and some viruses, however fungal infections rarely may occur in these patients.

Primary immunodeficiency in northwestern

Iran Mahnaz Sadeghi shabestari TB and Lung Research Center of Tabriz, Children’s Hospital, Tabriz University of Medical Sciences, Tehran, Iran

Primary immunodeficiency disorders are heterogeneous group of illnesses that predispose patients to infection and serious complications. Fifty seven patients with primary immunodeficiency were registered from 2007 to 2012 at the Pediatric Allergy, Immunology Department, Children’s Hospital, Tabriz University of Medical Sciences. Combined T and B cell immunodeficiencies were the most common form of PID in this region including severe combined immunodeficiency (33 %) followed by common variable immunodeficiency (21%) and other well defined immunodeficiencies (17.5%). The patients had a wide range of clinical features but recurrent infections, particularly in the upper and lower respiratory tracts were the most common manifestations. Although antibody deficiency is the most common primary immunodeficiency, frequency of combined T- and B-cell immunodeficiencies in our cohort is relatively higher in this region.



Keywords: frequency, primary immunodeficiency diseases, turk ethnic

Chediak-Higashi syndrome: A report three cases from the north of Iran with different

presentations J Ghaffari, H Karami, MS Rezaei, S Abediannkenari Department of pediatrics; and department of microbiology and immunology, Mazandaran University of Medical Sciences, Sari, Iran Chediak-higashi syndrome (CHS), an autosomal recessive is a rare disorder with clinical symptoms including occulo-cutaneous albinism, bleeding due to platelet dysfunction and recurrent bacterial infections. Our cases include: the first: a 1.5 year old girl was admitted because of fever and vomiting. He had twice admition with lower respiratory infections at 14th day and 2th month of age. Clinical examination showed multiple hypopigmentation in face and extremity. Iris was bluish and his hair had silver like color. There was Hepatosplenomegaly. A second case (2 month's age girl) suffered from hemophagocytic lymphohistiocytosis, which is known as accelerated phase. She had high fever, bleeding tendency, hepatosplenomegaly, and pancytopeny. A third case (7 month`s age boy) reffrred with recurrent otitis media and pneumonia. Hair and skin of this case is silvery color (hypopigmentaion). Conclusion: Chediak-higashi syndrome (CHS) is a rare primary immune disorder. Knowledge about that produces prevention from infection and early bone marrow transplantation can cure it. Keywords: Chediak-higashi syndrome, albinism, neutrophil granules, cases

Vocal cord paralysis mimics persistent asthma

Ahmad Bahrami Rassul Hospital, Tehran University of Medical Sciences, Thean, Iran A 17-month-old child with chronic severe cough for 4 months referred to our allergy clinic. His cough increased with crying, laughing and eating. The symptoms occur throughout the day. The patient was on flixotide (fluticason 125) bid for 4 months with the diagnosis of asthma without any improvement in his symptoms. Besides, his cough does not respond to albuterol. PMH: He was delivered with caesarian section due to his oligohydraminus. His vaccination was updated. He had admitted in hospital for pneumonia 3 times in past four months. He had no exposure to tobacco smoke. Physical examinations: his vital signs were stable, his lung, cardiac and abdominal examinations were normal. He had perioral cyanosis during his severe cough. He had clubbing. Chest x-ray and lung CT scan were normal. The barium swallowing was normal without any GEReflux. Accordingly there was no convincing evidence of asthma, so rigid broncoscopy was done and left true vocal cord paralysis was diagnosed. His parents were advised to stop flixotide and instead omeprazole (5mg bid) was ordered. Besides, they were advised for correct positioning during sleep and feeding. Intra vocal cord injection and speech therapy were considered for him. VCD frequently mimics persistent

asthma, but no relief with BD in VCD can be helpful for diagnosis. Keywords: asthma, vocal cord paralysis

Multiple cancers in a patient with common variable immunodeficiency

Mohammad Nabavi1, Hossein Esmaeilzadeh1, Saba Arshi1, Morteza Fallahpour1, Nima Rezaei2,3 1Department of Allergy and Immunology, Rasool e Akram Hospital. 2Research Center for Immunodeficiencies, Children's Medical Center 3Molecular Immunology Research Center; and Department of Immunology, School of Medicine 1,2,3 Tehran University of Medical Sciences, Tehran, Iran Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency. Recurrent bacterial infections are considered as the main clinical manifestations of CVID, while patients have also a predisposition to a number of complications including autoimmunity, granulomatus disease and malignancy. It has been estimated that the patients with CVID have an increased rate of malignancies, about 1.8–13 fold in all types of cancers, compared to the normal population. The presented case is a unique one, since the patient experienced cancers in at least three organs. Malignancy is one of the important complications of CVID which could lead to co-morbidity and mortality in the affected patients, especially in elderly ones. Keywords: immunodeficiency, CVID, Cancer The study of Helicobacter pylori in persistent

idiopathic chronic urticaria

M Sadeghi-Shabestari, M Rafeey, B Nasiri TB and lung research center, Children Hospital, Tabriz University of Medical Sciences

Background: Because of controversy found in prevalence of H. Pylori infection in idiopathic chronic urticaria patients, we decided to assess of the effect of Helicobacter Pylori in the treatment of patients with resistant idiopathic chronic urticarial. Methods: In a Clinical trial study, 120 patients in a range of 4-20 years with idiopathic chronic urticarial not responsive to routine treatments were studied and after performing a UBT they divided to two groups, UBT (-) and UBT (+). Eradication therapy for H. Pylori infection started for these patients. After 2 months UBT was performed for patients who received eradication therapy. In those patients who eradication was done, urticaria symptoms and level of responsiveness to treatment was recorded in a period of 3 to 6 months. Findings: Of 120 patients, 35% were males and 65% were females. Positive history of allergy was 47.5% vs 13.9% in the UBT (-) and UBT (+) groups. The period of symptoms was permanent in 52.5% of patients and 0% of controls. Severity of urticaria was severe in 12.5% of patients and 2.5% of controls. Conclusion: We showed a statistically significant difference in period of symptoms before diagnosis, severity of urticaria, abdominal pain, period of symptoms, positive history of allergy and cell blood count between two groups.



Also there is no significant relation between eradication of H. Pylori infection and remission of symptoms in idiopathic chronic urticaria patients. Keywords: H. Pylori infection, idiopathic chronic urticaria, eradication

Allergic Rhinitis Masoud Movahedi Department of Pediatrics, Tehran University of Medical Sciences Although allergic rhinitis was a rare condition in the past, but it has increased with civilization so that in some areas its prevalence is about 30-40%. AR is an inflammation of nasal mucosa characterized by nasal congestion, rhinorrhea, itching, sneezing and sometimes conjunctiva irritation. It has detrimental effects on quality of life. Its comorbidities are: sinusitis, conjunctivitis, otitis media, serous otitis, nasal polyposis, hypertrophy of tonsils and adenoids, obstructive sleep apnea. AR may coexists with asthma (up to 78% of asthmatic patients have AR, and 38% of patients with AR have asthma). AR is classified according to severity and its impact on asthma: Intermittent: <4 days/week or<4 weeks Persistent: >4 days/ week and>4weeks Mild: normal sleep and no impairment of daily activities, sport, leisure, normal school and work, no troublesome symptoms. Moderate/severe: one or more items: abnormal sleep, impairment of daily activities, abnormal school and work, troublesome symptoms. The goal of treatment is effective and safe prevention and relief of symptoms. Allergen avoidance, use of antihistamines, intranasal corticosteroids as an evidenced-based method is the management of AR. Keywords: rhinitis, allergic disorders, itching, sneezing

Primary Immunodeficiencies in Asia: Today

and Future Asghar Aghamohammadi, Hassan Abolhassani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Primary Immunodeficiency Diseases (PIDs) are heterogeneous group of inherited disorders, which are characterized by different defects in the development/function of the immune system. These defects render a patient susceptible to a variety of infectious diseases. The infections in PIDs can occur repeatedly, severely and atypically damaging the organs, reducing quality of life. Up to date about 180 PIDs have been phenotypically described, among them, there are over 120 primarily single-gene defects. There is no clear estimation of the frequency of PIDs, however it seems that many people carry a PID. Early diagnosis and adequate therapy are the keys for survival and a better quality of life, while delays in diagnosis and /or inadequate management may lead to permanent organ damage and shortening lifespan. Unfortunately failure to recognize these conditions is still a major problem for clinicians around the world and diagnosis of patients with PIDs is associated with a considerable delay ranging from 2.5 to 5 years in children and adults. Asia is the largest continent in the world

encompassing 30% of the Earth's total land area. Population of Asia is over than 4 billion people which are more than 60% of the world's current human population. The overall rate of consanguineous marriages in Asia is high and is about 20-40 %. High rate of consanguineous marriages in the Asia makes PIDs (especially autosomal recessive forms) more prevalent than those in the Western countries. Indeed, many defective genes that underlie PIDs were firstly described in the patients originated from this region. Base on the population of Asian countries and prevalence of PIDs (1/10000), number of PIDs cases may be about 400,000 cases, however total reported patients in these region were less than 4000 cases (1%) which is much lower than estimation. One major problem world especially in Asian countries is that general practitioners, physicians and pediatricians lack familiarity with PIDs and lack guidance regarding the appropriate treatment. Lack of awareness among medical community as well as under-developed infrastructural diagnostic and therapeutic facilities are the main problems encountered in the management of PIDs in the Asia. However, timely diagnosis is not the whole story. PIDs need continous care and sophisticated therapies which are not generally available in the most of Asian countries. Moreover, conducting research (which is an integral part of PIDs management), on gathered data/samples from patient repositories not only improves the care of defined PIDs, but also opens the way to the clarification of ever un-known PIDs. Reaching the goals of increased awareness of practicing physicians about PIDs, shortening the diagnostic delay, providing an appropriate management and accomplishing fruitful research needs the establishment of a regional organization specifically devoted to education, diagnosis, management and research of PIDs based on collaborative work among physicians who take care of PIDs patients in Asia. We hope that establishment of national and international organization related to PID issues will be able to improve situation PID in Asia consequently improving the diagnosis and treatment for PIDs patients across Asia. Keywords: Primary Immunodeficiency, Asia

Atopic dermatitis in children : a practical

approach Mohammad Gharagozlou Department of Pediatrics, Tehran University of Medical Sciences Atopic dermatitis (AD) is a chronic pruritic and inflammatory skin disease that typically begins in early childhood especially in the first months of infancy. AD is one of the most common skin disorders in children. The prevalence of AD in children has increased during the recent years, like other atopies. Its prevalence in Iranian children according to the ISAAC studies seems to be about less than 10%. AD has a significant role in lowering the quality of life of the patients and their families, owing to pruritus, sleep disturbance, pain, irritability, restricted activities and social impairment. It also could be a major cause of poor school performance. Complex interaction between several factors has been considered in the pathophysiology of AD including defect in skin barrier function, host immune response , environmental factors and infections agents. The most significant clinical feature of AD is the eczematous, erythematous, and scaly lesion which typically affects the face and extensors. Pruritus is



the most required component in the diagnostinc criteria. The clinical assessment should be included the evaluation of triggers which are important in control of AD and successful therapy. Management of AD is a multidisciplinary approach and needs the patient, families and health care persons patience. Regular daily topical emollients and intermittent topical corticosteroids are the cornerstones of therapy. Unresponsive eczema should be suspected of secondary infection, poor compliance and psychological factors. Keywords: Atopic dermatitis, allergic disorders, eczema

Serum immunoglobulin level in pediatric burnt patients

B Sobouti1, SH fallah2, Y GHavami3, M Moradi4 1Burn Research Center (BRC), Shahid Motahari Burns Hospital. 1,2,3,4Tehran University of Medical Sciences Background: Infections remain the leading cause of death in burn patients. Immune responses play an important role in patient’s defense mechanism against infection and decreasing morbidity and mortality associated with burn. Our goal was to determine serum immunoglobulin levels in pediatric burn patients in order to understand role of humoral immune defense in these patients. Methods: During this analytic cross sectional study from January 2011 to February 2012, all patients with burn injury and younger than 6 years old that were referred to Shahid Mottahari burn and reconstruction center were enrolled. Findings: The burn size in 12 patients(24%) was less than 30%, in 30 patients(60%) was between 30-50% and in the remained 8 patients(16%) were more than 50%. In 45 patients(90%) depth and severity of burn were 2b and in the remaining 5 patients10%), it was 3 or 4. In 28(56%), 1 (2%), 3(6%), 35(70%), 48(96%), 19(38%) and 6(12%) patients IgG, IgM, IgA, IgG1, IgG2, IgG3 and IgG4 were lower than normal values, respectively. No significant correlation was seen between burn size(TBSA) and value of immunoglobulin (P > 0.05). Although the drop in the serum concentration of immunoglobulins is irrespective to the burn size, more severe burn is associated with more decrease in the serum levels of IgA, IgM, IgG and its subclasses. However, further studies are needed to provide complementary data on this issue. Keywords: Pediatrics, burn, serum immunoglobulin Evaluation of children suspected to primary

immunodeficiency Asghar Aghamohammadi, Hassan Abolhassani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center,Tehran University of Medical Sciences Tehran, Iran Recurrent and chronic infections in children are common reason for physicians' visits, which make a diagnostic challenge to pediatricians. Although the most frequent manifestation of patients with primary immunodeficiency is recurrent infections in the first years of life, in patients with this symptom, all other causes of recurrent infections including allergy, anatomical and functional abnormalities, and secondary immune deficiencies should be considered. In defining of recurrent infections, rather than number of

infections, the nature and pattern of infections such as severity, long lasting of infection, resistant to treatment, unusual microorganism causing infection and unusual complications are important. In approach to patients with recurrent infections, to distinguish patients with PIDs from those with intact immune system or other underlying disorders, attention should be paid to warning signs and symptoms indicating persistence of PID. In taking medical history, in addition to the nature of infection, other factors such as age of onset of disease, site of infection, the type of microorganisms involved, and family history are helpful. Choosing the proper immunological test in the primary and advanced investigations should be decided regarding medical history, family history and physical examination. Therefore, the proper investigation can be chosen by using the diagnostic algorithm. In evaluation of humoral immunity, qualitative (immunoglobulins and subclass serum levels) and quantitative (response to vaccination against protein and polysaccharides antigens) surveys should be performed. Furthermore qualitative (lymphoctye proliferation after stimulation with specific antigen or mitogen) and quantitative (flow cytometry of special CD markers) evaluation is critical issues in the assessment of cellular immune system. Genetic studies are also available in the diagnostic setting of many hereditary disorders that can be also used to evaluate family members of the patient. Early diagnosis and adequate therapy are the keys for survival and a better quality of life, while delays in diagnosis and /or inadequate management may lead to permanent organ damage and shortening lifespan. Keywords: primary immunodeficiency, infection, children Drugs Induced Stevens-Johnson syndrome in

4 years old boy S Saket, M Mansouri, N Taee, H Sherkat Alabbasiyeh Lorestan University of Medical Sciences Background: Stevens-Johnson syndrome (SJS) is a rare but life threatening drug side-effect. 99 cases (including 6 mortalities) had been registered in Iranian ADR center, by now. Case Report: This case was a 4-year old boy, who was admitted in Boroujerd hospital in 24 June 2012 with 2 or 3 episodes of febrile seizures. The patient was treated with phenobarbital tablet (50mg/PO/q12hr) and ibuprofen syrup; he was discharged after partial recovery. During the first eight days after hospital discharge (since 26 June 2012 to 4 July 2012) parents had mistakenly given the antiepileptic drug twice more than the advised dose to the child. 6 days later (9 July 2012) the child was admitted again with rash, high fever and deterioration and was treated by co-trimoxazol and clarithromycin syrups, then was referred to Madani Children's Hospital in Khorram-abad while he was too toxic and with obvious skin and conjunctival lesions. The primary diagnosis was SJS and after primary care, he was referred to a subspecialty center. Conclusion: Although Mycoplasma pneumoniae is known as the most important organism leading to SJS, some drugs such as sulfonamides, NSAIDs, antibiotics, antiepileptics should be noticed in patients with skin lesions and the drugs should be discontinued whenever the rash is observed. Keywords: Stevens-Johnson syndrome, seizure, phenobarbital, pediatric

37

Frequency of sinusitis (CT scan) in hospitalized children with meningitis: a cross-

sectional study; Tehran, Iran

V Zzarabi, S Noorbakhsh, M Farhadi, A Tabatabaei Research Center of ped infect disease, Rasule Akram Hospital Background: Paranasal sinuses are common places for infection in Iranian children. Careful search for sinusitis and appropriate treatment is needed to decrease the risk of bacteremia and subsequent invasive infections (e.g. meningitis, subdural empyema, brain abscess). The objectives were to determine the frequency of sinusitis(CT scan) in children with meningitis. Methods: A prospective, cross sectional study done in pediatric infectious ward of Rasul Akram Hospital in Tehran, Iran (2010-2011). Exclusion criteria: We excluded all cases diagnosed as a concomitant or nosocomial infection (eg pneumonia, osteomyelitis, UTI etc) Findings: 65 cases with meningitis had full criteria for following the sinusitis. Paranasal sinus CT performed in 65 cases; 1 month-16 years; mean= 4.2 year, septic meningitis: 53.6% (36/65), aseptic meningitis: 46.4%(29/65). Undeveloped sinus 7.6% (n=5) < 4 months), Sinusitis 32% (20/65), including: Maxillary sinusitis in 16/60(64%), Pan sinusitis (12%), sphenoiditis: 8%, ethmoeiditis: 16%, frontal sinusitis=0, Chronic sinusitis: 50% (n=33) cases. Conclusion: Physicians treating hospitalized cases with sinusitis should be aware of the risk of meningitis (septic & aseptic meningitis) in 31% of cases. Meningeal manifestations or CSF changes might be due to bacterial sinusitis. The diagnosis of initial site of infection, cause or coinfection in meninges or sinus tract is very difficult. We recommend sinus tract evaluation in every case with meningitis(septic or aseptic). Adequate treatment in chronic sinusitis would be helpful in preventing of readmission. Keywords: Sinusitis, meningitis, paranasal sinus CT (computed tomography) scan

Evaluation the agreement between Quantiferon-TB assay and tuberculin skin

test in TB infected cases S Noorbakhsh, J Mousavi, M shokrolah MR, M barati Research Center of ped infect disease, Rasule Akram Hospital Background: Treatment of recent tuberculosis (TB) infection to prevent progression to active disease has been an essential component of public health efforts to eliminate TB. A recently developed whole-blood interferon (Quantiferon-TB) assay shows promise for the diagnosis of recent TB infection. Our objective was a comparison the agreement between the Quantiferon TB assay and the 5-TU dose of purified protein derivative (PPD) in the diagnosis of recent TB infection in household contact of cases with proven active pulmonary TB in a BCG-vaccinated population. Methods: A cross-sectional analytic study conducted in a tertiary referral hospital in Tehran, Iran (2009-2011) upon

59 recent household contacts of active pulmonary TB (bacteriologically proven) selected by convenient sampling. All the cases were immune competent which had received BCG vaccine at birth and without previous anti TB treatment. The PPD was defined as positive at an induration of > or =5 mm. Each child who tested as positive on the PPD was referred for medical evaluation. Results of QuantiFERON-TB assay and the PPD test compared in cases. All analyses were conducted using SPSS 11.5 and P-values<0.05 were considered statistically significant. Mc Namara and computing kappa statistics used for assessing the concordance between the PPD and whole-blood IFN- assay. The percentage concordance between 2 tests was reported as Cohen kappa (κ) statistic in the following categories: excellent(κ > 0.75), fair to good (κ = 0.4–0.75) or poor agreement (κ < 0.4). Findings: Cases(N=59) aged between 1-69 years; Mean: 42±17.84 years. 42.7% (54) of cases were males; 57.6% (66) were females. Family size was 1-8 persons; mean: 3.61-0.929. Overall, induration size for PPD test was between 0-40 mm; mean: 7.56 ±6.66 mm. The overall agreement between the PPD and the QuantiFERON-TB assay in studied cases was fair to good.(= 0.556); a significant difference observed between cases with positive PPD(3.97±2.295 mm) and negative PPD(0.85±0.261mm) in mean of Quantiferon-TB assay(T=2.301; P =0.025). PPD test in compare with Quantiferon-TB assay had sensitivity=57.1%, specifity=86.6%, Positive Predictive Value (PPV) =57.1 %, Negative Predictive Value (NPV) =86.6%; positive likelihood ratio + (LR+ = 4.29); negative likelihood ratio -(LR- = 0.49) Conclusion: Quantiferon-TB assay had greater specifity in compare with PPD and is a better indicator of the risk of M tuberculosis infection than PPD in Iranian cases as an intermediate tuberculosis-burden country with a BCG-vaccinated population. It might reflect recent rather than remote TB infections. Adding the Quantiferon -TB assay to conventional PPD test (low sensitivity= 57 %) is useful for definite diagnosis of TB infection in high risk groups (household contacts of active pulmonary TB). QuantiFERON-TB assay is excellent for decision the chemoprophylaxis treatment for close contact cases in our country. The cost/benefit should be evaluated by Ministry of Health in our country.

Neuropsychiatric disorder due to GABHS Streptococal infection in children: a case

control study

F Ebrahimi taj, S Noorbakhsh, E Shirazi, A Tabatabae Research Center of ped infect disease, Rasule Akram Hospital

Background: Recent evidence suggests that group A ß-hemolytic streptococcal infection may increase the risk for PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection). Objective of this study was comparison the titer of antibodies against GABHS (ASOT, Anti-Dnase B, and Anti streptokinase) between children with PANDS (obsessive-compulsive disorder, and attention deficit hyperactive disorders) and control group.

Infectious Diseases & Vaccination Abstracts

38 Proceeding of the 24rth International Congress of Pediatrics – Oct 2012

Infectious Diseases & Vaccination

Methods: A cross sectional/ cases control study had done in pediatric psychology clinics in Tehran, 2008-2010. We compared serum antibodies against GABHS(streptolysin O, deoxyr ibonuclease B, and Streptokinase) quantitatively (ABcam-ELISA, USA) between 79 cases with OCD, or ADHD and 39 controls(age-matched). The antibody titers (IU/ml) in their sera were compared and analyzed statistically. The area under ROC, sensitivity, specificity and positive predictive value of tests calculated. Findings: Most of cases studied in summer(57%) and spring(23%). 3 type of antibodies were higher in cases (p=0.000). Antisterptolysin O(cut off level 195) had 90% sensitivity; 82% specificity, PPV 92%, AUC: (CI= %95; 0.99-0.91). Anti streptokinase(cut off level 223) had 82% sensitivity, 82% specificity, PPV 95%, AUC: (CI= %95; 0.934-0.735). Anti DNase (cut off level 140) had 82% sensitivity, 82% specificity, PPV 95%, AUC: (CI= %95; 0.99-0.91). Conclusion: It presents possible role for streptococcal infection in OCD & ADHD disorders. We found a significant higher antibodies against GABHS in OCD & ADHD cases in comparison with healthy children. Treatment of streptococcal infection is achievable by using of long acting penicillin in our country. Use of aggressive treatment like plasmaphresis, IVIG… needs future RCT studies. Group A ß-hemolytic streptococcus (GABHS), PANDAS (pediatric autoimmune neuropsychiatric disorders), OCD(obsessive-compulsive disorder), ADHD syndrome (attention deficiet hyperactive disorders) Keywords: Group A ß-hemolytic streptococcus, PANDAS, obsessive-compulsive disorder, ADHD

Epidemiological characteristics of children’s respiratory tract viral infection in Chongqing

during 2009 and 2011

Xiaoying Lei, Donghong Peng Children's hospital of Chongqing medical university, China Background: Respiratory infectious diseases are very common in children, and respiratory viruses are the main pathogens. It is very important to identify the infectious pathogen to prevent antibiotics abuse. Our Objective was to compare the epidemiological characteristics and pathogenic condition of common respiratory tract viral infection in hospitalized children in Chongqing city of China. Methods: Retrospective analysis of 19452 cases admitted in the respiratory wards from 2009 to 2011, was detected by direct immunofluorescence for seven common respiratory viruses in children’s nasopharyngeal secretions. Findings:1. The positive rate was 48.26%, and positive rates for each virus were RSV: 29.23%, ADV3: 98%, IVA: 2.54%, IVB0: 48%, PIV1: 1.95%, PIV2: 0.25%, and PIV3: 9.83%. The peak periods of RSV infection were in December and January. Both PIV3 and ADV were common from April to July .2. The Statistics results of virus infection of different gender, age and season became statistically significant. 3. Among the 6341 cases in 2009, RSV, PIV3 and IVA were the top 3 viruses among pneumonia, bronchiolitis, bronchitis and laryngotracheobronchitis children; ADV, IVA and PIV3 were the top 3 viruses in the upper respiratory infection children. 4. The Statistics results of virus infections

between common pneumonia and severe pneumonia became statistically significant. Conclusions:1.Virus was still the main pathogen of respiratory infection disease. RSV still took the first place of viral pathogens, PIV3 and ADV followed it. RSV were common in autumn and winter while ADV and PIV3 were common in spring and summer. 2. The virus infections were relevant to gender, age and season. 3. RSV, PIV3 and IVA were the main viral pathogens in the lower respiratory infection. ADV, IVA and PIV3 were the main viral pathogens in the upper respiratory infection. 4. The virus infectious rate of severe pneumonia was higher than common pneumonia. Keywords: Children, Respiratory infection, Virus

High frequency of extrapulmonary tuberculosis in pediatric patients

Shahram Rafie, Ehsan Valavi, Fatemeh Hemmat nia, Peyman Parchami Ahvaz Jundishapur University Background: Tuberculosis (TB) is one of the leading infectious diseases causing significant morbidity and mortality worldwide. Although the infection of M. tuberculosis usually results in pulmonary TB, other organs and tissues can also be affected, resulting in extrapulmonary or disseminated TB. Extrapulmonary tuberculosis is not a rare kind of TB infection in Iran. In this study, we evaluated clinical and laboratory data of our TB cases diagnosed during last three years. Methods: The study sample included fifty two TB patients of Khuzestan province whose TB diagnosis were confirmed by mycobacterial culture performed at Management Centre for Communicable Diseases, Jundishapur University, Ahvaz, Iran, during the time period from March 2009 to April 2012. An individual was suspected to have active pulmonary TB (PTB) if the individual had pulmonary TB symptoms, systemic symptoms or extrapulmonary involvements. Information about prior TB exposure, infection or disease, past TB treatment was obtained as additional supporting evidence. Findings: Of the 52 patients, 36 (69.2%) were girls and the female to male ratio was 2.25. The mean age for all children was 9.4 years (9.83 yr in girls and 9.06 yr in boys). About 88.4% of patients were living in urban areas and the rest were rural. All were new cases and there was not any relapsed case. History of contact with TB patients was found in 14 children (26.9%). Extrapulmonary TB was found in 33(63.4%) children. The most common sites of involvement were lymph nodes in 25 (80.6%), pleura in 2 (6%), skin in 2 (6%) and each one of bone, eye and urinary tract involvement in one case. We have not any cases with TB meningitis or military TB in these years. All of our cases treated with the standard WHO guide-line and we had not any drug resistance. There was no death reported during the study period. Conclusion: Extrapulmonary tuberculosis remains an important health problem to the pediatric population of this province despite the overall decline in the incidence of tuberculosis. Early detection and thorough treatment for a suitable period are mandatory to improve the prognosis of this potentially curable infectious disease. Keywords: tuberculosis, extrapulmonary, mycobacterial infection



Neurological manifestations in acute onset of viral (Rota & Adeno) gastroenteritis

A Tabatabaei, S Noorbakhsh, F Ehsanipour, H.R Monavari, A Eizadi, M Faramarzi, S Javadinia Research Center of ped infect disease, Rasoole Akram Hospital Background:to evaluate the prevalence of neurologi cal manifestaions in acute onset of viral gastroientritis. Methods: A cross sectional /descriptive study performed upon 50 children admitted due to acute viral gastrointestinal infectionsin Department of pediattric Infectious Disease, Rasul Hospital,Tehran, Iran, 2010- 2011.I nitially, a questionnaire was completed by an authorized physician for each cases (e.g. age, gender, clinical signs, vomiting, diarreha (type,time of onset, frequency),attending time from onset,type of neurologic symptoms,analysis of lab test (stool direct exams, biochemical parameters, stool culture, direct viral test in stool). All cases with bacterial or other known causes(except viral causes) for gastroentritis,chronic diarreha excluded from study. The studied cases were evaluated for existence of neurologic signs .Stool samples were searched for viral antigens (Rota & Adeno virus) by Rapid immunochromatograpghic test. P-values less than 0.05 were considered statistically. Findings: Neurological manifestations reported in 16% of cases included seizure (12%); aseptic meningitis (4%). 20% of adenovirus, 13.5% of rotavirus and 33.3% of biinfection had neurologic signs, with no differences (P=0.6). Mean age of cases had not significant difference between cases with and without neurologic manifestations. There was no significant association between neurological symptoms with age (P=0.7), sex, virus type, and attending time (P> 0.05). Conclusion: We concluded that one out of six chidren with acute onset of acute viral GEwould develop neurological symptoms that is usually as seizure and is not related to age, sex, virus type, and attending Keywords: Convulsion, neurologic symptoms, diarreha, acute viral gastroentritis, Rota virus, Adeno virus

Fatal type 7 Adenovirus associated with Bocavirus infections in a Healthy child

Hosein Heydari2‚ Setare Mamishi1‚ Ghamar-Taj Khotaei1, Sara Moradi1 1Department of Pediatric, Tehran University of Medical Sciences. 2Qom University of Medical Sciences Adenovirus is a common cause of acute upper respiratory tract infection and rarely causes respiratory failure or septic shock. Most of these infections are self-limited, with severe or disseminated disease confined to immunocompromised patients, like patients with AIDS and transplant recipients. We describe a well documented fatal case of Bocavirus and adenovirus pneumonia with myocarditis in an immunocompetent child. Case presentation: A healthy 2 year-old child presented with a four-day history of fever and a two-day history of dry cough and dyspnea. There was history of travel to Kish 24 days ago. He was admitted to Children hospital with acute respiratory distress and low of consciousness. Heart rate was 130 beats/min, her temperature was 40.5°C, and her blood pressure was 120/80 mm Hg. Respiratory rate

was 58 and he had o2 saturating 40% on room air. Laboratory Data: White blood cell count 7,900/mm3 with a neutrophilia 73%‚ thrombocytopenia (59000/U), PT: 31sec‚ PTT: 120sec‚ INR: 5.3 ‚ cold agglutination test: negative‚ elevated SGPT (5100U/L), SGOT (5000U/L) and creatinine (2.4mg/dl). Triglyceride(TG) and Cholestrol were normal. Urinanalysis: positive blood. Erythrocyte sedimentation rate (ESR) was 39 mm/hour and C-reactive protein was 230 mg/litre. Serology was negative for influenza A & B, respiratory syncytial virus (RSV), para influenza‚ and hemorrhagic fever virus. Blood, stool and urine culture were negative. Throat culture was negetive but PCR showed type7 adenovirus and Bocavirus. Keywords: Adenovirus, Bocavirus‚ myocarditis Detection of HHV-6 in CSF of children with febrile convulsions: A Real Time PCR assay

J Yavarian, S Mamishi, NZ Shafiei Jandaghi, L Kamrani School of Public Health, Tehran University of Medical Sciences Background: Human herpesvirus 6 (HHV-6) causes childhood febrile illness named exanthem subitum. Primary infection usually occurs in the first few years of life and the virus is considered to be one of the most common causes of acute febrile illness in childhood which can associate with febrile convulsions(FCs). FCs are common in early childhood, affecting 2-3 % of children between 6 months and 5 years of age. Primary HHV-6 infection has been reported to cause 8-50% of FCs cases. The aim of the present study was to investigate the frequency of HHV-6 virus in CSF of hospitalized children with FCs by Real Time PCR. Methods: During the six months from October 2010 to March 2011, 114 children two years of age or less who were hospitalized at Children Medical Center of Tehran University of Medical Sciences with CFs and from whom CSF was obtained were enrolled in this study. Real Time RT-PCR with Taq Man probe was performed on these samples. Findings & Conclusion: The presence of HHV-6 virus was confirmed in 10 patients among the total of 114 cases (8.8%). Nine of these children were males and one was females. Nine of these children experienced the first FC and one the second. All were less than 13 months of age. These findings suggest that primary HHV-6 infection might be associated with FCs in children especially for a first episode of FCs but more investigations must be performed on large sample size to obtain more complete results. Keywords: HHV-6, Febrile Convulsions, Real Time PCR

Frequency of Alloicoccus otitidis,

Streptococcus pneumoniae, Moraxella catarrhalis and Haemophilus influenzae in children with otitis media with effusion in

Iranian patients Babak Saedi, Seyed Sajjad Khoramrooz, Akbar Mirsalehian, Mohammad Emaneini Tehran University of Medical Sciences Background: To determine the presence of common bacterial agents of otitis media with effusion (OME), together with investigation these agent in the adenoid tissue



and antimicrobial susceptibility pattern of isolated bacteria in Iranian children with OME. Methods: Polymerase chain reaction (PCR) and bacterial culture methods were used for detection and isolation of Alloicoccus otitidis, Streptococcus pneumoniae, Moraxella catarrhalis and Haemophilus influenzae in 63 middle ear fluid samples and 48 adenoid tissues from 48 OME patients. Fifteen patients were bilaterally affected. Antimicrobial susceptibility of all bacterial isolates were determined by disk agar diffusion (DAD) method. Findings: Bacteria were isolated from 47% (n = 30) of the middle ear fluid samples and 79% (n = 38) of the adenoid tissue specimens in OME patients. A. otitidis was the most common bacterial isolated from the middle ear fluid 23.8% by culture and 36.5% by PCR method. S. pneumoniae was the most prevalent pathogen (35.5% and 31.2% by culture and PCR) in the adenoid tissues. In 10 patients the same organisms were isolated from the middle ear fluid and adenoid tissue. Antimicrobial susceptibility pattern showed that most isolates of bacteria were sensitive to ampicillin, Amoxicillin/Clavulanate and fluoroquinolones. Conclusion: The present study, being the first report on the isolation of A. otitidis by culture method in Iran and Asian countries, shows that A. otitidis is the most frequently isolated bacterium in Iranian children having otitis media with effusion. In this study A. otitidis, S. pneumoniae, H. influenzae and M. catarrhalis are the major bacterial pathogens in patients with OME and we found that ampicillin and Amoxicillin/ Clavulanate have the excellent activity against bacterial agents in Iranian children with OME. Keywords: otitis media with effusion, Alloiococcus otitidis, Streptococcus pneumoniae, Moraxella catarrhalis, Haemophilus influenzae

An Outbreak of Pertussis in Kurdistan; Do We Need Changing Vaccination Policy

Jafar Soltani, Keivan Fiaz-Moghadam, Mohamad-Jamil Amjadi, Afagh Dejbord Kordestan University of Medical Sciences

Background: Pertussis is a major cause of childhood morbidity and mortality. It is a compulsory notifiable disease in Iran. However the reported numbers of cases are very sparse based on current surveillance. The fall in cases probably reflects a weak surveillance system. We report a community pertussis outbreak that occurred in Sanandaj, west of Iran during May-August 2012. Methods: All patients referred to Besat Hospital in Sanandaj with symptoms and signs suggesting Pertussis were investigated. Special checklist form was designed based on World Health Organization guidelines for surveillance and case definition of Pertussis. Nasopharyngeal swabs for all suspected cases were sent to Pasteur Institute in Tehran for bacterial culture and PCR. Findings: A total of 43 cases were investigated because of symptoms and signs suggesting pertussis. Twenty seven cases (62.8) occurred in infants aged <6 months. Cough duration in 26 (60.5%) patients were longer than 2 weeks. In all cases (100%) cough was paroxysmal that followed by cyanosis in 37 patients (86%). Posttussive vomiting was present in 27 (62.8%) cases as well as whooping in 9 (20.9%) patients. Chest X-rays were reported normal in 19 (44.2) patients. Based on clinical findings, 43 patients fulfilled WHO clinical case definitions for Pertussis. Culture of nasopharyngeal specimens were negative in all

patients, however PCR were reported positive in 10 cases to date including 4 cases with cough duration of less than 2 weeks. These cases classified as "Laboratory confirmed" based on WHO case classification. Vaccination history of pertussis cases was as follows: no vaccination in 14 (32.5%), all of these patients aged less than 2 month when symptoms started, DTP1 in 11 (25.6), DTP2 in 2 (4.7%), DTP4 in 14 (32.6%), DTP5 in 2 (4.7%) of patients. Conclusion: Weaning of immunity due to vaccine failure may be the cause of pertussis outbreaks. Attention should be paid to start DTP1 at earlier ages and vaccination of adolescents to protect newborn infants due to the higher incidence and mortality rate in this age group. The report emphasizes the needs to strengthen the surveillance of pertussis and to study vaccine efficacy in Sanandaj-Iran. Keywords: pertussis, outbreak, whooping cough, surveillance

The prevalence of pharyngeal colonization with streptococcus pneumoniae and its

antibiotic susceptibility in children referred to 17 Shahrivar Hospital in Rasht-Iran

H Hashemian, A Khaleghjoo, A Mojtahedi Gillan University of Medical Sciences Background: Streptococcus pneumonia (pneumococcus) is the most common cause of community-acquired bacterial respiratory tract infection. There are few data about antibiotic-resistant streptococcus pneumonia in Iran. The aim of this study was to determine the prevalence of pharyngeal colonization of S. pneumoniae and its antimicrobial resistance pattern in children. Methods: This cross-sectional study was performed from April 2011 to April 2012 on children aged 3 months to 12 years old who were referred to 17 Shahrivar Hospital in Rasht. After gathering background and demographic data, pharyngeal samples were collected by sterile swabs and were placed in Stuart transport media; then cultured on selective agar. After that, microbiological tests were performed and antimicrobial sensitivity tests were done by standard disk-perfusion method. Findings: Of the 372 children, 203 (54.56%) were boys and 169 (45.44%) were girls and the mean age was 56.82 months. The carriage prevalence for S. pneumoniae was 12.1%. The highest seasonal prevalence was seen in winter (18%) and autumn (17%) respectively. High antimicrobial resistance rates were seen to Co-trimoxazol (86.6%), Erythromycin (68.8%), Penicillin (55.5%), and Cloxacilin (51.1%); whereas low antimicrobial resistance rates were seen to Vancomycin (6.6%), Chloramphenicol (6.6%), Cefuroxime (11.1%), and Ceftriaxone (13.3%). In this study, prevalence of pneumococcal colonization in the children had no significant relationship with sex, place of residence, number of household members, parental smoking, or breast-feeding, but with antibiotic use during a month before study, having fever and going to day-care center had. Conclusion: Although the prevalence of pharyngeal colonization of pneumococci in this study was low, the resistant rate to the antibiotics was high. In order to avoid of excessive antibiotic use and resulting high rate of resistance, enough education not only to the physicians but also to general population is necessary. Keywords: antimicrobial susceptibility, colonization, Streptococcus pneumoniae



Immunogenicity to measles, mumps and rubella in Iranian infants with local MMR

vaccine

S Zarei1, A H Zarnani2, H Zeraati3, N Mohammadinia4, O Rastani4, F Sardari4, B Tavangar1, Y Vojgani5, M Jeddi-Tehrani1,5, F Shokri1,6 1Reproductive Immunology Research Center. 2Nanobiotechnology Research center, Avicenna Research Institute (ARI), Iranian Academic Center for Education, Culture & Research (ACECR). 3Department of Epidemiology and Biostatistics, School of Public Health, Tehran University of Medical Science. 4East Health Center, Shahid Beheshti University of Medical Sciences. 5Monoclonal Antibody Research Center, ARI, ACECR. 6Department of Immunology, School of Public Health, Tehran University of Medical Sciences Background: Immunization of infants against measles, mumps and rubella (MMR) is a common practice in most regions of the world. The present study reports the immunogenicity of a local MMR vaccine in Iranian infants vaccinated at 12 and 18 months of age. Methods: In this prospective study, 104 infants aged 12 months were administered with two doses of MMR vaccine manufactured by Razi Institute of Iran at 6 month time interval. Blood samples were collected 2 and 6 months after the first dose and 2 months after the second dose administration. Immunogenicity of the vaccine was evaluated by measurement of specific serum antibodies using commercial ELISA kits. Findings: Two months after the first dose injection, 52.9% of children were seroconverted (> 150 mIU/ml) against measles (mean±SD= 1459±1431 mIU/ml). The rate of seroconversion increased to 53.8% (1063±920 mIU/ml) and 97.1% (2492±1035 mIU/ml), 6 months after the first dose and 2 months after the second dose injections, respectively. For rubella, the seroconversion rate (> 4 IU/ml) was found to be 13.5% (34±11 IU/ml), 17.3% (35±13 IU/ml) and 44.2% (53±38 IU/ml) for the time intervals mentioned above. The seroconversion rate of mumps (> 20 RU/ml) was 69.9% (97±87 RU/ml) 2 months after the first dose injection and 60.6% (67±59 RU/ml) and 97.8% (132±62 RU/ml) 6 months after the first dose and 2 months after the second dose injections respectively. Conclusion: Immunogenicity of the measles and mumps components of the MMR vaccine seems to be similar to many other validated vaccines reported in the literature, but comparison between immunogenicity of the rubella vaccine component with the results reported for other commercial counterpart vaccines indicates a lower seroconversion rate for the local vaccine employed in this study. Keywords: vaccine, Measles, Mumps, Rubella, immunogenicity

Nasal carriage of meticillin resistant

Staphiloccusaureus among health care worker in a hospital in kermanshah, Iran

Sohrab Hemati, Nasrollah Sohrabi, Ali Maleki Hazrate masoume hospital, Kermanshah Background: Staphilococcus aureus is one of the most frequent species involved in nosocomial infection such as bacteremia and surgical wound infection. The most

important problem with this bacteria is Meticillin Resistant. S.aureus(MRSA) isolates which is resistace to multiple antibiotics may lead to increased morbidity and mortalitiy. The health care workers(HCWs) who are in close contact with patients are possible sources of nosocomial infection. The aim of this study was to determine the frequency of nasal carriage of MRSA among HCWs in a pediatric hospital (Hazrate masoume) in Kermanshah, Iran. Methods: In this study, a total 52 health care workers in Hazrate masoume hospital in Kermanshah were screened for MRSA. Swab of anterior nares transported and incubated in nutrient broth at 35 c for 24 h then positive sample incubated in mannitol agar(MRSA) at 35 c for(18-24h). S,aureus was identified as gram positive catalase, coagulase and DNAase test. MRSA isolates were identified by combined Oxacillin and meticillin disk diffusion method. Data analysis performed using SPSS software. Findings: Of the 52 health care workers screened, from 11 persons(21/1%) meticillin resistan S, aureus (MRSA) were isolated. All of these isolates were resistant againt oxacillin and meticillin disks and identified as MRSA. Most of samples were obtained from female staffs. Nurses were predominant carriers. Conclusion: MRSA carriage among health care workers in this hospital is high. Therefor for limiting staphilococcal nosocomial infection, S,aureus nasal carrier health care worker should be identified and trained. Continuous survilance and improvement of hygien standards in hospitals should be adopts. Keywords: nasal carriage, Staphiloccous aureus, MRSA, healt care worker

Molecular characterization of extended

spectrum beta-lactamase among Escherichia coli clinical isolates causing urinary tract

infections in an Iranian referral pediatrics center

Babak Pourakbari, Pouya Ostad Rahimi, Shima Mahmoudi, Ehsan Moghanloo, Farah Sabouni, Amir Esmail Alyari, Mohammad Taghi Haghi Ashtiani, Reihane Hosseinpour Sadeghi, Setareh Mamishi Pediatric infectious research center Background: Increased prevalence of Escherichia coli resistant to extended spectrum beta-lactamases (ESBLs) has been reported all over the world and is rapidly changing over time especially in Asia-Pacific region. The aim of this study was to determine the frequency of ESBL genes (blaTEM, blaSHV and blaCTX-M) among E. coli isolates causing urinary tract infections in an Iranian referral pediatrics center. Methods: In this study, 184 hospital and community isolates of E. coli cultured from urine samples between July 2009 and December 2010 were screened for ESBL production. Extended-spectrum β-lactamase (ESBL) production was detected using a double-disc synergy test and presence of blaCTX-M, blaSHV and blaTEM β-lactamase genes was evaluated by PCR. Findings: Among the E. coli strains isolated of each group, 22 (24.4%) isolates of group1, 3 isolates (17.6%) of group 2 and 34 isolates (44.1%) of group 3 were found to harbor ESBL producers. Twenty of 59 ESBL positive isolates carried more than one type of b-lactamase genes. Interestingly, 3 isolates with blaTEM, blaSHV, and blaCTX-M genes simultaneously were detected.



Conclusion: A majority of ESBL-positive strains showed resistance to Trimethoprim/sulfamethoxazole (93.8%), cephalotin (89.2%), and cefixime (77.1%). The most effective antibiotic in ESBL negative group was amikacin (susceptibility rate 95%), followed by gentamicin (91.4%), and nitrofurantoin (82.9%). A relatively high prevalence of ESBL-producing E. coli isolates as well as high prevalence of blaCTX-M in our study mentioned particular attention in our country. High rates of resistance to most of orally available agents in ESBL-producing E coli makes a considerable problem in the treatment of urinary tract infections. Keywords: E coli, ESBL, b-lactamase genes

Effect of nebulized L-epinephrine on the

treatment of croup

Aziz Eghbali, Ali Sabbagh, Manijeh Kahbazi, Nima Rezaei

Arak University of Medical Sciences

Background: As of controversial treatments for croup, this study was performed to assess the effect of nebulized L-epinephrine, readily available worldwide, on the children with croup. Methods: A double blind randomized controlled clinical trial was implemented on 174 patients with croup, aged 6 months to 6 years, whom admitted in the Amir Kabir Pediatric Hospital (Arak, Iran). After allocation randomly, at the early admission and after receiving of either nebulized L-epinephrine or saline, westley croup scores, heart rate, respiratory rate and blood pressure every half hour for 120 minutes were recorded. Findings: After interventions, mean croup scores between two groups have been significantly different over the time (p<0.009). Similarly, mean heart rate between two groups had significantly different during the time (p<0.026). Mean respiratory rate had no significant difference between two trails (p<0.25), but significantly had difference within two groups. Conclusion: There were considerably differences in reduction velocity of croup scores between nebulizer L-epinephrine versus saline. Keywords: croup, L-epinephrine, westley croup score

Genotypic characteristics of Pseudomonas

aeruginosa strains circulating in the tertiary referral Children Medical Hospital in

Tehran, Iran

Setareh Mamishi, Babak Pourakbari, Zahra Movahedi, Shima Mahmoudi, Farah Sabouni, Mohammad Taghi Haghi Ashtiani, Reihane Hosseinpour Sadeghi Pediatric infectious research center

Background: Pseudomonas aeruginosa is an important pathogen with the ability to cause diverse infection types in all departments of the hospital especially in intensive care units (ICUs). The aim of this study was to analyze the epidemiology relationships among clinical P. aeruginosa strains isolated from different wards of hospital. P. aeruginosa strains isolated from various samples collected from different wards of Children Medical Hospital (Tehran, Iran).

Methods: P. aeruginosa isolates were identified by standard laboratory procedures and tested for antimicrobial resistance to several antibiotic agents. The genetic similarity of the strains was investigated by amplification of the Enterobacterial Repetitive Intergenic Consensus sequence (ERIC-PCR). Findings: During the study period, 87 non-duplicate patients were colonized or infected with P. aeruginosa isolate. Among all isolates, resistance to piperacillin/tazobactam was the lowest (27%), followed by amikacin (31%), gentamicin (33%), imipenem (33%), ciprofloxacin (36%), meropenem (39%). Thirty-five patients (40.2%) were either colonized or infected with multidrug resistant P. aeruginosa strain (MDRP) over a 1 year period and 17 isolates were non-susceptible to all the tested antibiotics. One predominant profile (D) was identified in 59 strains. This profile first appeared in PICU and infectious ward in June 2010 and circulated until the end of the study in all wards. Twenty-two of 35 (62.8%) MDRP were found to be profile D. Conclusion: Molecular typing of our isolates suggests considerable cross-transmission of P. aeruginosa isolates not only between patients in one ward but also between patients from different wards that can partially be explained by the high number of patients transfer between different wards of hospital. Keywords: Pseudomonas aeruginosa, genotypic, cross-transmission

Antibiotic resistance profile of Acinetobacter baumannii isolates in pediatric intensive care

unit (PICU) Sedigheh Ghoorchian, Zohreh Ghalavand, Amir Aliramezani, Mona Mohammadzadeh, Mohammad Rahbar, Masoumeh Douraghi School of Public Health, Tehran University of Medical Sciences Background: Multidrug resistant Acinetobacter baumannii isolates have emerged as serious threat in hospitals. Seven cases of Acinetobacter baumannii infection in a pediatric intensive care unit (PICU) were evaluated. The aim of the current study was to assess the antibiotic resistance pattern of A. baumannii isolates. Methods: A. baumannii isolates were cultured from trachea or sputum from the patients admitted in PICU of a tertiary care hospital using conventional microbiologic techniques. The molecular identification of isolates was performed through amplification of blaOXA-51 gene using PCR. Antimicrobial susceptibilities of A.baumannii was determined according to CLSI recommendation by disc diffusion method to followings antibiotics: imipenem, meropenem, doripenem, amikacin, cefotaxime, ceftriaxone, ceftazidime, cefepime, gatifloxacin, tetracycline, gentamicin, piperacillin-tazobactam, cotrimoxazole, doxycycline, minocycline, tigecycline, and colistin. Findings: blaOXA-51 gene was detected in all of the isolates. More than eighty-five percent of isolates were resistant to doripenem, imipenem, and meropenem. Four of seven (57.1%) isolates were resistant to amikacin. Six of seven isolates (85.7%) showed resistance to cefotaxime, ceftriaxone, ceftazidime, cefepime, gatifloxacin, tetracycline, gentamicin, piperacillin-tazobactam, cotrimoxazole and doxycycline. In contrast, 57.1%, 28.5%,



and 28.5% of the isolates were sensitive to minocycline, tigecycline, and colistin respectively. Conclusion: The antimicrobial activities of tigecycline and colistin against A. baumannii isolates remained high for cabapenem-resistant strains. However, the in vitro activity of all carbapenems against A. baumannii was low. In addition, disc diffusion test can be used as a useful screening method for susceptibility testing for tigecycline and colistin against A. baumannii. Keywords: Acinetobacter baumannii, Pediatric, Antibiotic resistance

High prevalence of occult hepatitis B virus infection in children born to HBsAg-positive mothers despite prophylaxis withhepatitis B

vaccination and HBIG S Shahmoradi, Y Yahyapour, M Norouzi, M Mahmoodi, SM Alavian, Z Fazeli, SM Jazayeri Tehran University of Medical Sciences Background: Occult hepatitis B virus (HBV) infection is a well-recognized clinical entity characterized by the detection of HBVDNA in serum and/or in liver in the absence of detectable hepatitis B surface antigen (HBsAg). The frequency of the diagnosis depends on the relative sensitivity of both HBsAg and HBV DNA assays. The objective was to determine the prevalence of occult HBV infection in a high risk group of children who developed HBV infection despite immunoprophylaxis. Methods: The sera of 75 children born to HBsAg-positive mothers previously immunized by HBIG and prophylaxic vaccine regimen were assayed for HBV DNA by real-time PCR. Subsequently, the samples were tested by a sensitive standard PCR employing independent set of primers for all HBV genes and analyzed by direct sequencing. Findings: HBV DNA was detected in 21/75 (28%) of children, ranged between 77 and 9240 copies/mL. All were positive for anti-HBs. 5 (24%) were found to be positive for anti-HBc, and anti-HBc-only positive individual were not observed. 8 isolates (38%) did not contain any mutation. Other 13 infected children (62%) contained at least one mutation in regions known to be involved in functional and/or immune epitope activity. 10 were contained G145R mutations. Conclusion: HBV occult infection seems to be relatively frequent in immunized children born to HBsAg-positive mothers. HBsAg negativity is not sufficient to completely exclude HBV DNA presence. These findings emphasize

the consideration of occult HBV infection in hypo endemic areas. Keywords: Occult hepatitis B infection, HBsAg vaccine, Escape mutants, HBsAg mutant assays

Assessment of clinical and Para clinical tests to predispose of meningitis among Iranian

children with febrile convulsion Khatereh Khamenehpour, Sonia Oveisi Qods hospital, Qazvin Background: Febrile Convulsion (FC) is a frequent cause of hospitalization. Meningitis is one of the most important causes of febrile convulsion, which is diagnosed with lumbar puncture (LP). The necessity of LP is still debating. The purpose of the current study was to compare of LP in management of meningitis among children younger than 2 years old with febrile convulsion. Methods: In this cross-sectional study, three hundred and thirty eight patients were studied from March 2009 to March 2011. They were admitted at the Qazvin children hospital with febrile convulsion. Mean of ages of children was 15.45.±0.29. The percent of gender was 54.1% girls and 45.9 % boys. Age, temperature, family and personal history, duration of seizure, cause of fever, WBC, ESR and the results of LP for each case of fever were collected. We analyzed their serum profiles and the special signs by mann-whitney test. Findings: Seizure related to meningitis (viral or bacterial) were seen in 10 cases. We have separated two groups:; children presented meningitis in G1 and children with febrile convulsion in G2. The comparison between two groups G1 and G2 showed this result: age <24 months (p <0.878), temperature (p< 0.112), recurrence of seizure in the same day (p<0.14), duration of seizure (p<0.18), ESR (p<0.89) and WBC (p<0.043). Mean value of WBC in G1 was 16250±10211 and in G2 was 11234± 5347. Only 1 patient had neck stiffness. Conclusion: In children younger than two years, there were no significant differences in some paraclinic status and some signs between two groups that can be used to diagnose meningitis from other cases of FC except of WBC. Therefore, LP is more useful in this age range. It should be discussed case by case. However, it has been noted that meningitis is more dangerous and its diagnosis should be ruled out. Keywords: febrile convulsion, lumbar puncture, meningitis

44

Confidentiality issues in Pediatrics

Fariba Asghari Medical Ethics and History of Medicine Research Center, Tehran University of Medical Sciences

Background: Medical confidentiality is part of fiduciary duties of physician that ensures patients’ trust on the profession and encourages them to seek medical advices and give a truthful history. The main questions in this regard in pediatrics are: who possess the information? Who could give consent for disclosure of information? What are the exceptions to confidentiality? Discussion: Since children could not weigh risks and benefits and evaluate the long consequences of different choices, they are not considered autonomous and their parents make decisions on behalf of them. However as children grow they become capable of making wise decision and their involvement in care should increase to achieve their best compliance and respect their potential autonomy. Pediatricians sometimes face parents who wish to withhold the information from their child and ask the physician to maintain the confidentiality. It should be emphasized that the information belongs to the patients and physicians in corporation with the parents have to decide when and how the disclosure of information to the child will be in her best interest. Pediatricians who suspect a child abuse or neglect may decide to break the confidentiality to protect the child from a serious harm. Adolescences who have more developed decision making capacity might ask their physician to keep information confidential from their parents. For some conditions such as STD or asking for contraception, physician decisions have to be based on attaining the high compliance of the adolescence for the health support and may decide to keep the child’s confidentiality. Conclusion: In pediatrics the beneficence is the primary ethical principle and physicians have to decide in the child’s best interest and also maintain trust in the profession. The Best interest of the child is the most important key for keeping or breaking the confidentiality. Keywords: Confidentiality, Pediatrics, Best interests

Parental and children's Autonomy in

pediatrics medicine

Alireza Parsapoor, Fariba Asghari Medical Ethics and History of Medicine Research Center, Tehran University of Medical Sciences

As an accepted principle from the viewpoint of Islam, every competent person has the right to control over his life and property to the extent that such control does not violate rights of others and sanctity of life. According to this principle, any action on the physical integrity of an individual, besides observing scientific and technical rules should be taken by obtaining his/her legal guardian’s assent; otherwise doctor is not allowed to take any action. In cases that suitable interaction with patient for obtaining his legal acceptance are not possible, because of his/her limitation in decision making capacity, which is known as lack of capacity or competency, or he/she does not reach

legal age, patient’s surrogate decision maker takes the responsibility and according to the patient’s interests makes necessary decisions in place of him/her. In Pediatrics Medicine, doctor encounters patients with a wide range of mental and perceptual capacities with which they can participate in making their therapeutic decisions. This matter in addition to the important role of parents – who, according to law undertake child guardianship and have the right to make decision for him/her – most of the times brings about ethical challenges for doctor in making therapeutic decisions. In this article, we aim to review the domain of the autonomy of children and teenagers in different stages of their growth as well as the scope of therapeutic decisions taken by parents according to ethical, jurisprudential and legal basics. We also discussed the ways of dealing with such problems and ethical solutions for the inappropriate decisions made by parents for children. It seems as though, in the Iranian law system, which is based on Shiite jurisprudence, children growth is divided into 4 stages: non-discerning child (Gheyre-Momayyez), discerning child (Momayyez), adolescent and finally fully grown or mature (Rashid). It should be mentioned that every individual autonomic domain in each of these stages is different. It is generally accepted when teenagers reach legal age, nine for girls and fifteen for boys, they are considered to have capacity for making decisions respecting their health, on condition that such decisions do not impose financial burden upon them or parents. In cases that taking action necessitates appropriation of properties, child’s competency should be confirmed in court. On the other hand, according to religious law, parents are obliged to consider the best interests of their child, and if their malice or even inappropriateness of their decision (their bad management) is proven, they lose their guardianship in making decision for them, and an authorized person or institution should make decisions according to the best interests of the child. Keydwords: autonomy, pediatrics, medical ethics

Informed consent in children: autonomy or

paternalism? Reza Omani Samani Medical Ethics and History of Medicine Research Center, Tehran University of Medical Sciences Informed consent is considered the most important step in clinical interventions. Consent has always been an essential element in the physician-patient relationship and an ethical duty, but recently it became a legal requirement. One of the most important requirements of a good informed consent is the capacity of the patient which is absent for the children. Although the basis of the informed consent is the respect for autonomy, in the case of pediatric diseases, as the patient has not capacity to give the consent, the autonomy is not the issue. In this paper I argued getting informed consent just from the parents and presented a guideline to treat children which can prevent child neglect. Methods: A review of papers, law and codes both from Iran and abroad. An ethical, legal and religious study was done and all the argument presented and discussed. Conclusion: The informed consent from the parents is not

Medical Ethics


Medical Ethics

in line with respect for autonomy, so it is paternalism. Who can make decision for the child and who knows the best interest of the child? It seems that the decision by physician is the best interest of the child thinking medically. It is suggested to make this decision between both parents and physician. In the case of disagreement between these two, in the case of necessary treatment, the physician can make the decision, but in the case of elective treatment, the hospital ethical committee can be responsible for making decision. Legal support for this guideline is needed. Keywords: informed consent, capacity, children, autonomy, paternalism

Ethical issues in using Alternative and Complementary Medicine in pediatrics

Kiarash Aramesh Medical Ethics and History of Medicine Research Center, Tehran University of Medical Sciences Children, as pediatric patients, are considered as a vulnerable group both in biomedical research ethics and clinical ethics mainly because of their limited capacity in decision-making which usually necessitates a kind of surrogate decision-making for such patients. In such situation, decisions should be made based mainly on the patient’s interests rather his or her wishes. In medicine, this obligation can only be translated to evidence-based decision-making. In this paper I am coming to argue that we have just one kind of valid medicine which is the evidence-based one. Any claim or innovation in this field should only comes to practice according to evidence-based trials and appraisals. Therefore, the procedures of so-called alternative or complementary medicine should be applied in pediatric practice only if they enjoy a sound scientific basis and have been approved according to evidence-based medicine. In this regard, using pseudoscientific measures like homeopathy, energy therapy or humoral medicine, in treating pediatric conditions in the absence of scientific and evidence-based proof is unethical. Even in research on pediatric patients, any experimental remedy or procedure should be tested only if there are sound scientific bases and preclinical evidences for their safety and effectiveness. Keywords: Medical Ethics, Research Ethics, Alternative and Complementary Medicine, pediatrics

Child best interests, parental consent, and paternalism in medical decision making:

Islamic Perspectives

Farzaneh Zahedi1, Fattaneh-sadat Bathaei2 1. Endocrinology and Metabolism Research Center 2.Medical Ethics and History of Medicine Research Center, Tehran University of Medical Sciences

Background: The need to observe patients’ autonomy has been increasingly emphasized in medical practice. So, medical paternalism is no longer acceptable. However, the condition is different in pediatrics, since children are not competent enough to decide autonomously in many situations. We intend to discuss the issue of informed consent in children from an Islamic perspective. Methods: This is a narrative review using books and published articles which has focused on ethics in pediatric care. We searched recent 5 years resources on MEDLINE,

Cochrane and Google Scholar by keywords of consent, ethics, children and Islam. Findings: The results of our study showed a significant notice to the issue of informed consent in children. Textbook of Nelson, as one of the main resources for pediatricians, has devoted one chapter to the topic of “Ethics in Pediatric Care” including issues of assent and consent. Children may lack capacity to understand health issues and participate in decision making. In the legal system of many countries, it is assumed that healthcare professionals can trust on parents to decide according to the best interests of their children. So, the parents’ consent is enough for applying health care services. Meanwhile, when children develop into adolescents, their assent would be also needed in healthcare practice. Social and religious context may influence on the issue. According to the Civil code of the Islamic Republic of Iran, everyone who was born alive has the capacity to benefit the civil rights; however, the full authorization would be realized by achieving three elements of puberty (15 and 9 years old for boys and girls, respectively), wisdom (rationality) and maturity (18 years old). In Islam, also, children under the age of puberty are divided into capable (Momayez) and incapable (Gheir-e-momayez) to recognize the right and wrong. Conclusion: Obtaining assent of capable children is approved by Islam and widely practiced in Iranian society, although there is no law on this issue in the Iranian legal system. So, Pediatricians should respect children autonomy and encourage them to take the responsibility for their own health. Keywords: medical ethics, informed consent, children rights, paternalism

A review on policy constraints and strategies of supporting children as a vulnerable group

and providing equitable health services Fataneh Sadat Bathaei1, Farzaneh Zahedi2 1. Medical Ethics and History of Medicine Research Center, 2. Endocrinology and Metabolism Research Center, Tehran University of Medical Sciences Background: Health is a public good which is defined as complete physical, mental and social welfare. Establishment of systems providing appropriate and adequate health care is necessary to supply human beings health and is mostly provided by governments. Promoting health is the desirable and valuable outcome of investment in health programs and policies. World Health Organization emphasizes on equity, accessibility and acceptability of services as well as efficiency of providing care. Methods: The current study is a review article which attempts to investigate the studies on children's rights, their health necessities and different programs and strategies in various countries in this issue. Findings: Based on 24th item of children’s right convention, adopted by the general assembly of United Nations (UN) in 20th of November 1989, the countries recognize the right of children to access to highest standards of health care facilities in order to treat disease and restore health. These countries endeavor to ensure that no child is deprived of the right of access to such health care services. In this regard, different countries have adopted different strategies and programs to achieve the


Medical Ethics

goals set by the UN. One of these strategies is "integrated Management of Childhood Illness" which is the efforts to reduce childhood mortality due to conditions such as acute respiratory infections, diarrhea, measles, malaria, and malnutrition. This strategy was first used in 1996 with the goal of reducing under-five mortality rates in countries with infant mortality rate of 40 per 1000 live births. Another strategy is "Children's Health Insurance" that has a significant role in medical care and health outcomes. Ethical guidelines for protection of children as a vulnerable

group in clinical practice have been also compiled in many countries. Conclusion: Considering experiences of many countries, Iranian pediatricians and related organizations should define appropriate strategies to guarantee health care justice for children. Children rights can be also encouraged by hospital ethics committee via compiling appropriate ethical guidelines. Keywords: children rights, justice, health care goals, medical ethics

47

Physical health and developmental state of infants conceived by assisted reproductive

techniques by Royan Institute

Ramin Mozafari Kermani, Mohammad Reza Nateghi Jahade Daneshgahi, Tehran University of Medical Sciences Background: Neonates assisted by reproductive thechniques (ART) consist 2-4% of total number of neonates in developed countries. The aim of this study was assessment of physical and developmental health status of ART infants. Methods: Infants who borned by ART in Royan institute of Tehran were selected by non random consecutive method for a case series study. 400 infants were examed two times by a pediatrician and data were gathered by questionnaire which completed by a physician. Findings: From 400 infants, one third was preterm and low birth weight. One fifth of infants who had low birth weight were single. Anomaly of systems or organs were seen more in skin (11.2%), gasterointestinal system (6.7%) and urogenital system (6.7%). Involvement of these systems had higher incidence in preterm and multi fetal deliveries. Weight gain pattern of 4.7% infants from birth to 6 months old and 4.2% from 6 to 9 months old were less than -2SD of normal weight gain. Development in four fields of communication, fine motor, and gross motor and personal – social were assessed two times by Denver II test. 8.6% of infants had delay in one field. Gross motor delay were more common (4.2%) and were higher in ICSI, preterm and multifetal deliveries. Conclusion: Most ART infants had acceptable neonatal and infancy period. The most affecting factor of their health is multi fetal pregnancy, which results preterm delivery, Low birth weight and low height and head circumference. These are reported in single delivery of ART neonates more than single neonates of normal conception. Two third of these studied infants had normal physical exam and 6.7% had one of major anomalies which was higher in IVF infants. 8.6% of ART infants had delay in one of developmental field which had no significant difference with normal infants like other studies. Keywords: Infants, Assisted Reproductive Techniques, development, health status

Neonatal purpura fulminans: Diagnosis and

treatment; two case reports

Tahereh Esmaeilnia1, Faezeh Moghimpour Bijani2 1NICU of Valyasr hospital, pediatrics department. 2Mother, fetus and neonate research center of Valyasr hospital. 1,2 Tehran University of Medical Sciences

Purpura fulminans is called a hemorrhagic state which may be acquired or congenital. The former is caused by severe sepsis or infections, while protein C, S and antithrombin3 (AT3) deficiency underlies the latter. Tissue necrosis is the key injury in this disease which may lead to multiorgan failure and death. Hypoperfusion of extremities and subsequent tissue necrosis may necessitate amputation, a

common morbidity of this disease. Thus, the role of prompt and proper intervention in managing these patients is critical. Vasodilator agents e.g. nitroglycerine, are the main components of first-line medication. Improved perfusion and regressed ischemia are expected to be achieved through their administration. Fresh frozen plasma (FFP), heparin, concentrated protein C, platelet administration, IVIG, dextrane and plasmapheresis consist the second-line of treatment. Herein, two cases of neonatal purpura fulminans are presented. The first case developed the disease in the background of severe sepsis, while maternal homocysteinemia and 4G5G polymorphism were the underlying causes of the second one. Novel methods of treatment and their relative efficacy are compared. Keywords: Purpura fulminans, sepsis, protein C, FFP Resuscitation of preterm newborns with low

concentration oxygen versus high concentration oxygen

Amir Mohammad Armanian, Zohreh Badiee School of Medicine, Isfahan University of Medical Sciences Background: It is well known that a brief exposure to 100% oxygen for only a few minutes could be toxic for a preterm infant. The effectiveness of neonatal resuscitation was compared with low concentration oxygen (30%) and high concentration oxygen (HOG) (100%). Methods: Thirty-two preterm neonates were born in Isfahan Shahid Beheshti hospital with gestational age of 29-34 weeks who required resuscitation were randomized into two groups. The resuscitation was begun with 30% O2 in low concentration oxygen group (LOG). The infants were examined every 60-90 seconds and if their HR was less than 100, 10% was added to the previous FIO2 (fraction of inspired oxygen) until the HR increased to 100 and SO2 (saturation of oxygen) increased to 85%. In HOG resuscitation begun with 100% O2 and every 60-90 seconds, FIO2 was decreased 10 – 15% until the HR reached to 100 and SO2 reached to 85%. Findings: The FIO2 in LOG was increased stepwise to 45% and in HOG was reduced to 42.1% to reach stable oxygen saturation more than 85% at the fifth minute in both groups. At the first and third minutes after birth there was no significant differences between groups in heart rate and after 1, 2, 4 and 5 minutes after the birth there was also no significant differences in SO2 between groups, regardless of the initial FIO2. Conclusion: We can safely initiate resuscitation of preterm infants with a low FIO2 (approximately 30%) oxygen and then oxygen should be adjusted with the neonates needs. Keywords: Newborn, oxygen, resuscitation

The effect of different blood components on exchange transfusion outcomes

S Ghaemi, H Saneian, B Mo'ayedi, A Pourazar Isfahan University of Medical Sciences Background: Prescribing appropriate blood group for exchange transfusion makes an important role in patient's

Neonatology


Neonatology

outcome. The purpose of this study was to evaluate the effect of ABO compatible packed cell, dried O, and routine O groups on exchange transfusion outcomes. Methods: This multicenter clinical trial study is the combination of two studies which were conducted at Isfahan University of Medical Sciences, Isfahan, Iran. A hundred full term infants with more than 2.5 kg body weight, serum bilirubin >or= 20 mg/dl and confirmed ABO-Hemolytic Disease of the Newborn (HDN) were participated in first study. Among 40 infants, 20 underwent the exchange transfusion with O packed cell (group 1) and other 20 were transfused with O dried packed cell (Hematocrit = 90%) (group 2). In the second study with the same eligibility criteria with first study, among the 60 infants, 30 had exchange transfusion with O packed cell (group 3) and the rest were transfused with infant isogroup (group 4). Serum bilirubin and hemoglobin (Hb) were evaluated before and 6, 12, 24 and 48 hours after the exchange transfusion. Findings: The means of Hb after the exchange transfusion were 14.3 mg/dl in group 1, 15.62 mg/dl in group 2, 14.98 mg/dl in group 3 and 14.30 mg/dl in group 4 with significantly higher in group 2 compared with others (p = 0.02). The mean of the bilirubin after the exchange transfusion had no statistical significant difference among the four groups (p > 0.05). The mean of Hb and bilirubin before exchange transfusion had no statistically difference among all groups (p > 0.05). The mean of bilirubin before the exchange transfusion in infants who had two transfusion was significantly higher than the mean of the bilirubin before the exchange transfusion in infants with one time transfusion (p = 0.05). There was no significant difference among four groups in exchange transfusion frequency (p > 0.05). Conclusion: This study indicated that the level of bilirubin before exchange transfusion is the only important factor which sometimes causes the necessity of second or third exchange. Keywords: Routine packed cell, Dried packed cell, Exchange transfusion

A randomized controlled trial for pain reduction after vaccination of term- and near-

term newborns Fakhri Navaiee, Saeedeh Ghoreishi Isfahan University of Medical Sciences Background: Pain is an unpleasant feel and sensorial experience resulting from tissue damage. Newborn infants experience many of painful procedures such as vaccination injections, blood sampling, etc. Some recent studies have reported that breast-feeding or oral sucrose may reduce pain in neonates during painful procedures. In this study we compared the analgesic effects of breast-feeding, orally 20% glucose plus pacifier, and pacifier alone in after neonatal immunization injections. Methods: In this randomized controlled clinical, non-pharmacological and prospective trial 150 term or near term neonates were enrolled in the study. They were randomly classified assigned to 3 groups of equal number. The neonates of the first group, were breast fed before, during, and after the immunization injection (hepatitis B, BCG); the second group received oral glucose 20% (2ml or 1ml) followed by sucking pacifier, and the third group sucked pacifier. We assessed the pain response of the neonates by NIPS (Neonatal Infant Pain Scale) (NIPS) system, and we

monitored the heart rate and breathing pattern of newborns before and after immunization injections. Findings: The mean (standard deviation) of pain score was (2.26±2.078) in the first group, 3.00±2.347 in the second group, and 4.60±2.499 in the third group (P<0.001). Conclusion: The lower pain score in those newborns who were breast-fed before, during and after immunization injections than the other two groups is a confirmatory evidence of the usefulness of this intervention for reducing the pain among newborns. It should be considered in all painful procedures of neonates and infants. Keywords: Pain, immunization injections, breastfeeding, newborn infants, clinical trial

Soft tissue necrosis in neonates: a nursing problem

Seyed abdollah Mousavi Pediatric surgery department, Booali hospital, Mazandaran University of medical sciences, Sari Background: Sick neonates are particularly vulnerable to soft tissue injury but many of these injuries could be prevented if an adequate approach to monitoring and nursing is adopted. This article presents an initial approach to nursing care for soft tissue damages due to bed sore or peripheral IV infiltrations and to guide clinicians in management of these complications. Methods (cases): We describe a case series of cutaneous iatrogenic complications due to pressure ulcers or extravasation of drugs in neonatal groups. Specific therapies are suggested to management of these severe problems. Findings: Pressure ulcers were located on the head and cheek. Extravasation necrosis was mostly happened on the hand and ankle. Sever skin and muscle destruction, limb amputation and eventually death were some catastrophic complications that occurred due to malposition of umbilical arterial catheter. Most of the injuries were due to no satisfying nursing management and could be preventable. Conclusions: Bed sores and extra venous leaks can cause severe tissue injuries. Morbidity is increased by a delay in recognition and treatment of tissue ischemia. A protocol for the treatment of tissue damage is recommended. Keywords: tissue ischemia, neonate, extravasation The importance and effectiveness of vaginal delivery on neonatal immunity and disease

risk

Nastaran khosravi Tehran University of Medical Sciences The neonatal period is crucial for intestinal colonization. As the gastrointestinal tract of a fetus is near sterile, the newborn infant`s gut is undergoing colonization within a few days. Infants are therefore an open field for colonization by different types of bacteria. Gestational age, type of delivery and feeding affect the gut flora of young infants. For the colonization of infants born by cesarean delivery the environment is extremely important. In vaginally delivered infants, intestinal microflora acquires from maternal fecal and vaginal environment. Fecal colonization delayed in infants born by cesarean and significantly different from vaginally delivered infants. The different studies show the overall colonization rate of


Neonatology

bacteria was lower in the infants born by cesarean than in the vaginally delivered infants after birth. The rate of lactobacillus and bifidobacterium-like bacteria were higher in the vaginally delivered infants. The rate of C. difficile and Ecoli are higher in infants born by cesarean. Mode of delivery affects on neonatal immunity: Vaginal delivery has no effect on secretion of IL-13 and INF-γ, but there is an association between high secretion of these two cytokins and atopy or asthma in cesarean section. The different studies shows that the acute stress of labor maybe an immunologically beneficial process for the neonate. The labor is associated with fetal leukocytosis (selective for neutrophils, monocytes and natural killer cells). The studies show that the cesarean section is associated with a significant higher incidence of infectious disease during the first year after birth, and disease of respiratory, digestive and circulatory system. This could be due to a combined risk for them who experiencing (during the hospital stay) echological disturbance of intestinal colonization and interfered immunological factors. The intestinal colonization process in cesarean might play a role in development of food allergy and increased prevalence of atopic asthma and allergic disease. In many studies have been showed that the c-sectioned infants acquired S. mutans at a significantly younger age compared with the vaginally delivered infants and earlier colonization of S.mutans is significantly associated with higher incidence and more severe dental caries. Breast milk contains probiotics and prebiotic as dietary substances that promote the growth of beneficial gut flora particularly lactobacilli and bifodobacteria that capabale of conferring a health benefit to the host. Finally vaginal delivery and breast feeding strongly affect on neonatal intestinal colonization and influence the growth and development of neonatal immunity and decrease the incidence of NEC. Keywords: vaginal delivery, immun system, neonate

Comparison between measurement of

bilirubin by laboratory method and bilicheck in icteric neonatal infants

Masoumeh Hematyar, Mahtab Massahi

Islamic Azad University Tehran Medical Branch

Background: Hyperbilirubinemia is a common problem in the newborn infants. Severity and decision for its management are usually based on a serum bilirubin measurement which needs blood sampling. Transcutaneous measurement of bilirubin is a non invasive technique and its results correlates with total serum bilirubin. The aim of this study was comparison between measurement of bilirubin by laboratory method and bilicheck in icteric infants. Methods: This diagnostic study performed on 92 icteric neonates in Tehran Amir Al Momenin hospital in 2010-2011. In neonatas with indirect hyperbilirubinemia and without other problems a blood sample examined for measurement of bilirubin by laboratory and at the same time bilirubin measured by bilicheck at sternum area. The data analysed by SPSS software and correlation, regression tests. Also sensitivity and specificity of test was determined. Findings: The mean serum bilirubin was 14.5 ± 4.9 mg/dl by laboratory method and 14.7 ± 3.8 mg/dl by bilicheck. The sensitivity of bilicheck for detection of hyperbilirubinemia more than 17 mg/dl was 69%,

specificity was 85% and its accuracy was 79%. By correlation test there was a significant difference between two methods (P=0.01, r=0.7). Conclusion: Measurement of bilirubin by bilicheck has a relatively good sensitivity and specificity. So it is a safe method for measuring of bilirubin in neonates at risk of icter and also in follow up of bilirubin in hospitalized icteric neonates. Keywords: bilirubin, Bilicheck, neonatal jaundice

Effects of phototherapy on cytokines levels and white blood cells in Term neonate with

hyperbilirubinemia

Sedigheh Jahanshahifard Imam Reza Tamine Ejtemaei hospital, Urmieh

Phototherapy is the most common treatment used for severe jaundice. There is increasing evidence that phototherapy can directly affect the expression and function of cell surface receptors including adhesion molecules, cytokines, and growth factor receptors. The aim of this study was to investigate the effect of phototherapy use on the levels of interleukin (IL)-1α, IL-6 and tumor necrosis factor (TNF-α) as cytokine expressions from keratinocytes as well as white blood cell count in the treatment of neonate with hyperbilirubinemia. Methods: We studied 32 term newborns with hyperbilirubinemia. Blood samples were obtained before and 72 hrs after phototherapy. Serum levels of IL -1α, IL-6, TNF-α and WBC count were measured in the samples using appropriate methods. Findings: Serum TNF-α at 72 hr of exposure to phototherapy increased while levels of IL -1α and IL-6 at the same time were decreased. These changes were not statistically significant. WBC count rose significantly with phototherapy. Conclusion: Phototherapy in term neonate doesn’t affect cytokines level, but can raise peripheral WBC count. Keywords: term neonate, hyperbilirubinemia, phototherapy, cytokine

Evaluation of Auditory Brainstem Responses (ABR) in healthy term infants with elevated

bilirubin levels requiring exchange transfusion or were treated with exchange

transfusion

Seyed Saeed Nabavi, Mohammad hosein Behzad Moghadam, Mohammad hosein Arab Hoseini, Mahnaz Vaezi, Reza Rajabi Islamic Azad University

Background: Hyperbilirubinemia is a common cause of neonatal disease. Severe hyperbilirubinemia is a risk factor for auditory system injury. We performed this study to determine the usefulness of auditory brainstem responses (ABR) in early diagnosis of hearing impairments in healthy term infants with elevated bilirubin levels requiring exchange transfusions or treated with exchange transfusions. Methods: During a two-year period (2007 – 2009), in a prospective descriptive analytic study, 64 (32 females, 32 males), healthy term (> 37 weeks) infants who required


Neonatology

treatment or were treated with phototherapy or exchange transfusions for elevated bilirubin levels or jaundice, were studied. After obtaining consent from parents post treatment, infants were tested with auditory brain responses (ABR). Then the results were analyzed using SPSS 16 software. Findings: No significant correlation was seen between ABR with age, weight, bilirubin level or ABO group. 19 of 64 infants were treated with exchange transfusion, 3 of 19 infants (16%) showed abnormal ABR and 16 of 19 infants (84%) had normal ABR. No significant correlation between exchange transfusion and ABR was showed (p<0.05). Conclusion: These results showed that 14% of infants with elevated bilirubin who required exchange transfusion had abnormal ABR. This finding indicates that elevated bilirubin level without kernicterus requiring exchange transfusion is an important risk factor for hearing impairments and may lead to abnormalities in hearing tests. Although it is unclear, how long these tests remain abnormal, which requires further research. Keywords: Infant, hearing loss, exchange transfusion, auditory brainstem response, oto-acoustic emission (OAE)

Large nasal teratoma associated with

congenital heart anomalies in a neonate, a case report

Kamyar Kamrani, Seedebrahim Tabatabaiepoor Bahrami Hospital, Gilan University of Medical Sciences

Background: Teratoma is an encapsulated neoplasm which can be derived from all three embryonic layers. During early childhood, %40-50 of cases take place in sacrococygeal region. Case Presentation: A one-day old neonate was referred to our hospital due to respiratory distress and an enlarged facial mass. A MRI Scan showed a large mass in anterior part of the face, and echocardiographic investigation revealead severe tricuspid atresia, atrial septal defect, ventricular septal defect and pulmonary stenosis. Patiant was undergone surgery. Conclusion: This case demonstrates an unusual presentation of head and neck teratoma. Keywords: teratoma, congenital heart anomaly, neonate

Omphalocele and ectopia cordis in a neonate

with Pentalogy of Cantrell

N Taee, M Faraji Godarzi, F Tarhani Department of pediatrics, faculty of medicine, Lorestan University of Medical Sciences Background: Pentalogy of Cantrell is very rare congenital anomaly that presented with severe thoracoabdominal defect, omphalocele, lower sternal defect, anterior diaphragmatic defect anterior pericardial defect and ectopia cordis. Presentation of Pentalogy of Cantrell is variant from uncompleted to severe form with other organ involvement. Trisomy of 18, triploidy and other chromosomal abnormalities reported with Pentalogy of Cantrell. Pathogenesis is unknown, mesodermal developmental defects in 14-18 days after conception maybe responsible. Case presentation: A 6-hour newborn with large omphalocele and pulsatile mass above that reported. She was born from unrelated parents. Pregnancy was uncomplicated and there was no history of

polyhydramnious. In physical examination, she had respiratory distress. Spleen was in left side and liver was in right side and small intestine and colon were in middle portion of the omphalocele. In paraclinic, cardiac echocardiography reveals Tetralogy of fallot. Finally she died before 24 hours with cardiopulmonary arrest. Conclusion: All newborns with omphalocele must be evaluated for Pentalogy of Cantrell. Keywords: Pentalogy of Cantrell, omphalocele, ectopia cordis, Khorram Abad Evaluation of frequency of hypoxic- ischemic-

encephalopathy and it’s risk factors in neonates born in Fatemieh hospital-

Hamadan –Iran Behnaz Basiri, Maryam Shokoohi, Mohammad Kazem Sabzehie Hamadan University of Medical Sciences Background: Hypoxic-ischemic encephalopathy (HIE) is one of the most important causes of permanent damage to brain tissue that redounds to mortality and/or late sequelae such as cerebral palsy or delayed neural development. 15-20% of HIE cases die during neonatal period and 25-30% of whom survive suffer from neural development problems. The incidence of HIE is approximately 6 in 1000 term infants. Etiologies of perinatal hypoxia-ischemia include: maternal, placental, fetal and neonatal factors. In term infants, 90% of asphyxia events occur in the antepartum or intrapartum period. We designed this study in Fatemieh hospital that is a referral tertiary maternal-neonatal center for identifying the incidence of HIE and its risk factors. Methods: This is a retrospective descriptive cross- sectional study that performed in NICU of Fatemieh hospital, Hamadan, Iran between 2005- 2007. All of neonates with the diagnosis of HIE were included in the study and maternal, fetal and neonatal risk factors were founded from patient’s sheets. Findings: There were 17392 live births in Fatemieh hospital between 2005-2007. Eighty three cases of HIE were known; overall incidence of HIE was 4.7 in 1000 live births. Mortality rate was 43%. Maternal risk factors in 26% and neonatal risk factors in 23% of cases were known. Conclusion: With identifying of the etiologies and predisposing factors of HIE and control of them, we can reduce the incidence. Attendance of skilled personnel in neonatal resuscitation at the time of delivery is an important factor in reducing incidence of HIE. Keywords: neonate, Hypoxic-Ischemic Encephalopathy, perinatal asphyxia

Clinical findings resulted to diagnosis of

sepsis in patients of Booali &Imam Khomeini hospital, 2010

Roya Farhadi, Azar Kabirzadeh, Benyamin Mohseni Saravi

Mazandaran University of Medical Sciences

Background: Neonatal mortality is an important issue in health care system. One of the important diseases in neonatal period is sepsis. Clinical sign and symptoms in addition to lab tests and paraclinic exams are the most important way to accurate diagnosis and prevention of


Neonatology

mortality. This study was conducted with the aim of determining the most clinical sign and symptoms which resulted to diagnosis of sepsis. Methods: This is a descriptive cross-sectional study. The medical records of patients hospitalized in hospitals of Mazandaran University of Medical Sciences during 2010-2011 were reviewed. Variables were age, sex, birth and admission weight, clinical sign and symptoms, methods of delivery, admission and discharge condition, discharge status, the time elapsed between showing the symptom and admission to hospital, gestational age and the result of cultures. The data were recorded in a checklist and analyzed with SPSS and descriptive statistics. Findings: Results showed that 120 patients discharged during 1 year with diagnosis of sepsis. Discharged status of 27 (%22/5) were dead. Median age was 1 day with 8 hours SD, length of stay were 12±1 days, gestational age was 34±3 weeks and median birth weight was 2477±977 grams. The median time elapsed between showing the symptom and admission to hospital was 38±31 hours. 3 cases (%5/8) had leukocytosis; blood culture in 10 (%8/3) and urine culture in 8 (%7/6) patients were positive. Lumbar puncture Culture of 50 patients (%50) was negative and the rest of them weren’t punctured or the puncture weren’t successful. The frequent signs in patients were respiratory distress, poor feeding and lethargy. Result of culture showed that the E coli was grown. Conclusion: In this research clinical signs of sepsis were the same as the other articles and reference books. Of course the results can’t show which one was started first or the second because of medical history sheets don’t show this process. If it is necessary to know which one was the first started, should the forms revise. Keywords: sepsis, clinical findings, correct diagnosis, medical history

Neonatal seizure: Etiology, clinical type and

outcome Mohammad kazem Sabzehei, Behnaz Basiri, Ebrahim Davar Hamedan University of Medical Sciences Background: Seizures in the neonates are quite different from those in older childs and adults. The objective of this study was to determine the etiology, clinical type and outcome of seizure in hospitalized neonates. Methods: In this retrospective study, over a period of 3 years from September 2008 to September 2011, we assessed for seizures all neonates (Age 0-28 days) admitted to the besat hospital, hamedan. Data were collected from hospital records analayzed using spss 12. Findings: Seizures were reported in 102/1112 (9.1%) of neonatal admission. Among the neonates with seizure 57% were males and 23.5% were pretem. Regarding the type of seizure, subtle seizure occurred in 38.2%, tonic in 29.4%, clonic in 26.4% and myoclonic in 5.9% cases. The main diagnosis in neonates with seizures were hypoxic- ischemic encephalopathy 34.3%, infections 24.4%, intracranial hemorrhage 6.9%, hypoglycemia 5.9%, hypocalcemia 2.9%, inborn error of metabolism 1% and in 24.5% of cases the cause was unknown. Mortality rate was 14.7%. Conclusion: Neonatal seizure indicates significant underlying disease. HIE was the most common causes of neonatal seizure in our study.

Keywords: neonatal seizure, etiology, clinical type, outcome

Rate and type of error in documentation of

causes of perinatal mortalities in Sari hospitals, according WHO rules (a 5 years

study) Aliea Zamani Kiasar, Azar Kabirzadeh, Najmee Norozi, Esmailel Rezazadeh, Benyamin Mohseni Saravi Mazandaran University of Medical Sciences Background: Perinatal mortality rate is one of the important health care criteria in each society. In order to prevent this problem we should first try to find causes. So we need some sources to give us information to analyze. One of these sources is death certificate, the papers filling them became a legal obligation in hospitals since a couple of years ago. So the accuracy of death certificates is very important in order to give us correct knowledge. We studied the probable faults and errors in completing these forms and categorized them according to the WHO rules and guidelines. Methods: This is a study based on existing data that have been collected from 2003 to 2008. Information collected by filling a type of questionnaire that has been designed by the researcher based on ICD-10 and WHO guidelines. We used SPSS-19 software for analyze data. Findings: Total number of perinatal death was 269. 12 (4.5%) deceased patients didn’t have any death certificate in their hospital documents. Just 10 (4%) out of 257 death certificates have been filled completely correct. The most common error (49%) was error N.3 (not writing causes of death in A or C lines). Error N.1 (writing causes like cardiac failure, asphyxia, anoxia & prematurity in line A) and error N.2 (writing two or more causes in lines A or C simultaneously) were 23% and 15% respectively. Conclusion: For building a path in order to improving the perinatal care and reducing death rate we need to have more knowledge and correct information as well as a bright vision especially about causes of death. One of the biggest steps is to collect reliable information and document them accurately. Keywords: perinatal death, death certificate, causes of death, Error, medical coding, mortality tabulation, WHO

Determining frequency of asymptomatic hypoglycemia in high risk neonate in fatemieh

hospital from Apr to Sep 2006 Maryam Shokoohi, Behnaz Basiri Hamadan University of Medical Sciences Background: Hypoglycemia is a common metabolic disorder in a neonate. It is commonly associated with a variety of conditions like prematurity, IUGR, Infant of diabetic mother and LGA. Hypoglycemia may be symptomatic or asymptomatic in newborn.The objective of this study was to determine the frequency of asymptomatic hypoglycemia in high risk neonates. Methods: In this prospective cross-sectional study 83 hypoglycemic high risk neonates were assessed for determining frequency of asymptomatic hypoglycemia in Fatemieh hospital from Apr to Sep 2006.


Neonatology

Findings: Total frequency of asymptomatic hypoglycemia was 49.4 in hypoglycemic high risk neonates. Mean of blood suger value in asymptomatic group was higher. Gestational age, APGAR score and early feeding after birth had significant relationship with asymptomatic hypoglycemia whereas birth weight, sex, pariety, route of delivery, mother age and disease and infusion of glucose to mother during labour were ineffective factors. Keywords: neonate, asymptomatic hypoglycemia, high risk neonate

Evaluation the major and underlying causes of mortality of the neonates according to the

International Coding of Disease Ver 10 Fatemeh Eghbalian Hamadan University of medical sciences Background: According to the fact that the reduction of Neonatal Mortality Rate is correspondent to improvements in health and hygienic status of the society, understanding the major neonatal mortality causes will help the society to plan better prenatal and neonatal care systems. Methods: On this retrospective study, the major causes of the mortality of the neonates were extracted from files of BESAT hospital according to the International Coding of Disease Ver 10 (ICD10). Data were analyzed in proportion to gestational age, birth weight, gender and neonatal age. Findings: Mortality rate was higher during the first week of life (51/1%). Generally five main causes of death were: Neonatal cardiac dysrythmia, bacterial sepsis of newborn, atresia of esophagus without fistula, septic shock and respiratory distress syndrome. Five accessory causes of death were: bacterial sepsis of newborn, respiratory distress syndrome, preterm labor, congenital heart disease and acute respiratory distress syndrome of newborn. The main causes of death in these study were: congenital disease and infections (septicemia and Pneumonia). Data analysis indicated no significant relationships between death and gestational age, neonatal age and birth weight and it was not related to gender. Conclusion: With due attention to the easy application of ICD 10 for determining the major and underlying causes of death of neonates and the fact that autopsy is not routinely applicable in neonatology wards, routine using of ICD 10 for classifying the causes of death in death certificates is highly recommended. Keywords: neonatal mortality, International Coding of Disease

Anterior fontanelle size in healthy Iranian

neonates on the first day of life Hamideh Shajari, Neda Rashidiranjbar, Mahmoodreza Ashrafy Tehran University of Medical Sciences Background: There is limited data in the literature on the normal size of the anterior fontanelle. This cross-sectional study was to determine normal values of anterior fontanelle size on the first day of life using standard methods. Methods: Anterior fontanelle size was measured in 400 term and healthy neonates delivered at the Shariati Hospital, Tehran, Iran. Examination included assessment of

head circumference, anterior fontanelle size, weight and length. Type of delivery was recorded. Findings: The mean size of anterior fontanelle was 25.34 ± 13.27 mm, and it was established in both genders, 26.70 ± 13.19mm in boys, and 23.67 ± 13.20mm in girls. A significant difference between the mean anterior fontanelle size in boys and girls was found (P value = 0.023). There was no significant difference in anterior fontanelle size between the infants born with a normal vaginal delivery and those born with cesarian-section (P value = 0.08). There was found a significant negative correlation between the mean size of anterior fontanelle size with both weight and lenght (P value ≤ 0.05). No significant correlation was found between mean size of anterior fontanelle and head circumference or with gestational age of infant (P value ≤ 0.05). Conclusion: Our results proved possible to define a reference range and centile chart. The method used is simple and accurate and easy to use in the routine neonatal examination. Keywords: anterior fontanelle, neonate, routine neonatal examination, normal size

Socio-economic and medical determinants of

low birth weight in Iran: 20 years after establishment of a primary healthcare

network Fatemeh Jafari1, Javad Mousavi2, Hasan Eftekhar3 1Department of public health. 2Research and education unit, Zanjan blood transfusion organization. 3Department of health education and promotion. 1Health school, Zanjan university of medical sciences. 3Tehran university of medical sciences Background: Establishment of a primary healthcare network in Iran has provided free and universal access to primary health care. Although the health status of Iranians has improved since this network was established, the low-birth weight rate has not decreased. The objective of the present study was to describe socio-economic and medical factors related to low birth weight in the context of free and universal access to primary health care. Methods: This was a hospital-based prospective study. Data about socio-economic, reproductive and prenatal condition of 4510 live singleton births from June to October 2004 were gathered using a standard questionnaire by interview and record review. The effect of these conditions on birth weight was investigated using a logistic regression model. Findings: Of 4510 newborns, 305 (6.8%) were low birth weight. Among these low-birth weight newborns, there were 159 preterm and 146 term newborns. Mothers with a primary and secondary education [odds ratio (OR) 6.83, 95% confidence interval (CI) 2.35–7.34 and OR 4.81, 95%CI 1.95–6.37, respectively], who lived with farmer and unskilled worker husbands (OR 2.52, 95%CI 1.12–4.66 and OR 2.91, 95%CI 1.35–2.52, respectively), with a birth interval of 1 year or less (OR 3.54, 95%CI 1.80–5.95) and height less than 155 cm (OR 1.82, 95%CI 1.12– 3.31) were more likely to have low-birth weight infants. Conclusion: In the context of free and universal access to health care, it is recommended that policy makers should place more emphasis on education as it imparts knowledge and thus influences dietary habits and birth-spacing


Neonatology

behavior. This will lead to a better nutritional status, particularly in dealing with pregnancy, resulting in lower rates of low birth weight. Keywords: low birth weight, primary health care network, socio-economic, medical

Neonatal resuscitation: challenges in low-

resource settings Mliheh Kadivar Department of Pediatrics, Tehran University of Medical Sciences Background: The rapid progress of medical technology in perinatal-neonatal medicine has been resulted in increased number of maintaining the life of infants in serious and potentially life threatening situations with uncertain or even poor prognosis in future. During past decades with improving in perinatal and neonatal technology, admissions of premature and critically ill newborn infants to the NICU have been increased dramatically. Also survival rates of these infants have been increased, but further disabilities and the quality of life are still the main issues. Vitality comparing to viability is a main challenges in this regard. Nowadays, caregivers not only ask about viability, but also look at the long term neurodevelopmental outcome. Gestational age, along with birth weight and major congenital anomalies are resulted in higher mortality and morbidity. According to these issues in some developed countries there are some guidelines to not initiation of resuscitation when high morbidity is likely among the rare survivors such as confirmed gestational age of less than 23 weeks or a birth weight of less than 400 gr, anencephaly, lethal genetic disorder or malformation based on available data support high mortality rate or severe disability in future. While, upon parental request exceptions may be considered in special situations. Meanwhile, the ethical issues of neonatal resuscitation are not different comparing to others. The four main ethical principles; “autonomy, beneficence, nonmaleficence, and justice” should also considered the same as other medical situations. The key point is the neonates cannot communicate and express their desires. Parents have the major responsibility in this regard that should get inclusive and reliable information for further decisions. Based on the statement from the American Medical Association (AMA) Code of Medical Ethics': what is best for the newborn should be the crucial concern for decisions regarding life-sustaining treatment for critically ill newborn infants based on the result of therapy, risks of treatment vs non treatment, extension of therapy, pain and discomfort accompanied with therapy, and further quality of life. In many modernized countries nowadays “withhold or withdraw resuscitation efforts would be acceptable and considered ethical and legal if the parents and health care professionals agree that further medical intervention would be futile, would merely prolong dying, or would not offer sufficient benefit to justify the burdens imposed”. In these countries well organized system with multidisciplinary team have been established to support families. Unfortunately in limited recourses settings in the devolving countries there are many challenges in this regard such as; inappropriate communication between health care providers and parents, lack of transforming information to parents, lack of certain guideline for decision making, no proper ethical education to health care givers, inadequate health care providers, and unsuitable team-working. In addition,

limited resources as enough beds in intensive care settings, instruments such as mechanical ventilators, educated nurses, expert physicians, neonatal transport system all weighted on this problem. Discussion about health resource allocation should take wisely and diverse perspectives outside of healthcare professions such as epidemiological, economical, ethical, legal, and emotional points should be considered. It is especially very important in the devolving countries with higher birth rates and also mortality rates. Approximately 10 million neonates do not breathe immediately at birth which about 2/3 of them needs basic neonatal resuscitation. On the other hand, each year approximately 4 million newborn infants die after birth mostly in the developing countries. It is very important to look deeply to the impact of neonatal resuscitation, particularly on long-term outcomes in low recourses settings. Conclusion: So, new framework of practice in the intensive care setting needs implementation of “widespread knowledge and reflective application of ethical, medical, sociological, biological, and psychological principles in highly stressful environments and highly charged situations”, along with improved perinatal counseling. Keywords: ethics, neonate, neonatal intensive care, resuscitation

Evaluation of the therapeutic effects of

Iiduced local hypothermia in neonates with Hypoxic-Ischemic Encephalopathy

K Vakili Garmroodi, B Basiri, F Eghbalian Hypoxic-ischemic-encephalopathy (HIE) is one of the most important causes of permanent damage to brain such as cerebral palsy and mental retardation. Hypothermia or lowering temperature of brain or total body is a new and promising treatment. Present study was done to assessthe therapeutic effects of induced local hypothermia in HIE in Hamedan. Methods: Present study was performed as a randomized clinical trial upon 36 neonates who had criteria to be imported into the study. In first 6 hours after birth, neonates were randomly classified into two 18 person groups. In control group (CoG), neonates had routine treatments that consisted of preservative measures and if necessary anti-convulsive treatments. In case group (CaG), neonates received induced local hypothermia for 6 hours in addition to routine therapeutic managements.The data were analyzed with SPSS. Findings: 72.7% of neonates of CaG and CoG were males. There was no significant difference between two groups in sex, birth weight, gestational age and perinatal obstetric complications. Mean duration of admission was 7.72±4.23 days in CaG and 10.06±5.99 days in CoG, and there was no significant difference between two groups (P=0.199). Mean date of starting feeding was 3.44±3.11 and 4.53±2.74 days in CaG and CoG respectively; Hence there was no differences statistically (P=0.737). Mean date of becoming conscious was 3.72±3.19 days in CaG and 4.80±3.34 days in CoG, and there was no statistical significant differences between two groups (P=0.606). 16.7% of neonates in CoG and 50% of neonates in CaG had seizure, that this difference was significant statistically (P=0.038). Three neonates who received only routine managements died, whereas no death was observed among patients receiving hypothermia. By using statistical test, no difference was


Neonatology

seen in mortality rate between two groups (P=0.114). Conclusion: In present study, seizure rate among patients receiving local hypothermia alone was significantly lower than patients receiving routine managements, so it is suggested to perform local hypothermia as a therapeutic measure to reduce seizure rate. Keywords: hypoxic-ischemic encephalopathy, induced local hypothermia, seizure

Management and detection of G6PD

deficiency in newborn screening program in Shahid Beheshti University of Medical

Sciences between March 2010 to February 2011

Jalil Darozi, Faranak Rahimi, Ali Golmohammadi Shahid Beheshti University of Medical Sciences Background: Glucose-6-phosphate dehydrogenize deficiency is a genetic disorder that occurs most often in males. This condition occurs most frequently in certain parts of Africa, Asia, and the Mediterranean. The north and south of Iran this disorder is common. Methods: Newborn screening for detection of genetic and inherited disorders have started in Iran of 2007 in some medical science university. Total Newborns referred to the newborn screening centers. Health workers in those centers got four drop blood of heel of the newborn on the gatry piper and sent to university central laboratory for detecting G6PD deficiency. Samples were screened for the quantitative measurement of G6PD activity by enzymatic colorimetric assay by a special kit. The newborns between 3 – 5 days after birth have referred to 231 delivery units and newborn screening centers of the shahid beheshti university of medical sciences. The newborns with G6PD deficiency after 120 days of life sent to laboratory for detecting of quantitative measurement of G6PD activity again. we must educateto fathers and mothers of the patients for prevention of using drugs and foods that cause break down of red blood cells after confirming G6PD deficiency in newborn screening program. Findings: Between February to March 2011 in delivery units and newborn screening centers of the shahid beheshti university of medical science, 123332 newborns detected and after sending to university central laboratory for detecting G6PD deficiency 2325 newborns were suspected (%1/9 of total screening). 326 newborns were females (%14) and 1999 newborns were males (%86). Then after confirminf for G6PD deficiency in 120 days of life with quantitative measurement, 1488 newborns were confirmed (%1/2 of total screening). 179 newborns were females (%12) and 1309 newborns were males (%88). Conclusion: 1- lots of case founded and positive cases were males and in this study rate of male to female was 7/1. 2- In the newborn screening 64 % of suspected samples after 120 days of life with quantitative measurement were confirmed. Therefore special education for family and relatives with suspected neonate is very important and useful, because more than half of them had confirmed. 3- Public education is very important for detection and prevention of complication of Glucose-6-phosphate dehydrogenize deficiency (fauvism). Keywords: newborn screening,newborn screening centers, glucose-6-phosphate dehydrogenize (G6PD)

The relationship between behavioral hearing thresholds and estimated ASSR thresholds in children with Neonatal hyperbilirubinemia

Rasool Panahi1, Zahra Jafari2 1Department of Audiology, 2 Department of Basic Sciences in Rehabilitation. 1,2Faculty of Rehabilitation, Tehran University of Medical Sciences Background: Increased serum bilirubin levels during infancy increases the risk of hearing loss in infants. This study aimed to investigate the relationship between pure - tone hearing thresholds and estimated ASSR thresholds in children with a history of neonatal hyperbilirubinemia and evaluate the usefulness of ASSR in estimating hearing thresholds in children. Methods: This study was conducted on 19 children (10 girls and 9 boys) aged between 2.4 to 11 years with a history of neonatal hyperbilirubinemia more than 17 mg / dL. ASSR thresholds were compared with behavioral thresholds and were interpreted given the amount and type of hearing loss. Findings: From 19 studied children, 7 had normal hearing thresholds and 12 had varying degrees of sensorineural hearing loss. Generally high correlation (r ≥ 0.92, p <0.01) was found between ASSR and behavioral thresholds. The highest correlation was observed at a frequency of 4000 Hz (r = 0.96, p <0.01). There was no significant difference (p> 0.11) between behavioral and ASSR thresholds at audiometric frequencies in both hearing impaired (p>0.11) and normal hearing children (p> 0.13). Conclusion: The results of this study showed that the ASSR assessments provides reliable estimates of behavioral hearing thresholds in children with sensorineural hearing loss and normal hearing with a history of neonatal hyperbilirubinemia. Keywords: neonatal hyperbilirubinemia, audutory steady-state response, child, behavioral audiometry

Probiotic for prevention of necrotizing

enterocolitis in preterm infants Negar Sajjadian Tehran University of Medical Sciences Background: Necrotizing enterocolitis (NEC) is the most common serious acquired disease of the gastrointestinal tract in preterm infants. It is characterized by bowel wall necrosis of various length and depth. Probiotic bacteria are live microbial supplement that colonized the gastrointestinal tract and potentially provide benefit to the host. The most frequently used probiotics are lactobacillus and bifidobacterium. The primary objects was to compare the effectiveness and safety of prophylactic enteral probiotic administration versus placebo or no treatment in prevention of severe NEC (stage II or more) and / or sepsis in preterm infant. Methods: Only randomized and Quasi–randomized controlled trial were included. Preterm infant <37 weeks and/or birth weight <2500g enteral administration of probiotic at any dose and preparation for more than seven days compared to placebo or no treatment. The standard method of Cochrane neonatal review group guidelines were employed in creating this update.


Neonatology

Findings: Sixteen included studies reported outcome on 1371 infants treated with probiotics and 1376 control infant. It was showed that enteral administration of probiotics reduces the incidence of severe NEC (stageII-III), the number of deaths and NEC related mortality was significantly lower in the probiotic group and significant reduction in hospitalization days. There was significant reduction in time to reach full enteral feeds. There was a trend toward a benefit in reduction of sepsis however this did not reach statistical significance. Conclusion: More studies to address the precise efficacy in ELBW infant, the optimal preparation, dosing and duration of therapy are still needed. Keywords: probiotic, necrotizing entrocolitis, infant Comparison of the effect of early Surfactant

administration during NCPAP treatment with administrating of NCPAP alone on

mortality, morbidity and complications of RDS in premature infants: A randomized

controlled trial

Maryam Nakhshab1, Mehdi Tajbakhsh1, Soghra khany2, Mohammad khedemlou3 1department of Pediatrics.2faculty of nursing. 3department of Community Medicine.1,3 faculty of medicine. 1,2,3Mazandaran University of Medical Sciences

Background: Respiratory distress syndrome (RDS) is one of the leading causes of death in premature neonates. Early administration of nasal CPAP is applied as a noninvasive method in management of this disease and it seems to be promising. Also early surfactant administration is effective in reduction of RDS severity and mortality but the studies

on this form of surfactant administration have used it in the first hours after birth but in many centers the patients have been transported from far hospitals and are reached at a later age. Is this method of administration of surfactant beneficial in these situations? The aim of this study was to compare the effect of administration of surfactant during nasal continuous positive airway pressure (nCPAP) with only early CPAP administration for premature infants with RDS. Methods: This was a controlled randomized clinical trial on 60 premature neonates who were transported to the NICU of Boo-Ali Hospital. Neonates with gestational age less than 35 weeks, age equal to or less than 12 hours and the presence of symptoms of respiratory distress syndrome were placed on nCPAP and then randomly assigned to intubation, administration of surfactant and then extubation to nCPAP or treatment with nCPAP alone. The primary outcome was the need for intubation and mechanical ventilation on the basis of our defined criteria and the secondary outcomes were the neonatal mortality and other complications of RDS. Findings: In 13 months, 60 eligible neonates were randomly assigned into two groups of treatment and control. Our participants in surfactant group received surfactant in the mean age of 5.1 hour of age. The need for mechanical ventilation was statistically similar between two groups. There was no statistically difference among groups for rate of mortality or other complications of RDS. Conclusion: Early nCPAP alone is effective in treatment of RDS and because it is less dangerous and expensive than intubation and surfactant administration, in situations where it is not feasible to administer early rescue surfactant in the first hours of life it could be a more preferable way for treatment. Keywords: surfactant, continuous positive airway pressure, respiratory distress syndrome, neonate

56

Mean random urine albumin and IL8 in children with vesicoureteral reflux(VUR) and

those with improved VUR

Hamidreza Badeli1, Termeh khoshnevis2, Afagh Hassanzadeh Rad3, Mehrdad Sadeghi4 1,3 pediatrics research center. 4Reference Laboratory. 1,2,3,4Gilan University of Medical Sciences Background: Vesicoureteral reflux (VUR) is a risk factor for urinary system in children with urinary tract infection(UTI) and it can induce permanent adverse effects such as kidney scar, hypertension and ongoing declining renal function. Although up to now plenty efforts have done to elucidate renal involvement in UTI and cytokines are well known to modulate the inflammatory response in UTI and renal damage, however less is known on their role after acute infection has resolved.The aim of this study was to determine Mean Random Urine Albumin and IL8 in children with VUR and those with improved VUR. Methods: In this cross-sectional study which was performed in 17 shahrivar children hospital in Iran, urine samples of 51 children with VUR(A), recovered from VUR(B)and normal(C) were collected. Inclusion criteria for groupA and B were age between 2-10 years, no history of UTI during 3 months,diagnosed reflux or recovery of reflux and normal kidney function. Data such as age, sex, unilateral or bilateral reflux was extracted from files. The severity of reflux was defined by VCUG or DRNC and urine Microalbumin(MA), Creatinine(Cr) and interleukin8(Il-8) were measured in a single laboratory. Findings: 51 children with the mean age of 5.725± 2.50 were entered in this study. In group A, mean duration between reflux diagnoses and study entrance was 30±9.1 and in group B mean duration between reflux diagnoses until recovery was 19.9± 1.3 months. Furthermore results obtained no significant difference according to MA, MA/Cr, IL8 and IL8/Cr in groups. Conclusion: According to results, there is no evidence that MA is good indicator of ongoing injury in VUR and improved VUR after 3 months of last UTI and although in different studies urine IL-8 levels were elevated in a variety of infectious and non-infectious urinary tract conditions but may serve as a sensitive but not specific screening biomarker of urinary tract diseases. Keywords: urine albumin, interlukine 8, vesicouretral reflux, urinary tract

Outcome of children with end stage renal

disease in north-west of Iran over the past ten years

Fakhrossadat Mortazavi, Majid Maleki Tabriz University of Medical Sciences Background: Outcome of end stage renal disease (ESRD) in children has considerably improved since the development of dialysis and kidney transplantation. The aim of this study was to evaluate the management and outcome of ESRD children in a tertiary care hospital in northwest of Iran.

Methods: Medical records of children less than 16 years of age with ESRD (glomerular filtration rate less than 15 ml/min/1.73 m2) who were admitted in children's Hospital of Tabriz from October 1999 to October 2009 were reviewed retrospectively. Findings: Ninety four children with ESRD including 51 boys (%54.3) and 43 girls (%45.7) with a mean age of 7.9±3.49 years were studied. Fifty five patients (58.5%) had already reached ESRD at first presentation. Etiology of ESRD included: Congenital urologic anomalies (35.1%), glomerular diseases (24.5%), hereditary nephropathies (21.3%), systemic diseases (7.4%) and unknown etiology (11.7%). Eighty patients underwent renal replacement therapy(RRT) and were followed for a mean period of 4.86±2.77 years(range: 5 months- 9.5 years). Initial mode of RRT was hemodialysis in 81.25%, continuous ambulatory peritoneal dialysis in 16.25% and preemptive kidney transplantation in 2.5%. Thirty two patients (34%) underwent renal transplantation. The mean duration of staying on dialysis before transplantation was 12.4±11 months. The 1 and 3 year graft survival rates were 81.2% and 68.8% and the 1 and 3 year patient survival rates were 96.9% and 93.8%, respectively. Thirty one patients died (33%). The mortality of girls was significantly higher than boys (P= 0.04). There was a significant negative correlation between age and mortality (P=0.01). Heart failure was the most common cause of death followed by infections. Conclusion: In spite of financial support by governmental resources, ESRD children in our area have a poorer outcome in comparison with developed countries. Current high mortality rate will improve by timely referral, optimal utilization of all modalities of RRT and increasing transplantation facilities. Keywords: End stage renal disease, children, outcome, Renal replacement therapy Developmental origin of hypertension: focus

on prevention Farahnak Assadi Department of Pediatrics, Section of Nephrology, Rush University Medical Center, Chicago, Illinois, USA Essential hypertension (EHTN) is a major public health problem worldwide. EHTN is estimated to affect as high as 11% of children and adolescents worldwide. It imposes a large burden on society that is growing larger in terms of mortality or morbidity, quality of life, and costs. Children with EHTN are at increased risk of cardiovascular (CVD) and chronic kidney disease (CKD). Data indicating that EHTN has its origins during development and environmental chemicals exposures play a major. Risk factors for the development of EHTN include low birth weight, family history of EHTN, CVD or CKD, obesity, physical inactivity, high dietary fat and salt intake, low dietary potassium and calcium intake, chronic use of contraceptives, and use of illicit drugs. EHTN is not being detected early enough to initiate treatment regimens to reduce death and disability. Evidence suggests that preventive measures could substantially reduce the HTN burden and that such strategies are not yet in place. The 24-hour (ABPM) ambulatory blood pressure monitoring

Nephrology &Urology


Nephrology & Urology

(ABPM) allow for a more comprehensive blood pressure (BP) assessment. Children and adolescents should have their BP measured at least once per year. Diet high in fruits and vegetables and low-fat dairy products lowers blood pressure more than a sodium-restricted diet. Initiatives should be undertaken to make healthcare providers, policy makers, and patients more aware of the seriousness of EHTN, its risk factors, and opportunities for early detection and treatment through screening. Pediatricians and other primary care providers are the first line of defense against EHTN. They can play a significant role in early diagnosis, treatment, and patient education. The use of a team approach, especially one that incorporates increased data sharing using enhanced health information technology (such as electronic records and electronic data transmission) is an effective method for improving BP control. Keywords: cardiovascular disease, children and adolescents, essential hypertension, prevention, team approach

New treatments of acute renal failure in Hemiscorpius lepturus (Scorpion) stung

children Ehsan Valavi Ahwaz University of Medical Sciences Background: Scorpion sting is one of the health problems in many parts of the world including the tropical areas such as Iran. Hemiscorpius lepturus is one of the most dangerous scorpion species that causes local and systemic complications such as erythema, ecchymosis, cellulitis and necrosis at the sting site, fever, dizziness, anorexia, rhabdomyolysis, hemolysis, restlessness, confusion, seizures, cerebral infarction, disseminated intravascular coagulation (DIC), renal failure and cardiovascular complications, causing mortality and morbidities in victims, especially in younger children. Microangiopathic hemolytic anemia (MAHA) is another complication in these patients that consists of hemolytic anemia with negative Coombs test, chopped red blood cells, thrombocytopenia and increased lactate dehydrogenase. This may gradually lead to acute renal failure and hemolytic uremic syndrome (HUS), which are due to ADAMTS13 (A dysintegrin and Metaloprotease with thrombospodin- like motif type13) deficiency. ADAMTS13 cleaves the platelet-adhesive plasma protein von Willebrand factor. Diagnosis and Treatments: Complete blood count (CBC), prothrombin time (PT) and partial thromboplastin time (PTT), BUN, Creatinine and Urine analysis, should be monitors every 6 hours in the first day and then every 12h. Three to five vials of polyvalent scorpion anti-venom should be injected intravenously for all patients. In patients with hemolysis and hemoglobinuria, alkaline diuresis with 15-20 meq/L sodium bicarbonate in one and half times of the maintenance fluid demands from serum 5% dextrose water (DW5%) and loop diuretics (furosemide 2mg/kg/day) should be prescribed and if there is severe anemia, packed red blood cell (P.C) should be also given. If the PT or PTT is increased, or if there is evidence of DIC or HUS, fresh frozen plasma (FFP) with the rate of 20-30cc /kg/day should be administered in combination with diuretics or as a part of plasma exchange. This management is usually continued until the laboratory findings of venom cytotoxicity are resolved.

Conclusion: There are good results in treatment with plasma exchange and administration of plasma products including FFP which are rich of ADAMATS13, in severe cases of scorpion stung patients that developed DIC or HUS. Keywords: renal failure, scorpion sting, fresh frozen plasma

Interpretation of urinalysis

Farahnak Assadi Department of Pediatrics, Section of Nephrology, Rush University Medical Center, Chicago, Illinois, USA Urinalysis is the most commonly performed screening tests. Urinalysis screens for many different urinary tract disorders. However, clinicians respond to less than two-thirds of abnormal urinalysis. Periodic screening for abnormal urinalysis in healthy children has been recommended in three developmental stages including infancy, early childhood, late childhood, and adolescence. The normal range for urine specific gravity (SG) is from 1.001 to 1.040. Because of the wide physiologic range of urine concentration and dilution, measurements must be interpreted with reference to the state of hydration and blood osmolality. A urine SG<1010 in a dehydrated patient may be due to diabetes insipidus and urine SG>1010 in a well hydrated patient may be due to SIADH. The normal range for urine pH is from 4.5 to 8.0. Because of the wide physiologic range of urine pH, measurements must be interpreted with reference to blood acid-base balance. A urine pH >6.0 in a child with systemic acidosis may signify the diagnosis of RTA. Measurement of blood glucose level is useful in separating renal fanconi syndrome and tubulointrstitial disease from isolated renal glycosuria. In isolated renal glycosuria, the blood glucose level is normal. Isolated renal glycosuria usually requires no further work-up. The protein-dipstick is highly specific for albumin (95%). The sensitivity of protein dipstick is 85%, detecting albuminuria as low as 15 mg/dL. The heme-dipstick is highly sensitive for hemoglobin, myoglobin and red blood cell (95 %), detecting >2-5 red blood cells per high power field. False-negative tests may occur with dietary intake of vitamin C. Specificity for a positive test is about 84%. False-positives are due to oxidizing agents on poorly washed glassware. Red blood cell morphology is useful in separating glomerular hematuria from non-glomerular hematuria. Keywords: Interpretation, Screening, Urinalysis

A 3 –year old boy with hemolytic uremic

syndrome treated with rasburicase

Anoosh Azarfar, Mohammad Esmaeili, Aghilolah Keikhosravi Dr SHikh Hospital, Mashhad A 3-year-old boy presented with bloody diarrhea, abdominal pain, hemolytic anemia, thrombocytopenia, and acute renal failure. He had received antibiotic from 5 days ago. He was diagnosed with hemolytic-uremic syndrome (HUS). On the 1st day in hospital, serum creatinine was 5.6 mg/dl, urea: 246 mg/dl, hemoglobin: 8.5 g/dl, platelet count: 45,000/mm3, uric acid:15mg/dl. After treatment with rasburicase and supportive management the 5th day, urine output increased and gradually urea, uric Acid and

Proceeding of the 24th International Congress of Pediatrics – Oct 2012

Nephrology & Urology58

serum creatinine fell to 156 mg/dl, 5/6mg/dl, and 3.7 mg/ dL, respectively. On the 12th day, he discharged and received follow-up care as an outpatient. Keywords: Hemolytic uremic syndrome, Rasburicase

Relationship between hypertension with serum parathormone in end stage renal

diseases (ESRD) treated with hemodialysis Ahmad Shajari Yazd University of Medical Sciences A study was done on patients with ESRD patients treated with HD. Level of serum calcium, phosphorous, alkaline phosphatase, albumin and intact parathormone were measured. The number of patients were 15(males=8, females=7) and the mean age of studied patients was (12+4, 12-4). There was a significant positive correlation between stage of hypertension and calcium-phospherous and serum PTH product. Hypertension and secondrey hyperparathyroidism interact in the process of accelerated atherosclerosis in hemodialysis patients thus warranting appropriate measures to control hyperparathyroidism vigorously. Keywords: hemodialysis, hypertension, parathormone

Randomized controlled trial comparing oral

midazolam to no sedative for voiding dysfunction in children undergoing VCUG

A Azarfar, R Jalili, M Malaki,F Mortazavi, Behbehani, Y A Ravanshad Department of pediatric, School of Medicine, Mashhad University of Medical Sciences

Background: VCUG is the gold standard for detecting and grading of the vesicoureteral reflux. Since VCUG is an invasive procedure, it can cause distress in children who underwent this procedure, we can educate parents to control their child and we can use sedatives like Midazolam to prevent such distresses in children. The aim of this study was comparing oral midazolam to no sedative for voiding dysfunction in children undergoing VCUG. Methods: In a clinical trial we studied 84 children undergoing VCUG in Tabriz children hospital from 2011 till 2012. Children were randomly divided into two groups; In the intervention group, we used ampule midazolam 0.5 mg/ kg (oral) and then during the VCUG the possibility of voiding during VCUG and restlessness and stress were monitored again and disorders of urinary and voiding dysfunction were evaluated. In the non-intervention group all above steps were taken and then two groups were compared to the benefits and limitations of the use of midazolam in reducing urinary tract complications and stress of VCUG in children's actions. Findings: Minimum age was 5 years, maximum age was 13 years and mean age was 6.79±1.85 years. 24(28.6%) of patients was males and 60(71.4%) was females. 56(66.7%) had a history of urinary dysfunction. The patients' cooperation in the intervention group was mostly satisfying and good and there was a significant difference between two groups from this point of view(P=0.001). Distress and restfulness of patients in the intervention group was significantly lower than control group(P=0.001). Dysuria, distress and frequency were significantly lower in the

intervention group (p=0.001, p=0.001), while there was no significant difference between two groups in urinary incontinence, urinary refusal, insomnia attacks, and parents opinion in the child’s distress(P=0.633,P=0.575,P=0.625). Conclusion: Use of midazolam as 0.5 mg/ kg before the VCUG is effective in reducing children's distress and increasing their cooperation. Keywords: Midazolam, VCUG, children

Incidence and mortality of acute kidney injury according to RIFLE criteria in

critically ill children admitted to a pediatric intensive care unit

Kh Ebrahimkhani, N Hajizadeh, S Ebrahimkhani Children Medical Center, Tehran University of Medical Sciences

Background: To apply RIFLE criteria to characterize acute kidney injury (AKI) in critically ill children admitted to a pediatric intensive care unit (PICU). Methods: In a prospective descriptive study, data of eligible patients admitted to PICU over one year period were recorded. Patients with chronic kidney disease, receiving renal replacement therapy on admission and post-cardiac surgery were excluded. Findings: 296 patients aged 1 month to 17 years entered the study. Overall 47% developed AKI according to RIFLE criteria. RIFLE staging consisted of Risk 50.3%, Injury 33.8% and Failure 15.8%. Of these 65.5% occurred within the first 24 hours of admission (Admission AKI) whereas 34.5% developed AKI later (Delayed AKI). AKI was more common in patients with ventilation support. PICU mortality rate in patients with AKI was 26.6%, higher than non-AKI patients (p< 0.001). The mortality rate was not different among AKI severity stages (R= 35.13%, I= 40.54%, F= 24.32%). Mortality in Admission and Delayed AKI were 54% and 46%, respectively, p = 0.068. Risk factors identified to increase mortality in critically ill pediatrics with AKI were age (≤5 year); mechanical ventilation and length of PICU stay. Conclusion: This study is the first application of RIFLE-assessed AKI in Iranian pediatric patients admitted to a general PICU. AKI was common with higher mortality than non-AKI patients. RIFLE-assessed AKI was sensitive and informative. Further studies are needed to fully elucidate AKI by RIFLE in pediatric population. Keywords: acute kidney injury, RIFLE, pediatric intensive care unit, outcome

Roles for TASK2 potassium renal channels in

proximal renal tubular acidosis

Mohsen Akhavan Sepahi Qom University of Medical Sciences In proximal tubules, luminal and basolateral K+ channels have been found. Many inherited and acquired diseases have been identified that are caused by mutations or functional alterations in potassium channels, such as epilepsy, sensorineural deafness, cardiac arrhythmia, diabetes and renal hyper or hypokalemia. Renal potassium channels: Potassium channels have a tetrameric structure in which four identical protein subunits associate to form a fourfold symmetric (C4) complex arranged around a central ion conducting pore (i.e., a



homotetramer). There are over 80 mammalian genes that encode potassium channel subunits. K+ channels play critical roles in renal tubular transport functions directly by providing a secretory pathway for K+ or indirectly by controlling membrane voltage and K+ recycling across the plasma membrane. Potassium channels are expressed in virtually all cells of the human body and fulfill very different functions depending on their cellular environment, such as cell volume control, membrane potential stabilization and excitability, or regulation of hormone or ion secretion. Types of K+ Channels: Voltage-gated, Inward Rectifying, Ca2+ sensitive, ATP-sensitive, Na+ activated, Cell volume sensitive, Type A, Receptor-coupled TASK potassium channels: The two-pore-domain K(+) channels, or K(2P) channels, constitute a novel class of K(+) channel subunits. They have four transmembrane segments and are active as dimers. It is widely distributed, being particularly abundant in the pancreas and placenta, but it is also found in the brain, heart, lung and kidney. TASK is very sensitive to variations in extracellular pH in the physiological range. TASK2 potassium channels: TASK-2 is a member of the two-pore domain K+(K2P) channels family exist as dimmers, each subunit comprising four transmembrane domains (TMDs) and two pore-forming domains. Athours found strong labeling acid- and volume-sensitive TASK2 K channel that is expressed at high levels in various epithelial tissues, including the pancreas, placenta, lung, small intestine, colon, and especially the kidney but is not found in the nervous system, skeletal muscle, or heart. In human and mouse, TASK-2 is mainly expressed in the renal proximal tubules and papillary collecting ducts. No labeling was observed in glomeruli, distal tubule, cortical collecting duct, and medulla. By radiation hybrid mapping, the human TASK2 gene (KCNK5) was localized on chromosome 6p21. The importance of TASK-2 in NaHCO3 absorption in the proximal tubule has been established. Keywords: K+ Channels, TASK2 K channel, Proximal tubule

Comparative analysis of ultrasound and

DMSA in diagnosing acute pyelonephritis in hospitalized children at children' hospitals of Qom in the time interval between September

2004 to September 2009 Mohammad javad Qasemzade Islamic Azad University of Qom Background: Urinary infection is so prevalent in children and sometimes its complications are very dangerous and its diagnosis ways are varied and its clinical symptoms vary from non-symptomatic to highly-symptomatic. The goal of conducting his study was a comparative analysis of ultrasound and kidney scan with DMSA in diagnosing acute pyelonephritis in hospitalized children in children' hospitals of Qom in the time interval between Shahrivar 1383 to Shahrivar 1388. Methods: During a descriptive study 435 files from those belonging to patients hospitalized in these centers from Shahrivar 1383 until Shahrivar 1388 with the final diagnosis of pyelonephritis were examined and 50 files which had the appropriate criteria to enter our study were chosen and their clinical and laboratorial information was recorded in questionnaires and were analyzed after

gathering the data by the SPSS software in addition tables were used for rhe description of the data and the KAPPA coefficient was used for the comparison of the rate of agreement among the data. Findings: The ultrasound accuracy was 26%, and DMSA Renal Scan accuracy was 48%, and 33.3% of our patients had abnormal ultrasound and DMSA. Renal Scan and the relationship between ultrasound and DMSA Renal Scan was statistically meaningful(P=0.03). Conclusion: According to the high spread of acute pyelonephritis, various ways with different accuracies in its diagnosis and multiple studies have been conducted on its diagnosis inside and outside the country. DMSA Renal Scan is a very accurate diagnostic test for the diagnosis of acute pyelonephritis. Keywords: DMSA, sonography, acute pylonephritis

The urine PH in acid base and electrolyte

disorders

Hasan Otukesh Aliasghar Childrens Hospital, Tehran University of Medical Sciences The urine pH level indicates the amount of acid in urine. Abnormal pH levels may indicate a kidney or urinary tract disorder. The normal values range from 4.6 to 8.0. Urine pH can be affected by various factors after collection such as leaving the urine standing in an uncovered container. Bacteria usually increase the pH as they break down urea in the urine to ammonia. In normal status, urine PH is affected by diet, hydration and drugs. A high urine pH (alkaline urine) may indicate: Gastric suction, renal or kidney failure, renal or kidney tubular acidosis, urinary tract infection or vomiting without dehydration. A low urine pH (acidic urine) may indicate: chronic obstructive pulmonary disease (such as emphysema), diabetic ketoacidosis, diarrhea, starvation and severe dehydration. Keywords: urine PH, acid base disorders, electrolyte disorders

Immunization and kidney disease Azar Nickavar Aliasghar Childrens Hospital, Tehran University of Medical Sciences Prevention of infectious disease is an essential component of pediatric practice especially in children with kidney disease. Different compartments of immunity system including cellular and humoral immunity, complement system, phagocytosis and localized immunity might be involved in kidney disease. However, specific pharmacologic treatments have additional immunosuppresive effect. Therefore, protective effects of immunization might be deteriorated in children with kidney disease. Lower seroconversion rate, lower peak antibody titer to various immunizations and rapid decline of antibody level in nephrotic range proteinuria or peritoneal dialysis (loss of Igs into the dialysate fluid) contribute to reduced protection of immunization in children with kidney disease. Recommendations of immunization in children with kidney disease and renal transplantation include: 1-Consideration of all age appropriate and special vaccines in chronic kidney disease (ESRD). 2- Contraindication of live viral vaccines in immune compromised patients (high



steroid administration, renal transplantation, and chronic glomerulonephritis). 3- Improved immunization response by increasing dosage and reinforcement of vaccination schedules. 4- Monitoring of vaccination response by measuring specific antibody level every other year and repeated administration in suboptimal protection (low ab titer). 5-Passive immunization with IgG preparation based on serologic susceptibility and disease exposure. 6-Appropriate age for recommended immunizations before renal transplantation: 1-Live virus vaccine at least 1 month before transplantation, not recommended after transplantation. 2-Contraindication of live bacterial vaccines (BCG and Ty21a typhi) after transplantation. Keywords: immunization, kidney, transplantation

Il-19, Il-20,Il18AG137, IFNgamaAT,

TNF308GA genes polymorphisms and haplotype analysis in a vesicoureteral reflux

population

Simin Sadeghi bojd, Mohammad Kordi Children and adolescences health center, Ali Ebn-e Abitaleb Hospital, Zahedan University of Medical Sciences Vesicoureteral reflux (VUR) is a common childhood problem causing renal wounds and escalating the risk of renal deficiency and hypertension. A vast literature suggests that genetic variations play a significant role in pathogenesis of VUR. The aims of the present study was to estimate whether genetic polymorphisms of IL-19 (GC rs 2243158, AT rs2243158) and IL-20 (AG rs2981573, TG rs2981572), IL18AG137, IFNgamaAT genes are involved in development of VUR. Methods: Tetra amplification mutation refractory systees in 110 healthy controls and 124 VUR children. Findings: Significant association was found between the combined genotypes of IL19GC + CC and IL20TG+GG and increased risk of VUR [OR=1.90, 95% CL; 1.06-3.41 ; OR= 1.87, 95% CL, 1.06-3.29 ] respectively. Frequency of allele G in both sites of IL-20 (IL20AG rs2981573and IL20TG, rs2981572) showed a statistically significance difference (p = 0.01) between cases and controls in comparison with the wild type. The combined haplotype analysis of IL-19 and IL-20 polymorphic sites revealed that HT2, HT3 and HT5 haplotypes, marginally increased the risk of VUR but not statistically significant. Gene – gene interaction data of IL-19 (GC rs 2243158, AT rs2243158) and IL-20 (AG rs2981573, TG rs2981572) in various genotypes patterns highlighted that the most of the genotypes combination increased the risk of disease insignificantly. Frequency of allele G in both sites of IL18A137 showed a statistically significance difference(p= 0.023) between cases and controls. Frequency of allele A in both sites of INFgammaAT showed a statistically significance difference(p= 0.003) between cases and controls. Frequency of allele G in both sites of TNF308GA did not show a statistically significance difference(p= 0.306) between cases and control. Further studies with large samples size will be suggested to elicit exact effect of these cytokine genes in progression of VURm. Polymerase chain reaction (Tetra - ARMS PCR) was applied for analyzing of four polymorphic sites of IL-19 (GC rs 2243158, AT rs2243158), IL-20 (AG rs2981573, TG rs2981572) IL18AG137and IFNgamaAT gen.

Conclusion: This is the first evidence regarding IL-19 and IL-20 cytokine genes polymorphism and risk of VUR suggesting further study with large sample size and in different population for confirming the presented data. Keywords: Il-19, IL-20, polymorphism, VUR

Renal transplantation outcome in cystinosis

patients Rozita Hoseini Aliasghar childrens hospital, Tehran University of Medical Sciences Cystinosis is a rare inherited disease that leads to renal failure. Fanconi syndrome is the major renal involvement in cystinosis patients. Renal transplantation is the treatment of choice in cystinosis children with ESRD. This study is a follow up of 21 cystinosis children transplanted in Labafi nejad hospital. We compared the graft survival rate between cystinosis patients and children with other causes of end stage renal disease. Three cystinosis patients involved by primary non function because of graft vein thrombosis and/or severe acute tubular necrosis. All these three patients had low weight at the time of transplantation. The remaining cystinosis patients had excellent graft survival rate and only one patient of them lost her graft 3 years post-transplant due to noncompliance. The graft survival rate after excluding the patients with primary non function was 100%, 94%, 94% and 94% at 1, 3, 5 and 10 years after transplant respectively. This graft survival rate in cystinosis patients was significantly better than patients with other causes of ESRD especially in long term. The mean serum creatinine in patients with functioning graft 10 years after transplant was 1.6 mg/dl. We showed that cystinosis patients had much better graft function in long term after transplant in comparison with other causes of renal failure. Keywords: renal transplantation outcome, cystinosis

Assessment of urinary N-Acetyl-Beta-D-

Glucoseaminidase (NAG) in epileptic children before & after treatment with antiepileptic

drugs M Mazaheri, A Samaee Semnan University of Medical Sciences Antiepileptic drugs have various side effects on renal function. This study is planned to evaluate reliability of NAG index (urinary NAG/cr)for screening of renal injury in patients who recieved carbamazepin or valproat sodium. Methods: In this study 105 epileptic patients whose age were 3-16 yr old were age matched group: A: patients who did not take any teatment(tx); B: patients who took min.6 months tx, with carbamazepin (monotherapy); C: patients who recieved min. 6 month tx. with valproat sodium (monotherapy); 4th group is selected as healthy control group( D). Patients number was the same in all groups. Blood sample was drawn for biochemical tests, urine sample was sent for all patients for assessing NAG, creatinine and urinalysis. Findings: Biochemical tests like BUN, Cr and Eectrolytes in all children was normal. Sonographic finding in all was normal too. Mean urinary NAG index was 0.68 and 0.74 in control and group A respectively.There was not significant



difference between them. Mean NAG index in groupB was 1.71 (P<0.001), it was 2.3 times of group A. Also in group C it was 2.45, 3.3 times of group A (P<0.001). Comparing group B and Group C showed that NAG index in group C was 1.5 times of group B (P<0.001). Conclusion: Treatment with carbamazepin or valproate sodium can increase urinary NAG significantly in comparison with control group which did not take any drugs. NAG index in patients can be raised by taking valproate sodium more than carbamazepin. It means both drugs can cause renal tubular function deterioration but valproate sodium can cause more severe damage in kidney. Keywords: epilepsia, renal function, NAG

Dipstick urine analysis screening among

asymptomatic school children Abdolhamid Jafari, Zahra Hashemian, Mehrdad Shakiba Yazd University of Medical Sciences Background: A dipstick urinalysis screening was conducted to detect such prevalence and to set up a more effective screening program for children Worldwide. The basic dipstick method is the most rapid screening procedure that could be helpful in the early detection of renal or urinary tract diseases among apparently healthy or asymptomatic subjects in the hope of preventing and retarding progression to chronic renal failure. Methods: A cross-sectional study was conducted in two educational areas of Yazd, Iran randomly in 3014 primary schools children for 3 month follow up program mass. First morning mid steam urine samples were obtained from students and were tested by dipstick method. Findings: Ninety four (3.1%) children had urinary abnormalities at the first screening; Proteinuria was the most common abnormality found with a prevalence of 1.79% (54subjects). Among all the students, hematuria was found with a prevalence of .5%(16 subjects) followed by nitrituria (0.69%) and leukocyte esterase (0.29%). Conclusion: Asymptomatic urinary abnormalities might be detected by urine screening program at school age. In our study we found 16 new cases of renal disease (0.5%) and the most common form of urinary abnormalities was proteinuria. Keywords: dipstick urine analysis, urine analysis screening, school aged children

Evaluation of anemia and thrombocytopenia

in patients with acute and chronic renal failure

Akbar Dorgalaleh1, Shaban Alizadeh1, Esmaeil Sanei Moghaddam2 1Hematology Department, Allied Medical School, Tehran University of Medical Sciences. 2Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine and Zahedan Regional Educational Blood Transfusion Center Background: Acute renal failure describes as a syndrome by rapid decline in the ability of the kidney to eliminate waste products, regulate acid–base balance, and manage water homeostasis. When this impairment is prolonged and entered chronic phase, erythropoietin secretion by this organ was decreased and toxic metabolic accumulated and

causes hematological changes include decreasing of HCT, MCV and RBC and platelet counts. This study evaluated anemia and thrombocytopenia in patients with acute and chronic renal failure. Methods: This study conducted on 130 patients with renal impairment. Initially renal function tests were done in patients and after confirmation of impairment, patients were divided into two groups of acute (<3 months) and chronic (>3months) renal failure based on duration of disease. Then complete blood count performed for each patient and finally obtained data were analyzed by SPSS software. Findings: Comparison between 100 patients with acute and 30 patients with chronic renal failure revealed that severity of anemia (HCT, Hb and MCV) between these two groups was not statistically different (P>0.05) but thrombocytopenia in patients with chronic renal failure was statistically different from acute ones (P<0.001). Conclusion: It was recommended that platelet count be checked periodically in patients with chronic renal failure to prevent the risk of bleeding. Keywords: acute renal failure, chronic renal failure, anemia, thrombocytopenia

The Effect of Phototherapy on Urinary Calcium Excretion in term admitted to 17th

Shahrivar Educational Hospital Afshin Safaei-Asl, Marjaneh Zarkesh, Kaveh Hajikhani Gillan University of Medical Sciences Background: Phototherapy plays a significant role in the treatment and prevention of hyper bilirubinaemia in neonates. However, this treatment modality may itself results in the development of some complications. A less-known complication of the phototherapy is hypocalcemia. Some studies reported hypocalcemia accompanied by increase in urinary excretion of calcium. The aim of this study was to evaluate the effect of phototherapy on urinary excretion of calcium in term neonates. Methods: In a before-after study 80 term icteric newborns undergoing phototherapy were selected through accidental sampling. The consent was taken from parents on admission. Serum samples for calcium, bilirubin and sodium and urine samples for calcium, sodium and creatinine before and 48 hr after phototherapy were checked. Paired t-test and wilcoxon test were used for statistically analysis. Findings: In this study 80 term newborns (46 males and 34 females) with mean age of 7.01±4.13 days (3-26 days), gestational age 38.4±0.54 weeks (38-40 weeks), Birth Weight 3198±373.2 gr 2500-4420 gr), serum bilirubin 16.54±0.92 mg/dl (15-19 mg/dl) were included. The mean level of serum calcium before and after phototherapy were 9.37mg/ml, 9.25 mg/dl respectively (p>0.05). The difference between pre- and post-phototherapy urine calcium levels were found to be statistically significant (p<0.05). The mean fractional excretion of sodium and mean of QTC before and after phototherapy had no statistical difference (p>0.05). Conclusions: The result of this study showed that phototherapy may increase urinary calcium excretion in term neonates. Further investigation for clarifying the importance of this phenomenon is recommended. Keywords: hypocalcemia, Jaundice neonatal, phototherapy



Contrast-Induced Nephropathy (CIN) Nilofar Hajizadeh Children's Medical center, Tehran University of Medical Sciences Iodinated contrast material is a common adjunct to radiographic procedures. Far more common is intravenous use of contrast material for computed tomography (CT). However, the injection of intravascular iodinated contrast can result in a number of complications ranging from anaphylaxis to renal failure. A transient mild rise in serum creatinine following contrast administration is a common phenomenon. The vast majority of these patients rapidly recover normal renal function with no long-term problems. Some patients, however, experience a significant decline in renal function following contrast administration. Following onset of CIN, serum creatinine usually peaks in 3–5 days with a return to normal renal function within an average time of 7–21 days. However, long-term morbidity remains a concern; up to 30% of adult patients with CIN will develop some permanent renal impairment, including 1% who will require dialysis. CIN is said to be the third most common cause of hospital-acquired acute renal failure after surgery and hypotension. Preexisting renal dysfunction (creatinine levels in excess of 2 mg/dL) appears to be a primary risk factor. Dehydration, congestive heart failure, sepsis, diuretics, nephrotoxic drugs, and prolonged hypotension all contribute to a prerenal reduction of renal perfusion, which can markedly enhance the toxicity of contrast material. Preventative Strategies : 1- Identify and discontinue any nephrotoxic drugs at least 48–72 h prior to contrast administration. 2- Request that nonionic, iso- or low osmolar contrast material be used. 3- Begin IV hydration with 0.45%/0.9% NaCl or 150 meq sodium

bicarbonate in 1 L of 5% dextrose/water at 1 mL/kg/h for 6–12 h prior to the procedure and continue for 12– 24. 4- N-acetylcysteine twice daily administered the day before and the day of the procedure. Keywords: contrast-induced nephropathy, nephrotoxic drugs, hospital-acquired acute renal failure, Iodinated contrast material

The use of serum procalcitonin level in the prediction of high grade vesicoureteral reflux

in urinary tract infection

Nahid Rahimzadeh Aliasghar Childrens Hospital, Tehran University of Medical Sciences Procalcitonin is a reliable and specific marker of bacterial infection such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux. We investigated this association in children who admitted because of acute pyelonephritis. Findings: Forty eight children with the first febrile UTI were included. Twelve patients had low grade VUR, nine patients had high grade VUR (≥ 3) and twenty seven patients didn’t have any VUR in their imaging assessment. There was a significant association between high grade VUR and higher levels of procalcitonin (Pv=0.04). The sensitivity of procalcitoninlevel ≥ 0.31ng/ml was 90% and specificity was 32% for diagnosis of high grade VUR. Conclusion: We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high grade vesicoureteral reflux. Keywords: Procalcitonin, reflux, urinary tract infection

63

Practical evaluation of the child with microcephaly

Farhad Mahvelati Pediatric neurology Research Center Background: The assessment of growth in general and more particularly the measurement of the head circumference is an integral part of the pediatric neurological examination. Very important clues can be revealed from the head size and shape which will guide the differential diagnosis and the need for further investigations. The head grows rapidly antenatally and in the first three months. The rate of increase in head circumference is 3cm per month. The anterior fontanelle closes between 9-18 months. Between 4-6 years of age the head circumference increases by one cm per year. The head circumference is determined by measuring the greatest occipito-frontal circumference. There is a difference of opinion on the definition of microcephaly; simply speaking, the word means a head circumference smaller than normal. This has occurred for 1 of 3 reasons: either because the brain has not developed properly, or it has stopped growing or due to premature closure of sutures. Most authors define microcephaly as an occipitofrontal head circumference 2 or more standard deviations below the mean for age and sex, whereas others believe that the cutoff should be 3 SD. Microcephaly can be isolated, with no other obvious abnormalities, or it may be associated with other anomalies, which is termed syndromic. When present at birth, it has been termed “primary” microcephaly as opposed to “secondary” microcephaly, which develops later. Some authors believe that the term primary microcephaly should be used to imply genetic influences, whereas secondary microcephaly would designate factors that are environmental in nature. The best classification is simply according to its time of onset (eg, congenital or postnatal) and its presumed pathogenesis (genetic or environmental). The objective of this article is to make practical recommendations concerning the evaluation of the child with microcephaly. Recommendations are presented for neuroimaging, genetic testing, and screening for coexistent conditions Keywords: evaluation, head circumference, microcephaly

Prevalence of physical, verbal and

interactional aggression among preschool aged children

Alipasha Meysamie, Reza Ghalehtak, Arash Ghazanfari, Maryam Daneshvar-fard, Mohammad Reza Mohammadi Tehran University of Medical Sciences Background: Childhood aggression may lead to severe social disorders in adolescence and adulthood. Psychiatric treatment is focused on preschool aged children. Our scope was to estimate the prevalence and predisposing factors of childhood aggression.

Methods: Data was collected through a structured 46-item questionnaire investigating symptoms of physical, verbal and relational aggression completed by parents and teachers of day-care centers. A total of 1403 children attending 43 kindergartens were assessed. Analysis was performed through complex sample analysis and logistic regression method was used. Findings: According to the parent rating in each subset of aggression including physical, verbal and relational respectively 9.9% (95% CI=7.4-13), 6.3% (95% CI=5.3-7.5) and 1.6% (95% CI=1-2.8) of children received high scores while based on teachers’ rating the prevalence of 3 kinds of aggression, physical, verbal and relational were 10.9% (95% CI=8.9 -13.2), 4.9% (95% CI=3.8-6.4) and 6% (95% CI=4.4-8.3) respectively. A wide range of family environment factors including having single parent, having a working mother, death of someone close to the child, and having less educated mother were significantly associated with different types of aggression; additionally, there was some evidence of a relationship between age, sex and health status of the children and aggression, after controlling for other variables. Conclusion: This study revealed that the children’s family environment plays an important role as external factor in determining the child’s potentially aggressive behavior. Given this, intervention strategies should be planned at the families so as better prevention will be achieved through education the caregivers specially mothers. Keywords: Iranian children, Physical Aggression, Verbal Aggression, Relational Aggression, Preschool Children

Efficacy and safety of gabapentin for

prophylaxis of migraine headache in children Razieh Fallah Department of Pediatrics, Shahid Sadoughi University of Medical Sciences

Background: In frequent or disabling migraine headaches, prophylactic therapy is indicated. The purpose of this study was to compare efficacy and safety of gabapentin for migraine prophylaxis in children. Methods: In a quasi-experimental study, monthly frequency, severity and duration of headache and Pediatric Migraine Disability Assessment score(pedMIDAS) and side effects of 45 migraineurs children aged 5-15 years, referred to Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran, between September 2010 and November 2011 and were on gabapentin for three months, were evaluated. Findings: Twenty five girls and 20 boys with a mean age of 10.45 ± 1.68 years were evaluated. At the end of treatment, good response(>50% reduction in monthly headache frequency) was seen in 72% of patients. Mean monthly migraine frequency, severity and duration of headache decreased from 16.4 ± 6.7 to 6.21 ± 2.9 attacks (P =0.001), from 6.91 ± 1.1 to 3.4 ± 1(P =0.01), and from 3.1 ±1.4 to 1.2 ± 0.67 hours (P =0.002), respectively. Headache disability improved with a reduction of Pediatric Migraine Disability Assessment score from 34.2 ± 11.65 to 21.12 ± 7.4 (P=0.02). Transient side effects were seen in 18 % of the patients: drowsiness in 13%, and irritability and weight

Neurology & Psychiatry



gain (each one) in 2.5% of them. No life- threatening side effects were reported. Conclusion: Based on results of this study, gabapentin is effective and safe in prophylaxis of migraine headache in children. Keywords: Headache, Migraine, Prophylaxis, Gabapentin

Predicting developmental disorder in infants

using an artificial neural network

Farin Soleimani1, Akbar Biglarian2 1Pediatric Neurorehabilitation Research Center. 2Department of Biostatistics.1,2 University of Social Welfare and Rehabilitation Sciences Background: Early recognition of developmental disorders is an important goal and equally important is avoiding misdiagnosing a disorder in a healthy child without pathology. The aim of present study was to develop an artificial neural network using perinatal information to predict developmental disorders at infancy. Methods: A total of 1,232 mother–child dyads were recruited from 6,150 in the original data of Karaj, Alborz Province, Iran. Thousands of variables are examined in this data including basic characteristics, medical history, and variables related to infants. The validated Infant Neurological International Battery test was employed to assess the infant’s development. Findings: The concordance indexes showed that true prediction of developmental disorder in the artificial neural network model, compared to the logistic regression model, was 83.1% vs. 79.5% and the area under ROC curves, calculated from testing data were 0.79 and 0.68, respectively. In addition, specificity and sensitivity of the ANN model vs. LR model was calculated 93.2% vs. 92.7% and 39.1% vs. 21.7%. Conclusion: An artificial neural network performed significantly better than a logistic regression model. Keywords: Infant, Risk factor, Neural network, Developmental disability, Prognosis Latest on the side-effects of Buspirone used in

the treatment of Paediatric Psychiatric and Neurological related stress disorders

Mirza Shahid Arshad, Shamsi Allami Gujrat, Pakistan Background: We had proved that Buspirone tablets were superior to other anxiolytic drugs in the treatment of Paediatric Neurological and Psychiatric related stress disorders and presented our study at the 20th Annual Congress of Paediatric diseases in Tehran. However, after prescribing Buspirone for over two years we found certain new side effects of this drug not mentioned in literature. So, our objective was to determine the newer side effects of Buspirone used in the treatment of Paediatric Psychiatric and Neurological related stress anxiety disorders. Methods: A total of 100 patients, fifty females and fifty males aged between 11-15 years were treated with Buspirone tabs 5mg/b.d for their psychiatric or neurological related stress disorders(anxiety) who were reporting to, or admitted at Al-Shafa Hospital, Gujrat, Pakistan over a period of one year, during my administration of that hospital in the indoor and outdoor

departments and they and their parents were questioned for possible side effects while no other anxiolytic drug was administered to them. The patients were followed up either in the indoor or outdoor departments or on subsequent visits and by telephone. The medicines were provided by a reputed Iranian pharmaceutical company based in Tehran, Iran for this study. Findings: Buspirone had presented with side effects of eye pain in 33 of these patients. This is quite not present in literature. Conclusion: Buspirone may have side effects of eye pain and intensification of already existing eye pain while treating Paediatric Psychiatric and neurological related stress disorders(Anxiety). Thus it is suggested to carry out eye examinations and rule out eye disorders/ diseases prior to commencing Buspirone reatment in children. Keywords: Neurological, Stress, Psychiatric, Anxiety, Buspirone, Side-effects

Serum ferritin level in febrile children with

and without seizures A Talebian, S Andalib, SA Moravveji, Z Vakili Kashan University of Medical Sciences Background: Although febrile seizure is the most common cause of convulsion in childhood and strongly age-dependent, its incidence is rare before 9 months and after 5 years. Moreover, iron deficiency in these ages may have a detrimental effect on neurological and intellectual functioning. This study was designed to determine the serum ferritin level in febrile children with and without seizures. Methods: This case-control study was performed on 80 children(6 months to 5 years) referred to Kashan Shahid Beheshti hospital from January until June 2010. They were divided into two equal case and control groups(children with febrile seizures and febrile children without seizure, respectively). Children with the CNS disorder, developmental delay, renal failure, and shigellotic gastroenteritis were excluded. Ferritin, Hb, MCV and MCH values between the two groups were compared. Findings:The mean serum feritin levels were 97.6±90.6 ng/ml for cases and 109.2±106.2 ng/ml for controls (P=0.351). The mean corpuscular hemoglobin was 11.17±0.881 g/dl in the case group and 11.04±0.963 g/dl in the control one (P=0.534). Moreover, the mean MCV in the febrile seizure and fever groups were 73.71±4.16 fl and 71.86±4.42 fl, respectively (P=0.049). Also, the mean MCH in the febrile seizure and fever groups were 24.80±1.64 pg and 23.83±2.16 pg, respectively (P=0.033). Conclusion: No relationship between the serum ferritin level and febrile seizure is seen in both groups. Keywords: Febrile seizure, Ferritin, Iron deficiency anemia, Children

Effectiveness of cognitive-behavioral group therapy on girl children with social anxiety

Bahram Mirzaian, Zahedeh Rahmanian Payame Nour University

Background: Individuals with social phobia suffer from both cognitive distortions and social avoidance while it damages on function of many children and teenagers. So

65Proceeding of the 24th International congress of pediatric –oct 2012

Neurology Abstracts

we attended to survey the effectiveness of cognitive-behavioral group therapy for social anxiety of girl children. Methods: Method of this study was an Experimental type. After performing social anxiety inventory, structured clinical interview based on DSM-IV was conducted & 18 students(n = 9 for each group) were selected while it was assigned randomly in two experimental group(cognitive intervention-treatment group) and control group(no treatment). After a 12-session treatment [three months, each session being 2 hours], social anxiety inventory was repeated as a post-test in both groups and the first and second average of experimental group and control group was surveyed. Findings: The statistical analysis of t–test showed that there was a significant difference between social anxiety with 1% error in the experimental group and control group. Moreover, the clients could save the result of treatment until once month later. Conclusion: cognitive-behavioral group therapy was effective on social anxiety in fifth grade girl students. Keywords: cognitive-behavioral group therapy, social anxiety, children.

Effects of omega 3 and zinc supplementations in attention deficit/ hyperactivity disorder as

complementary therapies

H Sheykholeslami, F Dorreh, B Salehi, F Jafari Arak University of Medical Sciences

Background & objective: Attention-deficit/hyperactivity disorder (ADHD) is the most common neurobehavioral disorder of childhood. Multiple factors have been implicated in the etiology of ADHD. Multiple factors such as deficiency of some micronutrients have been correlated with ADHD. In this study, we have been evaluated the role of zinc and omega-3 supplements as adjunctive drugs for the treatment of this disorder. Methods: This study was a randomized double-blind and single-center clinical trial with placebo in which 150 children with age 6 to 15 years old that were known as new cases of ADHD were evaluated for 8 weeks. In addition to the drug of choice (methylphenidate) for the disorder, patients received placebo in the control group, zinc sulfate in second group and omega-3 in third. Clinical improvement was checking by Conners’ Parents Rating Scales and Conners’ teacher Rating Scales before and then 2 weeks, 4 weeks and 8 weeks after treatment. Result were analyzed with SPSS software. Findings: The average age of children participating in this study was (9.1±2.2), (111 boys vs 39 girls). In our study, mean scores of Conners’ teacher and Parents Rating Scales showed significantly improvement during treatment in zinc group compared to control (P=.014). But there was no significant difference after consumption of omega-3 compared to placebo in mean scores of Parents and Conners’ teacher in this study (P=.89). Conclusion: Zinc supplementation accompanied by the main treatment significantly improve Symptoms of ADHD, especially in inattention subtype. But omega-3 supplementation is not superior to placebo in treatment of this disorder. Keywords: zinc, omega-3, ADHD, attention deficit, hyperactivity disorder

Evaluation of prevalence of epilepsy according to EEG in children admitted for

seizure, Azad University Hospitals, 2006-2011 Marjan Mohammadnoori Department of pediatrics, Islamic Azad University, Tehran Medical Branch Background: Seizure is a complex neurologic disorder with disfunction of neurons and abnormal electrical discharges because of cerebral tumors, cerebrovascular diseases, poisons and fever. Children with relapsing seizure are at risk of behavior disorders. So on time diagnosis and treatment are important. The first test is Electroencephalogram (EEG) that have a good specificity. In this study, we evaluated the prevalence of epilepsy according to the EEG in children admitted for seizure in Azad University Hospitals from 2006 to 2011. Methods: In this cross-sectional study, 100 children admitted for seizure in Azad University Hospitals from 2006 to 2011 were enrolled. The prevalence of epilepsy according to the EEG was assessed among them. Findings and Conclusion: In this study 32% of children had epilepsy that was not significantly associated with age, sex, and family history of seizure among them(P>0.05). Totally, according to the obtained results in this study and comparison with other studies it may be concluded that the prevalence of epilepsy according to the EEG in children admitted is very low. Keywords: seizure, Electroencephalogram (EEG), epilepsy

Molecular diagnosis of Duchenne and Becker

muscular dystrophies Anna Isaian, Mahmoud Reza Ashrafi, MohammadTagi Hagi Ashtiani, Narjes Soleimani Molecular Genetic lab, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences Background: Duchenne and Becker muscular dystrophies (DMD/BMD) are X-linked neuromuscular disorders affecting 1 in 3500 live-born males caused by mutations in the dystrophin gene (DMD). This gene, consisting of 79 exons covering 2.5 Mb of genomic DNA, is characterized by a remarkable mutation diversity. About 65% of DMD/BMD cases show partial gene deletions (60%) or duplications (5%) of different sizes that are preferentially clustered in two major hotspots of mutation, spanning exons 3–7 and 44–55 Point mutations and deletion/insertions of few nucleotides account for the remaining cases (35%). Currently, > 200 different mutations have been identified. Most of these mutations were found to be unique to the patient. No “hot-spot” regions could be identified. Methods: We screened 35 DMD/BMD patients and genomic DNA was extracted from fresh blood samples using salting-out method. Rapid detection of deletions by Multiplex-PCR allows specific DNA regions are amplified in multiplex reaction usually use 20 pairs of primers deletion mutations detection within the Dystrophin gene(pm,3,4,6,8,12,13,17,19,43,44,45,47,48,49,50,51,52,60,42) for detection of about 85% of deletions. The deletions can be identified from the pattern of bands as visualized on ethidium-bromide stained agarose gels. Once the deletion has been detected, it becomes a marker for the family and



prenatal diagnosis can easily be provided. Recently, multiplex ligation-dependent probe amplification assay (MLPA)], multiplex amplifiable probe hybridization assay (MAPH) and real-time PCR have been suggested as alternative methods to the current diagnostic approaches. Findings: At present study we screened number of DMD/BMD patients for dystrophin deletion and our data indicated that the most of deletions located in exons 45-52 which are in agreement with those findings in European DMD/BMD patients. Conclusion: In conclusion, the combination of multiplex PCR of hot spots exons with quantitative real time PCR may be a cost effective method for detection of most large deletions and duplications in the dystrophin gene,before using expensive methods like MLPA. Keywords: DMD/BMD Duchenne and Becker muscular dystrophies MLPA multiplex ligation-dependent probe amplification assay

Communicative problems exacerbate postural instability among children with

autism spectrum disorder Parisa Ghanouni2, AmirHossein Memari1, Shahriar Gharibzadeh3, Jandark Eghlidi4 1School of Medicine, 2Sports Medicine Research Center, 3Biomedical Engineering, 4Rehabilitation sciences. 1,2Tehran University of Medical Sciences. 3Amirkabir Technical University. 4Shahid Behesti University of Medical Siences Background: Children with autism spectrum disorder (ASD) are diagnosed based on problems in social, communication, and language. However they are not purely intact in motor skills such as postural control. Keeping upright posture is the complex skill which depends on multiple variables. The aim of this study was to seek whether there was any association of severity of disorder in speech, sociability, cognitive and physical health in children with ASD with their postural control. Methods: Static posturography was performed in 21 boys with high-functioning ASD aged 9-14 and recruited from all autism special schools of Tehran. Children should have stood comfortably on a force plate while looking at the marker in front of them for two trials each lasted 20 seconds. Also, parents were asked to fill autism treatment evaluation checklist (ATEC) in order to determine child’s severity in social areas. Findings: Problems in sociability area significantly related with range ML (medio-lateral) (r =0.56, p =0.008), Root mean square (RMS) ML (r =0.51, p =0.01), total RMS (r =0.48, p=0.02), mean frequency (r = -.53, p=0.01). Speech and cognitive areas correlated with mean frequency (r = -0.46, p=0.03/ r = -0.52, p=0.01 respectively) and physical health problem was related with ML-RMS (r =0.47, p =0.031). Conclusion: Social and communicative problems affect the ability of maintaining postural control in children with ASD. Consequently, it is recommended to clinicians that planning therapeutic interventions should be with regards to both domains. In other words, alleviating social deficits might optimize the potentials of improving postural stability among individuals with ASD. Keywords: Severity of disorder, postural sway, autism spectrum disorder

Prevalence of parental consanguineously marriage in mental retard children

Sedighah Akhavan karbasi, Motaharah Golestan, Raziah Fallah Department of Pediatric, Shahid Sadoughi University of Medical Sciences Background: Two significant causes of mental retardation are chromosome–based and genetic disorders. Parental consanguinity as a cause of mental retardation is debatable. Since consanguineous marriages are common in Iran, the purpose of this study was to determine the prevalence of parental consanguineous marriage in children with mental retardation. Methods: In a cross-sectional study, 320 mental retard children whom referred to Genetic Center were evaluated from viewpoint of parental consanguineously in Yazd, Iran in 2010 via questionaire. Findings: Medical records of 142 girls (44.4%) and 178 boys (55.6%) were evaluated. Parental consanguineous marriage was seen in 63% of them and parents were first cousins in 51 % of subjects with consanguinity coefficient 1/8 with 12.5% common genes and 12% parents had degree of consanguinity four with consanguinity coefficient 1/16 with 6.25% common genes and 27% of parents were distant relative. But 32% of first cousin parents also were double cousins. Conclusion: Based on results of this study, the prevalence of parental consanguineous marriage is significantly high among children with mental retardation. Genetic consults should be done in parental consanguineous marriage and positive family history of mental retardation.

Keywords: mental retardation, children, consanguineous marriage

Evalution of magnesium levels in serum and cerebrospinal fliud of patients with febrile seizure hospitalized in Bahrami hospital in

Tehran in 2010-2011

N Khosroshahi, F Shahab, M Shariati Tehran University of Medical Sciences

In the past,decreased levels of magnesium in serum and csf of patients with FC were reported.The purpose of this study was to identify the possible role of magnesium in febrile seizures in children. Identifing this condition, we may control seizures and also prevent subsequent convultion. Methods: In this cross sectional study, inclusion criteria were existence of convultion due to fever and exclution criteria were having a known neurological disease which could induce seizure and children younger than 1 month. In each group (cases included children with febrile convulsion and controls included febrile children without convultion), Mg was measured in blood and cerebrospinal fliud of 81 children and then they were compared.The data were analyzed by SPSS(α =0.05). Findings: The mean serum and CSF levels of Mg in case and control groups were equal (p<0.87 and p<0.22 respectively). There was no difference between two groups in terms of sex, but mean age was significantly different(p<0.003). Conclution: There was not a relationship between serum and CSF levels of magnesium and the presence of febrile


Neurology Abstracts

convultion.Therefore, it is not suggested to measure level of magnesium in serum of CSF in children with fever routinely. Keywords: febrile convultion, serum magnesium, cerebrospinal magnesium, childern

Prevalence and risk factors of sleep bruxism

in pre-school aged Iranian children

Shabnam Jalilolghadr, Ameneh Barikani, Atena Soltanzadi, Atousa Akhgar

Qazvin University of Medical Sciences Background: Sleep bruxism is a prevalent oral habit among children and potentially destructive for oral tissue. It may also be related to a number of disorders such as sleep complaints and headache. The aim of this study was to assess prevalence and risk factors of bruxism and to investigate the relationship between intestinal parasitic infections and bruxism among pre-school aged children in Iran. Methods: A cross-sectional study was conducted on 400 kindergarten children in Iran using cluster random sampling method. Two standard questionnaires including: Preschool Behavior Questionnaire (PBQ) and Iranian version of Children's Sleep Habits Questionnaire (CSHQ) were administrated among parents of subjects. All subjects were undergone an dental examination. Parasitological diagnosis test was performed using direct stool exam. Categorical variables were compared by chi-square test. P-values less than 0.05 were considered statistically significant. Findings: 214 subjects were males. Bruxism was reported in 76 (19%) of participants. Subjects with positive family history of stress, sleep disturbance and nocturnal symptoms (restless, sweating, and snoring), nail biting, dental disorders, ADHD and parasite infection were at higher risk of reporting teeth grinding. 19.2% of all subjects had family history of bruxism (P=0.002). Dental decay was significantly different between subjects with and without bruxism (p<0.001). Parasitic infection was observed in 13 children (7 with bruxism and 6 without bruxism). A statistically significant association was observed between intestinal parasitic infections and bruxism (P=0.02). 43.2% of all subjects had history of using stimulants (P=0.001). Conclusion: Bruxism is common in children. Intestinal parasites may contribute to initiation of bruxism. Increasing the awareness of dentists and physicians about this pathology is necessary. Keywords: sleep bruxism, children, risk factors, parasitic infection

A survey on Guillain-Barre syndrome among under 15 year old children in Iran 2007-2011

Sussan Mahmoudi, Pariva Tayefeh Hashemi,

Mohammad mehdi Gooya, Seyed Mohsen Zahraee Center for Communicable Diseases Control, Ministry of

Health and Medical Education

Background: Guillain-Barre syndrome is a postinfectious polyneuropathy involving mainly motor nerves. The paralysis usually follows a nonspecific viral infection by about 10 days. Weakness usually begins in the lower extremities and progressively involves the trunk, the upper

limbs, and finally the bulbar muscles. Proximal and distal muscles are involved relatively symmetrically, but asymmetry is found in 9% of patients. The onset is gradual and progresses over days or weeks. Bulbar involvement occurs in about half of cases. Respiratory insufficiency and death can result. Methods: This study was descriptive and cross-sectional. Data gathering was through AFP (Acute Flaccid paralysis) surveillance system which is the surveillance strategy for poliomyelitis eradication.The World Health Organization recommends that countries conduct surveillance for AFP cases. The AFP case definition is: “all cases of acute flaccid paralysis, including Guillain-Barre Syndrome, among children aged less than 15 years and all cases of suspected poliomyelitis among persons of any age.”All the hospitals, private clinics, physiotherapy and rehabilitation units and public health care centers all over the country were included in the sampling frame. Data compiled in EPI-6 and descriptive statistics, were used for analysis. Findings: In this study, 1567 cases of 2896 AFP cases (%54) were classified as Guillain-Barre syndrome. 848 cases (%54) were males and 719 cases (%46) were females. 60 cases(%.04) were under 1 year old, 498 (%32) were 1-2 years old, 327(%21) were 3-4 years old and 682 (%43) were 5 years old or more. 505 cases (%32) were febrile at the onset of paralysis. The number of the occurene of paralysis was 348(%22) in winter, 472(%30) in spring, 343(%22) in summer and 404(%26) in autumn. Paralysis was asymmetric in 307(%20) of cases and symmetric in 1260(%80) of cases.The progression of paralysis was rapid (less than 4 days) in 1248 (%80) of cases. Follow- up after 60 days revealed that 993(%63) of cases were completely recovered, paralysis remained in 516(%33) of cases, 32 cases (%.02) were lost during follow-up and 26 (%.02) of them died. Conclusion: This study reveals that Guillain-Barre syndrome is the leading cause of Acute Flaccid paralysis among under 15 year old children. So all cases of Guillain-Barre syndrome should be notified immediately to the nearest health center. Keywords: Guillain-Barre syndrome, Acute Flaccid paralysis, paralysis

Acute flaccid paralysis (AFP) among under 15 year old children in Iran 2007-2011, the

most common causes

Pariva Tayefeh Hashemi, Sussan Mahmoudi, Mohammad mehdi Gooya, Seyed Mohsen Zahraee

Center for Communicable Diseases Control, Ministry of Health and Medical Education

Background: Poliomyelitis is an acute viral infection which ranges in severity from a nonspecific illness to paralysis with permanent disability. In 1998 the World Health Assembly adopted a resolution to eradicate polio. To achieve poliomyelitis eradication, the World Health Organization recommends that countries conduct surveillance for cases of acute flaccid paralysis (AFP). The AFP case definition is:“all cases of acute flaccid paralysis, including Guillain-Barré Syndrome, among children aged less than 15 years and all cases of suspected poliomyelitis among persons of any age.” This syndromic reporting strategy is not only a key strategy for global poliomyelitis eradication, but also gives a precise figure of the most common causes of acute flaccid paralysis. The principal



AFP surveillance components are: a) reporting: it should be on time, including zero reports. b) Sensitivity of surveillance: at least 2 cases of non-poliomyelitis AFP should be detected annually per 100 000 population aged less than 15 years. c) case investigation: all AFP cases should have a full clinical and virological investigation with having adequate stool specimens, ie: two stool specimens, collected at least 24 hours apart, within 14 days after the onset of paralysis, all virological studies on AFP cases must be performed in a laboratory accredited by the Global Poliomyelitis Laboratory Network. d) Follow-up: AFP cases should have a follow-up examination for residual paralysis at 60 days after the onset of paralysis. Methods: This study was descriptive and cross-sectional. All the hospitals, private clinics, physiotherapy and rehabilitation units and public health care centers all over the country were included in the sampling frame. Data compiled in EPI-6 and descriptive statistics were used for analysis. Findings: During 2007-2011, total number of 2896 cases has been reported through AFP surveillance system (562 in 2007, 561 in 2008, 548 in 2009, 622 in 2010 and 603 in 2011). Number and percent of Guillain-Barre syndrome were 297(%52/8) in 2007, 333 (%59/4) in 2008, 309 (%56/4) in 2009, 321(%51/6) in 2010 and 307(%51/8) in 2011. The most common causes of AFP other than Guillain-Barre syndrome were arthritis(%2) and myositis- polymyositis(%2) in 2007, arthrithis, myositis-poly myositis, ADEM and ataxia (%2 for each one) in 2008, arthritis(%5) and ataxia(%3) in 2009, arthritis(%5), viral infection, synovitis-tenosynovitis and viral infection (%4/5 for each one) in 2010 and arthritis(%7/5) and myositis- polymyositis(%6) in 2011. Guillain-Barre syndrome was the most common cause of AFP during 2007-2011(%54) Conclusion: This study reveals that Guillain-Barre syndrome and arthritis are the leading causes of Acute Flaccid paralysis among under 15 year old children. All cases of Acute Flaccid paralysis including Guillain-Barre syndrome should be notified immediately to the nearest health center. Keywords: Acute Flaccid paralysis, polio eradication, AFP surveillance system

Recurrent Guillain –barre syndrome in 8

years old girl. A case report

M Faraji Goudarzi, N Taee, F Tarhani Lorestan University of Medical Sciences

Background: Guillain–barre syndrome is a peripheral neuropathy that occurs after viral infections with progressive ascending paralysis and muscle weakness. Sometimes paralysis associated with severe form and respiratory distress occurs. Diagnosis of this syndrome is clinical but for rule out other causes of acute paralysis, paraclinic maybe useful. In some reports recurrent disease was milder than acute disease and related to short course of IVIG therapy and occurred from 2 months to several years after primary illness. Case presentation: An 8 year old girl had acute flaccid paralysis 3 years ago and treated with IVIG for 3 days (2 gr /kg) with complete cure. She was well until 8 year old; then acute flaccid paralysis developed with milder clinical findings, paralysis and gait disturbance and hyporeflexia. She treated with IVIG for 5 days. All symptoms resolved after 7 days.

Conclusion: Recurrent Guillain–barre syndrome maybe occurs after the first attack. It is milder than acute disease and recurrences maybe related to short course of IVIG therapy. Keywords: Guillain–barre syndrome, recurrent, acute flaccid paralysis

Physical quality of life in children with

autism

Nekoo Panahi, Amir Hossein Memari Tehran University of Medical Sciences Background: Children with autism spectrum disorders (ASD) fail to develop appropriate peer relationships and are less likely to participate in organized activities. While recent studies have indicated poorer quality of life in parents and their children with ASD, researches demonstrated a positive relationship between leisure participation and quality of life (QOL) in children. Therefore we aimed to estimate a physical aspect of QOL in children with ASD by assessing their leisure habits and sport participation. Methods: The study was undertaken on a sample of 92 children with ASD aged 6.33 to 15.16 (9.8±1.8) years. We designed a checklist to survey daily activities and leisure activity profiles including participation in playing alone, playing with peers, watching TV and playing computer games. Parental demographic variables were collected as well as developmental characteristics of the children. Findings: The results of the study showed that 5.4% of subjects were engaged in regular, 8.7% in irregular and 85.9% in no leisure sport activities. The time spent on playing alone, playing with peers, and sedentary pursuit behaviors were 94(±81.1), 11.8(±28.2), and 92.4(±112.1) minutes respectively. Boys were more involved in sedentary pursuit behaviors than girls (p=0.002). Analysis of variance showed that children were involved in playing alone significantly more than playing with peers (p<0.001). Girls spent significantly more time on playing alone compared to boys (p<0.001). It was shown that the time spent on leisure sport activities is significantly associated with poverty level (p=0.01) and marital status of parents (p=0.027). Conclusions: Few children with ASD have been involved in regular leisure sport activities. They spent significant time on playing alone and engaged in few minutes of playing with peers. It was likely that the child sex, parental poverty level and marital status were associated with sport activity of the children with ASD. Keywords: autism, children, quality of life Efficacy and safety of cinnarizine compared

with propranolol in the prophylaxis of childhood migraine headache

Mahmoudreza Ashrafi1, Mansoureh Togha2 1Children’s Medical Center. 2Sina Hospital. 1,2Tehran University of Medical Sciences1 Background: Early diagnosis and appropriate interventions are mandatory to prevent migraine headaches and to improve quality of life for children. There are limited clinical studies about prophylactic drugs for childhood migraine headache. Our study compares the


Neurology Abstracts

efficacy and safety of cinnarizine with propranolol in the prophylaxis of paediatric migraine headache. Design: Randomized double-blind clinical trial. Methods: One hundred and twenty patients aged between 6 and 17 year old were selected according to the International Headache Society criteria for migraine to participate in a double-blind clinical study for 12 weeks. They were randomly divided into 2 groups. Sixty patients in group A received cinnarizine 50 mg/day for 12 to 17 year olds and 37.5 mg/day for 6 to 12 year olds, while group B received propranolol, 1 mg/kg/day divided into 2 doses. Findings: Cinnarizine reduced the baseline headache frequency more than 50% in 74.6% of patients. The mean headache frequency per month was reduced from 6.6 ± 1.8 to 2.7 (SD 1.1) attacks per month (p < 0.001). In the propranolol group the baseline headache frequency was reduced more than 50% in 72.5% of patients. The mean headache frequency per month was reduced from 7.1 (SD 1.5) to 2.9 (SD 1.2) attacks per month (p< 0.001). There was no significant difference between the two groups (p= 0.35). No serious side-effects were observed during the study. Conclusion: This study suggests cinnarizine is an effective and safe drug in the prophylaxis of paediatric migraine. Keywords: migraine, headache, propranolol, cinnarizine

Epilepsy surgery guided by

Magnetoencephalography (SickKids Experience)

M Mohammadi Children’s Medical Center, Tehran University of Medical Sciences Magnetencephalography (MEG) is a technique which studies magnetic fields produced by cortical neurons during normal and abnormal activities. It is a useful method for lateralization as well as localization of epileptic source in children. Recent studies have indicated its efficacy in determining epileptogenic zone in children suffering from intractable localization epilepsy. Although other methods such as scalp and intracranial video-EEG monitorings as well as neuroimaging techiques such as 3T MRI, PET and SPECT are additional methods to study epileptogenic zone in children with intractable localization related epilepsy, but MEG is more practical and conventional in many aspects. In my short introductory lecture regarding application of MEG in pediatric epileptology, I will share my experience during my sabbatical leave at hospital for sick children in Toronto. Keywords: intractable epilepsy, magnetoencephalography, localization related epilepsy, epilepsy surgery

Relationship between EEG abnormalities and autistic behavior in Iranian children with

Autism Parvaneh Karimzadeh Shahid Beheshti University of Medical Siences Autism or autistic spectrum disorder (ASD) is a Neuro developmental disorder that was first described by Leo

Kanner in 1943. The major manifestations of autism are verbal and non verbal communication deficits, restriction in social interaction and stereotyped repetitive behaviors. High frequency of abnormal epileptic form discharges in EEG is reported. Prevalence range of EEG abnormalities is 10.3- 72.4 % and epilepsy 0 – 44.5 %. Autistic regression disorder is a subgroup of Autistic patients (20-49 %) who Experience regression in their neuro developmental behaviors. Previous studies reported the role of abnormal EEG (with or without seizure) in autistic regression. This study was done to confirm the relationship between EEG abnormalities and autistic regression. Methods: Children with clinical diagnosis of autism in pediatric neurology department of Mofid children hospital during the years 2010-2012 referred for sleep Electroencephalography. The diagnosis of autism was made in children who met the criteria of DSM-IV-TR. Findings: 34 children who met the criteria for autism were referred during 2 years: 6 males (18 %) and 28 female (82 %). The children were between 3 and 13 years old. The EEG was abnormal in 62%. Among the abnormal EEG 12% showed generalized spike activity, 4 % showed focal abnormal epileptiform discharges and 46% showed slowing and abnormal background activity, the remaining had normal EEG. Epilepsy was diagnosed in 28 % of these patients. The major type of seizure was tonic seizure and staring spells. Conclusion: Our study showed the prevalence of epilepsy in autism was significantly higher than general population. Girls showed higher incidence of abnormal EEG and epilepsy than boys. Regression of neurodevelopmental behavior was found in association of abnormal EEG in children. Children without seizure showed higher neurodevelopmental score than children with autism and seizure. We suggest performing EEG for more evaluation of children with autism is needed. Keywords: autism, EEG abnormailties, regression

Pediatric Autoimmune Neuropsychiatric Diseases Associated with Streptococcal

Infection Vahid Aminzade, Babak Khati Gillan University of Medical Sciences The term Pediatric Autoimmune Neuropsychiatric Diseases Associated with Streptococcal Infection has been used to designate a group of neuropsychiatric disorders(PANDAS), notably tic disorders, Tourette syndrome, and OCD believed to be related to an antecedent streptococcal infection and for which an autoimmune pathogenesis has been postulated especially in a susceptible host who produces antibodies to GAS that cross-react with the cellular components of the basal ganglia particularly in the caudate nucleus and putamen. The clinical characteristics for the diagnosis of PANDAS are (a) presence of OCD, a tic disorder, or both; (b) onset between 3 years of age and puberty; (c) episodic course with abrupt onset or dramatic exacerbation of symptoms; (d) symptom exacerbations temporally related to group AB-hemolytic streptococcal infections, as demonstrated by a positive throat culture result, elevated antistreptococcal antibody titers, or both; and (e) association with neurologic abnormalities, notably choreiform movements during periods of symptom exacerbation. The diagnosis is suggested by a positive throat culture result or by positive antistreptococcal



antibodies. Treatment contain use of antibiotics directed against the putative inciting streptococcal organisms coupled with the management of the various neuropsychiatric symptoms by means of serotonin-reuptake blockers and behavioral therapies. A randomized, placebo controlled trial of IVIG (l g/kg for 2 consecutive days) and plasmapheresis (five to six procedures performed on alternate days) resulted in significant and persistent improvement. When children are given IVIG, improvement is seen 3 weeks after treatment or even later and persists for 1 or more years. With plasma exchange, symptom improvement is noted toward the end of the first week of treatment and persists for 1 year or longer. Tic symptoms are more effectively treated with plasma exchange, whereas IVIG and plasma exchange appear almost equally effective for symptoms of OCD. Tonsillectomy may also represent an effective treatment option in children severely affected by PANDAS. Keywords: PANDAS, OCD, streptococcal infection, Tourette syndrome

A 7 year old girl with Sturge-Weber Syndrome and Oromaxillofacial

Osteodystrophy

Shokoufeh Ahmadipour, Simin Gheini, Homa Babaei Lorestann University of Medical Sciences

Background: Sturge-Weber Syndrome is a rare sporadic vascular disorder with frequency of approximately 1 per 50,000. SWS consists of a constellation of symptoms and signs including a facial nevus(port- wine stain), seizure and hemiparesis. Mental retardation or severe learning disabilities are present in at least 50% in later childhood. One of the uncommon radiologic signs in SWS is Oromaxillofacial Osteodystrophia that is reported 5 cases until now. A 7 year old girl with mental retardation and a large facial nevus at birth time and recurrent severe seizures, droolling in 1st year of life without any treatment,was admitted because of severity of seizures and weakness. In following she had Oromaxillofacial Osteodystrophia. Keywords: Sturge-Weber Syndrome, seizure, mental retardation

Comparison of the plasma Zinc level between

children with febrile convulsion and non-convulsing febrile children

Farhad Abolnezhadian Shiraz University of Medical Siences Febrile convulsion is the most common seizure disorder in childhood which has a good prognosis. There are different hypothesis about neurotransmitters and trace elements (such as Zinc) changes in CSF and serum which can play a role in pathogenesis of febrile convulsion. This study was designed for evaluation of the plasma Zinc level of children with febrile convulsion in comparison with febrile children without convulsion and children without fever and convulsion. Methods: Two hundred and twenty eight numbers of children were enrolled into the study, among them 83 were without fever and convulsion, 65 were febrile and 80 had febrile convulsion. Plasma Zinc level in all three groups were measured by atomic absorption method. Data

analyzed by SPSS V.18 and serum zinc level compared between groups by ANOVA. Findings: Mean plasma Zinc level in children without fever and convulsion, febrile and febrile convulsion children was 87.68 ± 13.71 µg/dl, 91.19 ± 13.60 µg/dl and 64.27 ± 12.32 µg/dl respectively (P Value < 0.001), which means that febrile convulsion children had statistically lower zinc level than others. Furthermore plasma zinc levels in febrile convulsion children were not significantly different in two genders and also in various age groups. Conclusion: These findings suggest that plasma zinc level declines during febrile convulsion. On the other hand its level is not changed in febrile children without convulsion and non-febrile children. So lower zinc level in these patients wasn't related to febrile situation. Keywords: febrile convulsion, plasma Zinc level, fever Evaluation of personal - social developmental

skills levels in children 1-4 years old with early treated Phenylketonuria

Firoozeh Sajedi Pediatric NeuroRehabilitation Research Center (PNRC) Backgrounds: The present study investigated the level of personal-social developmental skills in children 1-4 years old was born with early treated phenylketonuria. Methods: This cross-sectional study has been done (case - control) based on 40 children referred to clinics. List of phenylketonuria (PKU) patients who were diagnosed by screening at birth and treated and are now 1-4 years old were recalled to the clinic. Information through a demographic questionnaire, ASQ (Ages & Stages Questionnaire) and clinical assessments were collected. 40 children as control group were selected randomly from kindergartens of Tehran and completed the questionnaires. Data were analyzed by using descriptive characteristics and inferential tests after the entry into the software package SPSS. Findings: In this study participated 40 children in the control group and 40 children in the case group. Children mean age was 7/30 (± 5/10) months in the control group and 7/27 (± 0/11) months in the case group. Between the two groups regarding of delivery and the current height and weight and head circumference at birth, there was no significant relationship. Between the two groups regarding personal-social developmental skills, there was a significant difference(P <0.001). Conclusion: The results showed that the level of personal - social developmental skills in the children with early treated Phenylketonuria is lower than normal children. Early diagnosis and intervention for all children with early treated Phenylketonuria recommended in low birth and age. Keywords: Phenylketonuria, early treated, personal, social developmental

The effect of behavioral parent training in

reducing externalized behaviors of children with Attention Deficit Hyperactivity Disorder Guita Movallali,Mohsen Amiri Pediatric Neurorehabilitation Research Center, University of Social Welfare& Rehabilitation Sciences Background: The purpose the current study was to investigate the effectiveness of the effect of behavioral


Neurology Abstracts

parent training to mothers of children with Attention Deficit Disorder (ADHD) in reducing externalizing behavior (aggression & Rule breaking) of this Children. Methods: 30 mothers of children with ADHD were selected using at-hand samplings who were divided into control and experimental groups (15 mothers in each). Both groups filled in children behavioral performance questionnaire and CBCL questionnaire. In the next step the experimental groups were instructed a nine sessions length behavior modification method program while the control group went on their normal procedures. At the end of the instructional sessions both groups filled out the mentioned questioners as the post test. Findings: Result of the t test showed that behavioral parent training decreased the aggression (P<0/05) and Rule breaking (P<0/05) of the children of the experimental group in comparison with the control group. Conclusion: Findings show that implementing the behavioral parent training to mothers decline behavioral problems in their children. Keywords: behavioral parent training, ADHD, externalizing behavior, CBCL First unprovoked seizure treat or not to treat

approach to the management Mohammad Ghofrani Pediatric Neurology Research Center, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences The main reason for prescribing antiepileptic drugs (AED) is to prevent further seizure. Thus such therapy is recommended when there is a reasonable chance seizure will recur. Decision whether to initiate therapy requires weighing the risk of treatment against the medical and psychosocial risks of further seizures. Risk Factors for recurrence studies of persons with a single unprovoked seizure have shown recurrence rates ranging from 16% to 61% depending on duration of follow-up and method of case ascertainment. A reasonable estimate of the general recurrence rate is 40%. Despite conflicting reports in the literature, some conclusion can be drawn about the risk factors for recurrence. These risk factors include: 1- Causes, 2- Seizure type, 3- EEG findings, 4- Family history of seizure, 5- A history of febrile seizure, 6- TODD paralysis (a transient post-ictal focal deficit), 7- Abnormal neurologic finding. When some or all of these factors are combined, the chance of recurrence is extremely high. Camfield et al found that children with partial seizure, an abnormal EEG and abnormal neurologic finding had a 90% chance of recurrence. Whereas patients with generalized seizure, a normal EEG and normal neurologic finding had only a 30% chance of recurrence. When to Treat: Patients at higher risk of recurrence might be considered for treatment after a single unprovoked seizure: 1- Patients with single seizure and EEG epileptiform abnormalities. 2- Patients with single seizure and associated medical or neurologic condition (CP, MR, etc) that increases the risk of further seizures. 3- Patients with single seizure which is associated with a known cause (tumor, stroke, tuberus sclerosis). When Not to Treat: 1- Patients with single seizure and normal EEG, 2- Patients with single seizure and unknown cause, 3- Simple partial seizure, 4- Seizure during sleep, 5- Blood, liver or kidney diseases, 6-Drug related seizures. Keywords: First unprovoked seizure, treat, not treat

Discriminant of validity the Wender Utah rating scale in Iranian adults

Farideh Farokhzadi, Mohammad Reza Mohammadi, Maryam Salmanian Psychiatry and Psychology Research Center, Tehran University of Medical sciences The aim of this study was the normalization of the Wender Utah rating scale which is used to detect adults with Attention-Deficit and Hyperactivity Disorder (ADHD). Methods: Available sampling method was used to choose 400 parents of children (200 parents of children with ADHD as compared to 200 parents of normal children). Wender Utah rating scale, which has been designed to diagnose ADHD in adults, is filled out by each of the parents to most accurately diagnose of ADHD in parents. Findings: The value of KMO (Kaiser-Meyer-Olkin) was 86.5% for depression, 86.9% for oppositional defiant disorder, 77.5% for school related problems, 90.9% for conduct disorder, 79.6% for anxiety and 93.5% for Attention deficit/hyperactivity disorder, also the chi square value based on Bartlett’s Test was 2242.947 for depression, 2239.112 for oppositional defiant disorder, 1221.917 for school work problems, 5031.511 for conduct, 1421.1 for anxiety, and 7644.122 for ADHD. Since mentioned values were larger than the chi square critical values table (P<0.05), it found that the factor correlation matrix is appropriate for factor analysis. Conclusion: Based on the findings, we can conclude that Wender Utah rating scale can be appropriately used for predicting depression, oppositional defiant disorder, school work problems, conduct disorder, anxiety, in adults with ADHD. Keywords: Attention-Deficit and Hyperactivity Disorder, Wender Utah Rating Scale, Validity

Comparing fficacy of botulinum toxin type-A injection in hamstring and calf muscles with

and without foot serial casting on improvement of gait in children with cerebral

palsy Susan Amirsalari1, Hamid Dalvand2, 3Leila Dehghan 1Baqiyatallah University of Medical Sciences. 2Pediatric Rehabilitation Research Center. 2,3Department of occupational therapy, University of Social Welfare & Rehabilitation Sciences The goal of this study was to compare the efficacy of botulinum toxin Type-A (BTX-A) injection in hamstring and calf muscles with and without ankle serial casting on improvement of gait in children with cerebral palsy(CP) Methods: This study was a prospective and double blind clinical trial performed on 25 children with hemiplegic and diplegic CP (2-8 years) in Tehran, who were referred to Valiasr rehabilitation foundation. Participants were chosen by simple randomized sampling and matched based on age, GMFCS and type of disorder and randomly divided into two groups; children in the first group (13 children) were injected BTX-A, the second group (12 children) received BTX-A injection and foot serial casting was started one week after the injection. Clinical assessment was



performed by the Gross Motor Function Measure(GMFM), Modified Ashworth Scale and range of motion (ROM), which were performed before injection and 1, 3, 6 and 12 months after injection. The results were analysed by statistical tests such as Wilcoxon signed and mann-whitney U. Findings: The difference between gross motor function, right and left knee spasticity and right and left knee passive ROM in the two groups before and 1, 3, 6 and 12 months after injection were not statistically significant (p>0.1). The difference between right and left ankle spasticity and passive ROM before and after 1 and 3 months follow- ups wasn't significant (p>0.1), however, significant difference were found at 6 and 12 month follow-ups(p<0.05) Conclusion: we concluded that injecting BTX-A with foot serial casting versuses BTX-A alone is useful for decreasing spasticity and improveing passive ROM of ankle in the children with CP, but injecting in hamstrings is not useful in decreasing spasticity or improving passive ROM in knees. Keywords: cerebral palsy, serial casting, botulinum toxin type-A A Novel 4p16.3 Microduplication associated with MR and facial dismorphy in an Iranian

patient by MLPA

Mohammad Taghi Akbari, Farzane Bahari Tehran Medical Genetics Lab Background: Mental retardation (MR) is a relatively frequent disorder and occurs in 1-3% of general population. Etiology of MR is various and includes genetic and environmental factors. One of the most common causes of MR is chromosomal aberrations such as microdeletions and microduplications. These aberrations often remain undetected by routine karyotyping. Methods: The aim of this study was to detect copy number variations using multiplex ligation-dependent probe amplification (MLPA) in patients with idiopathic mental retardation. In this study 50 patients with unexplained MR, a normal conventional karyotype and no clinically recognizable syndrome were screened for microdeletions and microduplications associated with MR by P096 MR2 MLPA kit. Findings: In total one patient with duplication in 4p16.3 was identified. This duplication was found in a 12 year old boy with severe mental retardation and facial deformity. The duplicated region contained 10 known genes including GAK, FGFRL, SPONE2, CTBP1, SLBP, TACC3, FGFR3, LETME1, WHSC1 and WHSC2. Some of these genes have been shown to be dosage sensitive, and may have a role in the 4p duplication phenotype. MLPA results for parents of the patient showed that this duplication is occurred de novo. Interestingly the site of this duplication is the same region for the deletion which causes Wolf Hirschhorn Syndrome (WHS).WHS is a malformation syndrome associated with mental retardation. Some clinical symptoms that observed in our case were similar to symptoms in WHS. Our study showed that not only deletion but also a small duplication in WHS region may lead to MR and facial deformity. Conclusion: This study demonstrated that submicroscopic rearrangements significantly contribute to idiopathic mental retardation and result in several mental retardation syndromes. Therefore screening patients with unexplained MR for these aberrations can be useful for detection of probable causes

of disease. Keywords: mental retardation, MLPA, microdeletion, microduplication

Which adhd children will be improved

through neurofeedback therapy ? Sahel Hemmati1, Mehran Ahmadlou2 1Pediatric Neurorehabilitation Research Center, University of Social Welfare & Rehabilitation Sciences. 2Dynamic Brain Research Office Neurofeedback training is increasingly used for ADHD treatment. However some ADHD patients are not treated through the long-time neurofeedback trainings with common protocols. In this paper a new graph theoretical approach is presented for EEG-based prediction of ADHD patients’ responses to a common neurofeedback training: rewarding SMR activity (12-15 Hz) with inhibiting theta activity (4-8 Hz) and beta2 activity (18-25 Hz). Eyes closed EEGs of two groups before and after neurofeedback training were studied in ADHD patients with (15 children) and without (15 children) positive response to neurofeedback training. Employing a recent method to measure synchronization, fuzzy synchronization likelihood, functional connectivity graphs of the patients’ brains were constructed in the full-band EEGs and 6 common EEG sub-bands produced by wavelet decomposition. Then, efficiencies of the brain networks in synchronizability and high speed information transmission were computed based on mean path length of the graphs before and after neurofeedback training. The results were analyzed by ANOVA and showed synchronizability of the neocortex activity network at beta band in ADHDs with positive response is obviously less than that of ADHDs resistant to neurofeedback therapy before treatment. The accuracy of linear discriminant analysis (LDA) in distinguishing these patients based on this feature is so high (84.2%) that this feature can be considered as a reliable characteristics for prediction of responses of ADHDs to the neurofeedback trainings. Also difference between flexibility of the neocortex in beta band before and after treatment is obviously larger in the ADHDs with positive response in comparison to those with negative response which may be a neurophysiologic reason for dissatisfaction of the last group to the neurofeedback therapy. Keywords: neurofeedback, ADHD, children

Overview of neuromuscular disorder

Seyed Hassan Tonekaboni Neurology Research Center, Shahid Beheshti University of Medical Sciences Neuromuscular diseases are a clinically and genetically heterogeneous group of disorders and anatomically extended from anterior horn cell to the muscle fibers. They can be classified as acquired or genetic. Regarding anterior horn cell the acquired form is poliomyelitis and spinal muscular atrophy is the genetically determined one. The genetic disorders of the peripheral nerve are named Charcot-Marie-Tooth with different types of Mendelian inheritance. They are electrophysiologically classified as axonal or demyenilating. The acquired demyelinating neuropathy is autoimmune based. The majority of


Neurology Abstracts

myasthenia in children are congenital forms. Congenital myastenic syndromes are anatomically categorized according to the site of the abnormality as pre-synaptic, synaptic or post-synaptic. The primary muscle tissue can be divided into congenital myopathies, muscular dystrophies, metabolic conditions, and acquired diseases. Endocrine myopathy and Juvenile dermatomyositis are acquired and the remaining are hereditary. A successful clinical approach to a patient with a suspected myopathy is based upon a thorough medical history and neurological examination. Associated clinical symptoms such as myotonia, contractures, myoglobulinuria, cardiac disease and respiratory insufficiency can be extremely helpful in focusing the differential diagnosis. Electrophysiological studies are necessary particularly if the condition resembles a neuromuscular junction disorder or CMT. Muscle imaging, ultrasound and MRI are useful loop for investigation of muscle disease. Muscle biopsies with all staining and molecular genetic tests are the last resort with very high accuracy. Keywords: neuromuscular disorders, congenital myopathies, electrophysiological studies

Comparison of the sleep patterns of Iranian pre-school children with and without sleep problem and its association with life style

factors Naser Kalantari, Maryam Javadi, Parisa Amiri, Shabnam Jalilolgadr, Nasrin Omidvar, Bahram Rashidkhani Qazvin University of Medical Sciences Background: Sleep pattern is affected by different factors including hormonal and chronobiological mechanisms, socio-cultural factors, exercises and parental employment. The objective was to compare the sleep patterns of Iranian pre-school children with and without sleep problem and its association with life style factors. Methods: The BEARS (Bedtime problems, Excessive daytime sleepiness, night Awakenings, Regularity and duration of sleep, Snoring), Iranian pediatric sleep questionnaire was used to identify the 3-6 y pre-school children having sleep problem (case group=70) and children without sleep problem (control group=140). All statistical significance was set at P <0.05. To assess the relation between sleep pattern and other variables the Man- Whitney U, χ2, Binary Logistic regression, Bivariate correlation, and Kruskal- allis H were used. Findings: Total hours and days spent for computer games, T.V watching and physical activity as well as bed time snack was not significantly different between the case and control groups (P> 0.05). Sleep patterns of children and their mothers was significantly associated (P<0.05). Usual bed time in two groups was significantly associated with parental jobs (P<0.05). The children having sleep habit of co-sleeping, had 2.9 fold increased odd of sleep problem (OR=2.92, 95% CI:1.36, 6.18, P=0.001) and the children whose fathers had the total sleep time of less than 8 hours during weekdays demonstrated 2.8 fold increased odd of sleep problem (OR=2.84, 95% CI: 1.24,6.49,P=0.01). Conclusion: Our findings support the association between family and lifestyle factors with sleep duration and sleep problem among preschool children. Keywords: preschool children, sleep pattern, parents

Relation between sleep habits and dietary pattern among Iranian preschool children

Maryam Javadi, Amir Javadi, Shabnam Jalilolgadr, Fatameh Akbari, Azita Homayonfar Qazvin university in medical science The objective was to investigate the sleep habit of preschool Iranian children and the role of calorie, macronutrients and caffeine intake in a mixed normal meal on sleep induction. Methods: A total number of 600 children of 3 to 6 years of age (108 Boys and 102 girls) randomly selected from 15 kindergartens in the city of Qazvin, Iran. Dietary intake measured using a 24 hr diet recall completed by a health worker. Sleep problem and sleep habits of the children were determined based on Iranian version of BEARS (Bedtime problem, Excessive sleepiness, Awakenings during the night, Regularity of sleep, Snoring) questionnaires was used to collect the following data total daily sleep time, usual bed time and usual wake-up time in the morning. Findings: Total sleep duration, sleep bedtime and wake-up time in the morning were 10.30(10-12) and 11(10.5-12.5 pm and 8.58-10.5 am) hours respectively. 28% of children had bed time sleep at 10 pm or later. The results of this study showed a significant relation between calorie and macronutrients intake of the children and the wake-up time in the morning as well as between children sleep habits with mothers job(p <0.05). Conclusion: Based on the results of this study a significant relation was seen between dietary pattern and mothers' job effect on children sleep pattern. Keywords: sleep habit, dietary pattern, preschool children

The effects of silently-whispered and/or

softly-pronounced verbal communication in occupational therapy for autistic children; A

qualitative study Fatemeh Behnia, Sara Nakhai Pediatric Neurorehabilitation Research Center, University of Social Welfare and Rehabilitation Sciences Research shows that autistic people view nonfeature areas of the faces significantly more often and core feature areas of the faces (i.e., eyes, nose, and mouth) significantly less often than normal controls. They’re also impaired relative to controls on face recognition tasks. We compared silently-whispered and/or softly-pronounced verbal instructions with loud and serious ones during occupational therapy (OT) sessions for autistic children to see whether it can improve eye contact and facilitate viewing core feature areas of faces and improve overall symptoms. Half of the children received OT sessions with silently-whispered and/or softly-pronounced verbal instructions (G1), and the other half with normally loud and serious verbal instructions (G2). Both groups showed improvement but G1 looked at therapist’s face for a longer time, tried to imitate therapist’s mouth movements, purposefully smiled at her, got better at following instructions, collaborated better during the therapeutic tasks, and were better at drawing faces. Results showed that silently-whispered and/or softly-pronounced verbal communications besides other OT techniques could help alleviate autistic children’s



symptoms and improve eye contact and face processing in them. Keywords: autism, occupational therapy, communication, silent, children

Effect of Occupational Therapy Home

Program on activities of daily living of 5-12 years old children with cerebral palsy

Sara Afshar, Mehdi Rassafiani, Sahar Johari, Razieh Falsafian, Seyed Ali Hosseini Pediatric Neurorehabilitation Research Center

Background: Cerebral palsy is one of the most common causes of physical disability in childhood that leads to various difficulties for person and reduces the level of independence and participation in activities of daily living. The aim of this study was to investigate the effects of Occupational Therapy Home Program on activities of daily living of 5-12 years old children. Methods: In this clinical trial, 48 children in the intervention and control groups were selected and randomly divided into two groups. Common practices of occupational therapy within four months for both groups were applied. The intervention group received Occupational Therapy Home Program in the form of workshops and educational pamphlets with telephone follow-up in addition to their visual treatment plan. Activities Scale for Kids (ASK) was used to assess activities of daily living. Kolmogorov-smirnov test, Fisher exact test, independent t-test and paired sample t-test was used to analyze the results. Findings: The results showed significant difference activities of daily living between two groups (p=0,026). There were significant differences in all subtest of activities of daily living (p<0,05),except play one (p>0,05). Conclusion: Occupational Therapy Home Programs increased activities of daily living of children with cerebral palsy and can be useful in addition to common occupational therapy programs. Keywords: occupational therapy, activities of daily living, cerebral palsy, education

Childhood Stuttering Soheila Shahshahani Pediatric NeuroRehabilitation Research Center Stuttering is most common disorder in speed, rhythm and fluency of speech that in which sounds, syllables, or words are repeated or prolonged, disrupting the normal flow of speech. These speech disruptions may be accompanied by struggling behaviors such as rapid eye blinks or tremors of the lips. Stuttering can make it difficult to communicate with other people, which often affect a person's ability to have friends, academic performance, and job opportunities. The impact of stuttering on a person's functioning and emotional state can be severe and may include fears of having to enunciate specific vowels or consonants, fears of being caught stuttering in social situations, self-imposed isolation, anxiety, stress, shame, or a feeling of "loss of control" during speech. The exact etiology or cause of stuttering is unknown, two types are recognized: developmental and acquired stuttering. Stuttering is usually diagnosed by a speech-language pathologist. Although

there is currently no cure for stuttering, there are a variety of treatments available. The nature of the treatment will differ, based upon a person's age, communication goals, and other factors. Treatment includes fluency shaping therapy, modification therapy, Electronic fluency device, using Anti-stuttering medications, support groups and the self-help movement. Among preschoolers, the prognosis for recovery is good, about 65% of preschoolers who stutter recover spontaneously in the first two years of stuttering, girls seem to recover well. Once stuttering has become established, the prognosis is more guarded, and only 18% of children who stutter after five years recover spontaneously.

Keywords: stuttering, fluency, childhood, development

Alternate-Day Prednisolone in Infantile Spasms

Sepideh Amouian,Mahmoud Mohammadi,, GholamReza Zamani,MahmouReza Ashrafi, Abolfazl Nasirian, Nahid Khosroshahi Golestan University of Medical Sciences Background: Hormone therapy (i.e., ACTH or prednisolone) have been the main treatment modalities in infantile spasms. The objective of our study was to compare the efficacy of daily used prednisolone (2mg/kg/d) vs. alternate day regimen in infantile spasms. Methods: 38 patients (15 girls and 23 boys, mean age of 11.2±6.8 months), referred to Pediatric Neurology Clinics affiliated with Tehran University of Medical Sciences, were randomized to receive daily (Group1, n= 20) or alternate day (Group 2, n= 18) prednisolone for 6 weeks and they were assessed for improvement of EEG and/or spasms. They were also assessed for the corticoids' complications every two weeks until the end of the study. Findings: After 6-weeks, 4 (33.3%) patients in each group revealed no improvement in EEG. Normal EEG or ≥50% reduction of electrographic abnormalities ≤ was found in 5 (41.6%) and 8 (66.7%) of the group 1 and 2, respectively (P 0.133). The rate of spasms had no change in 4 (26.7%) from group 1 and in 2 (16.7%) from group 2. Other patients revealed 50% reduction or cessation of spasms (P≤0.333). The patients in group 1 had more weight gain. There were no significant differences in other complications between the two groups. Conclusion: Daily or alternate-day prednisolone is almost equally effective for improvement of spasms and EEGs in infantile spasms. Because of some adverse events and similar effects of both regimens, the alternate day regimen of prednisolone may be more beneficial in the treatment of infantile Spasm. Key words: infantile Spasms, prednisolone, alternate day The impact of preterm birth on processing of auditory stimuli at the brainstem level in pre-

school age

Sara Hasani, Zahra Jafari Tehran University of Medicial Sciences Preterm birth is one of the risk factors that need to comprehensive examination. Our study was designed to investigate the impact of preterm birth on processing of


Neurology Abstracts

auditory stimuli and brain structures at the brainstem level in pre-school age. Methods: Auditory brainstem response test with low rates stimuli was recorded in 60 children with 4 to 6 years old, 30 of them had a very preterm labor and late preterm labor and 30 ones had a term labor as a control. Findings: Significant differences were observed in inter peak intervals of I-III, III-V and absolute latency of III wave (p<0.019). No significant differences were observed in amplitude of I and V waves. Tested ear had no effect on the results. Conclusion: Our results support the effect of pre-term birth on auditory brainstem responses and synchronization of auditory stimuli in pre-school age, and also need to follow up them in the next years. Keywords: child, auditory brainstem responses, click stimuli, preterm birth, latency Identification of Chromosome Abnormalities

in Subtelomeric Regions Using Multiplex Ligation Dependent Probe Amplification

(MLPA) Technique in 100 Iranian Patients With Idiopathic Mental Retardation

Roshanak Vameghi1, Farkhondeh Behjati2, et al 1Pediatric Neurorehabilitation Research Center. 2Genetics Research Center. 1,2University of Social Welfare and Rehabilitation Sciences

Background: The purpose of this research was to determine the results of subtelomeric screening by the

Multiplex Ligation Dependent Probe Amplification (MLPA) Technique in 100 selected patients with idiopathic mental retardation (IMR) in Iran. Methods: A number of 100 patients with IMR, normal karyotypes and negative fragile-X and metabolic tests were screened for subtelomeric abnormalities using MLPA technique. Findings: Nine of 100 patients showed subtelomere abnormalities with at least one of two MLPA kits. Deletion in a single region was found in 3 patients and in two different subtelomeric regions in 1 patient. Duplication was only single and was present in 2 patients. Three patients were found to have both a deletion and duplication. MLPA testing in the parental samples of 7 patients for whom it was accessible showed that 4 patients were de novo, 2 patients had inherited from a clinically normal mother and one had inherited from a clinically normal father. Screening with two MLPA kits (SALSA P036 and SALSA P070) proved abnormality in only five of the 9 patients. In 3 of these 5 patients we were able to conduct the array CGH whole genome test which confirmed the abnormality in all 3 patients. Conclusion: So, the prevalence rate of abnormal subtelomeres using MLPA technique in idiopathic MR patients in our study was 5-9%, the higher figure referring to the positive results of one of two MLPA kits, and the lower figure representing the results of positive double-checking with two MLPA kits and confirmation by array CGH. Keywords: subtelomeric abnormality, MLPA-mental retardation, idiopathic, array CGH

76

Hospital dentistry: indications & contra-indications

Hossein Afshar Faculty of Dentistry, Tehran University of Medical Sciences The development of hospital dentistry services has had a very slow pace in the last 50 years. In the last decades along with the growth of graduate and post graduate programs and the increasing training programs of the dental specialties including oro and maxillofacial surgery, pediatric dentistry, as well as the development of hospital dentistry fellowship programs, there has an opportunity arisen for the rendering of acceptable hospital dentistry services in the world and in Iran, which have all been well able to respond to following dentistry patients: 1- Patients unable to cooperate with the certain physical, mental or medical compromising disability. 2- Patients with dental restorative or surgical needs for whom local anesthesia is ineffective because of acute infection, anatomic variations or allergy. 3- The extremely uncooperative, fearful, anxious, physically resistant, or uncommunicative child or adolescent with substantial dental needs for whom there is no expectation that the behavior will soon improve. 4- Patients who have sustained extensive orofacial or dental trauma and/ or require significant surgical procedures. 5- Patients requiring immediate comprehensive oral or dental needs. 6- Patients requiring dental care for whom the use of general anesthesia may protect the developing psyche and/ or reduce medical risks. Moreover, parents’ growing awareness about the importance of the primary dentition that I have witnessed in the past 30 years both as an academic staff and as a pediatric dentist in the private clinic has necessitated new cases of rendering hospital services in pediatric dentistry; and with presenting 500 of such cases I try to elaborate on. Keywords: hospital dentistry, dental needs, coopration

Non-nutritive sucking

Mehdi Ghandehari, Sara Ghadimi Faculty of Dentistry, Tehran University of Medical Sciences To summarize, non-nutritive sucking in infants is nearly universal and is considered normal. The point at which non-nutritive sucking becomes a habit and is not considered normal is unclear. Numerous studies of the prevalence of thumb and digit sucking indicate that a large majority of Newborns suck their digits, but the percentage drops steadily with increasing age. These studies indicate that children spontaneously discontinue non-nutritive sucking sometime between 2 and 4 years of age. A variety of non-nutritive sucking habits exist, but thumb, digit, and pacifier sucking are most common. Pacifier habits depend on the child’s cultural background and may be encouraged in one setting and not in another. Children usually have little choice in the matter. Children often combine a non-nutritive habit with another repetitive activity, For example they may suck a thumb while carrying a personal blanket,

stuffed toy or favorite doll. Other children play with their hair or rub an article of clothing. In addition, certain situations and times of day influence the habit. Tired children are more likely to suck their thumb, as are children in new or threatening environments. Sucking a digit when entering and exiting sleep is the most common observation. Question often arise about non-nutritive sucking in infants and its effect on the developing orofacial Structures is whether non-nutritive sucking harmful or constructive? should it be encouraged or discouraged? Non-nutritive sucking, which consists of sucking fingers, pacifiers or other objects is considered a normal part of fetal and neonatal development. As early as 13 to 16 Weeks in utero, the fetus has started sucking and swallowing movements. Respiratory like movements also begin during this stage. These fetal movements are considered to be important precursors of the life-sustaining requirements of respiration and deglutition. Keywords: non-nutritive sucking

Radiation effects on oral tissues in Pediatrics

Laleh Mohamadi, Sahar Mohamadi Shahid beheshti University Radiation therapy for malignant lesions in the oral cavity is usually indicated when the lesion is radiosensitive, advanced or deeply invasive and cannot be approached surgically. Combined surgical and radiotherapeutic approach often provides optimal treatment. Increasingly, chemotherapy is being combined with radiation therapy and surgery. Children receiving radiation therapy to the jaws may show defects in the permanent dentition such as retarded root development, dwarfed teeth, or failure to form one or more teeth. If exposure precedes calcification, irradiation may destroy the teeth bud. Irradiation after calcification may inhibit cellular differentiation causing malformations and affecting general growth. Such exposure may retard or abort root formation, but the eruptive mechanism of teeth is relatively radiation resistant. Irradiated teeth with altered root formation still erupt. In general, the severity of the damage is dose dependent. In this paper we discussed the radiation effects and some cases will be introduced. Keywords: pediatrics, radiation effect, oral tissue, teeth

Diagnostic efficacy of different methods in the

assessment of adenoid hypertrophy Mohammad Sadeghi, Babak Saedi

Tehran University of Medical Sciences Background: This study was designed for better understanding of the role of different methods of nasal endoscopy in the assessment of adenoid hypertrophy and comparing them with lateral neck radiography and patients' symptoms. Methods: From August 2007 until January 2009, in the otolaryngology ward of a tertiary referral center, 89 patients who had symptoms related to chronic mouth breathing participated in this study. History of the

Otorhinolaryngology, Surgery & Dentistry



symptoms related to adenoid hypertrophy was obtained from them. In addition, all patients underwent nasal endoscopy and lateral nasopharynx x-ray. The clinician who did nasal endoscopy was blinded to information about clinical data and x-ray and vice versa. Afterward, the relationship between symptoms and each diagnostic procedure was evaluated. Findings: Patients had a mean age of 9.47 ± 4.68 years. In the evaluation of the relationship between symptoms grading and grading in lateral neck radiography, this relationship was significant about snoring. Furthermore, there was a significant relationship between the endoscopic size of adenoid and number of the episodes of acute otitis media. The sum of symptoms grading had a significant relationship with the size of adenoid in lateral neck x-ray, but not in nasal endoscopy. Conclusion: The results of the present study indicated that both radiography and nasal endoscopy could define the relationship between adenoid hypertrophy and associated symptoms and therefore are complementary. Between them, despite the popularity of nasal endoscopy, radiography can serve as a better planning tool. Keywords: adenoid hypertrophy, X-ray, endoscopy

Non-surgical treatment of medically resistant

adenoidal hypertrophy: a new perspective and an alternative to surgery

Mohsen Naraghi Department of Otorhinolaryngology, Head and Neck Surgery & Otolaryngology Research Center, Tehran University of Medical Sciences Adenoidal hypertrophy (AH) is the most common cause of pathologic mouth breathing in children. Other problems of in AH children include local such as nasal congestion, hyponasal speech, snoring, sleep apnea, chronic sinusitis and recurrent otitis media. Systemic long-term consequences include growth retardation, decreased cardiac ventricular function, increased diastolic blood pressure, behavioral changes, learning difficulties, poor attention span, hyperactivity and decreased intelligence quotient. Adenoidectomy is the standard treatment for patients with AH. Potential risks of anesthesia and complications of surgical procedure have persuaded promotion of non-surgical alternatives. Many investigations have been made in medical treatment of adenoid hypertrophy. They are included treatment of allergy, nasal sprays and antibiotics. However, in some cases AH continue to produce symptoms despite maximal medical therapy. In such case these patients are usually referred by pediatricians to otolaryngologists for adenoidectomy. During the last decade, three new alternatives to conventional adenoidectomy surgery -to reduce complications- have been presented by the author for the first time in Iran. However all these three procedures were performed under general anesthesia and could reduce but not eliminate the possible risks of surgery. During the last two years, the advances in expertise and technology led to the new advent of using outpatient setting for non-surgical procedure by the author as an innovative method for treatment of AH. Up to now more than forty children who were definitive candidates for surgical adenoidectomy were managed with radiofrequency treatment under endoscopy control without any need to surgical adenoidectomy and general anesthesia. The age range was between 4 to14. The compliance of the

children to such treatment was assessed by prior simple endoscopy at another session before the procedure. The parents were informed about the procedure and completed questionnaire before and one month after procedure. All children underwent direct endoscopic examination of adenoid before and after procedure. The pre-procedure adenoid size was in grade 3 to 4 from 4-grade endoscopic scale. The period of follow-up was up to 2 years. All patients were improved after non-surgical procedure. The results were impressive with excitement of some of the parents for obtaining results by incredibly simple procedure in contrast to surgery. Non-surgical radiofrequency reduction of adenoid size has the advantages of excellent magnified view of adenoid area, accurate reduction of central obstructing part of adenoid, non-bleeding, evaluation and treatment of other non-adenoid obstructing problems, complete preservation of normal nasopharyngeal structures, no general anesthesia, no operating room stress, no hospitalization, high safety, no complication and better tolerance of children with fast and easy procedure. Keywords: adenoid, adenoidectomy, hypertrophy, non-surgical, radiofrequency Role of early diagnosis and early Intervention

in comprehension of linguistic complex structures by children with hearing impaired

children R Teymouri1, S Hemmati2, F Teymouri3 1,2Pediatric Neurorehabilitation Research Center, University of Social Welfare & Rehabilitation Sciences. 3Bijar Health Network, Kurdistan University of Medical Sciences Background: Hearing Impairment certainly affects the normal development of speech and language acquisition. Clinical factors that may influence on the language acquisition and development of hearing impaired children are numerous, some of which include: the age of hearing impairment onset, the age of hearing loss detection…. The aim of this article was to study the role of early diagnosis and early intervention in comprehension of linguistic complex structures by children with hearing impaired children. Methods: In this case-control research, twenty normal children aged 6-7 years and twenty children with severe to profound hearing impairment aged 8-12 years were selected in a simple random sampling from normal kindergartens and schools and exceptional schools for hearing impaired people. The children didn’t have any confirmed diseases or neurological disorders except hearing loss in students with hearing impairment. The comprehension of sentences was tested by using a researcher-made task called sentence-picture matching task. At first the content validity was determined and then the reliability was confirmed with Cronbach Alpha Test. Data were analyzed by statistical test such as Independent Samples T-Test and Pearson's Correlation Test and using SPSS software. Findings: The results of this study showed that the hearing impaired children whose hearing loss was detected before acquiring the language and who had received early rehabilitation services presented better and had more comprehension of linguistic complex constructions compared to children whose hearing loss was diagnosed after the critical age.


Otorhinolaryngology & Dentistry

Conclusion: The results of this study suggest that the age of hearing impairment onset, the age of hearing loss detection and the age of receiving intervention services are associated with linguistic performance. There is also a correlation between these clinical factors and the ability of syntactic comprehension. These results imply the necessity of early detection and early intervention in hearing impaired children. Keywords: hearing impairment, comprehension, early detection, early intervention

The otitis media in sensourineural hearing

impaired children Naeimeh Daneshmandan Pediatric Neurorehabilitation Research Center, University of Social Welfare & Rehabilitation Sciences Background: Hearing loss in children is a silent and hidden disability. It has reported that three children in 1000 are born with congenital, significant bilateral Sensourineural hearing loss and three additional children in 1000 will acquire deafness in early childhood. On the other hand, otitis media (OM), the most common infectious disease of childhood, is the most common cause of hearing loss in children. Sensourinural hearing impairment (SNHI ) is not curable, but hearing impaired children would benefit from hearing aids and aural habilitation. The main purpose of this paper is to report the prevalence of otitis media and its effect on auditory threshold to highlight the impact of medical care on outcome of aural habilitation. Methods: A cross-sectional study developed on 80 SNHI children in Deaf Welfare Clinic between 2011-2012. Each case examined by otolaryngologist. OM were assessed by Impedance Audiometry, and auditory threshold(AT) evaluated by pure tone audiometry via dB HL. Auditory thresholds were recorded before and after treatment (BT) and (AT). Findings: Tympanic membranes were abnormal in 20(25%) children. Ear examination showed ear wax in 26 case, wax and OM in 6 (wax=40%) and OM in 11 cases (OM =21.25%). Mean of AT in right ear(BT) was 75.8(SD14.65) and (AT) was 63.6 (SD15.6) with PV=0.011. Mean of AT in left ear (BT) was 75.6(SD16.79) and (AT) was 63.6(SD21.41) with PV=0.018. The results were predictable through Wilcoxon Non parametric Test. Conclusion: Middle ear infections in SNHI are as common as other normal hearing children and can affect auditory threshold in this group. So regular otologic examination and audiometric evaluation in SNHI children is recommended. Keywords: ear disease, deaf, children, wax, otitis media Topical use of Mitomycin C and Fluorouracil

on coldknife myringotomy Yalda Jabbari moghaddam Tabriz University of Medical Sciences Background: The hypothesis is a comparison of the histopathological effect of topical use of Mitomycin C and 5- Fluorouracil in preventing myringotomy closure in rats. Study design is clinical trial. Materials: The study was performed on 43 rats that were divided into three groups. Study groups (A and B) and control group (C) after bilateral cold-knife myringotomy,

we applied Mitomycin C (MMC) 4 mg/ml to group A, 5-Fluouracil (5FU) 50 mg/ml to group B, and normal saline to group C. An examination of all ears of rats was carried out by otoscope on days 0, 1, 3, 5, 7, and then every five days up to 70 days. Each day’s closed myringotomies of all groups were examined. Findings: The mean of post myringotomy opening time was 37, 16, and 12 days respectively in MMC, 5FU, and saline. Patency duration of MMC group was significantly long (p<0.0001), but in histopatholgical examinations, sclerosis of tympanic membrane in MMC group showed the highest patency duration (p<0.0001). Conclusion: Mitomycin C significantly prolonged the duration of myringotomy patency time – longer than 5-Fluouracil and saline but with the adverse effects of tympanic membrane fibrosis. Keywords: Mitomycin C, 5-Fluouracil, myringotomy Maternal related factors affecting on cleft lip

and palate: A case control study Mehdi Mokhtari1, Hossein Motaarefi, Uonees Mohammadi, Elmira Akbari, Sakineh Zeynali Tehran University of Medical Sciences Oral clefts are common congenital anomalies and have different factors. The aim of this study was the survey of prevalence of oral clefts and maternal related factors between infants. Methods: In a case control study, 26 patients with cleft lip and palate who were diagnosed during 2009 and 2011 at Qamar bani hashim hospital (Khoy) were compared with 52 healthy infants. The two groups were matched by age and sex. Data collection instrument was interview with mothers of infants and their hospital records. The data were analyzed using SPSS-16 software. Findings: The prevalence of cleft lip and palate was about 1.27 in 1000 live birth. Positive family history, passive smoking, location, cosmetics, age of parent, consanguinity, psychiatric drugs, antibiotics and NSAIDs group increased the risk of developing cleft lip and palate. Conclusion: Life style changes can be considered as one of the interventions which can prevent cleft lip and palate development. With regard to maternal factors in the developing cleft lip and palate, inclusion of preventive programs in prenatal care programs can be prevent of this disease. Keywords: case control, cleft lip and palate, maternal risk factors

Study of interns' knowledge level about pediatric oral-dental health during 2011-2012

in medical universities in Tehran, Iran Mohammad Nasiri1, Maryam Karamali2 1Department of Pediatrics, Shahid Beheshti University of Medical Sciences. 2Department of OB & Gyn, Arak University of Medical Sciences Background: There are several oral problems in pediatrics with normal functioning, general health and quality of life for a pediatric to interact. The most common problem is tooth decay and periodontal disease. Although dental caries is preventable, this prevention must be start in practitioners offices, because the rate of children references to practitioners are more than dentists. This study has been



performed for understanding about the knowledge level of the teeth and mouth educational hospital interns of Tehran Medical Universities. Methods: In this cross-sectional descriptive study, 391 educational hospital interns from university of Tehran, Shahid Beheshti and Azad have been studied through 2011-2012. The information has been gathered via questioners' sheets. Findings: In this study the average age of items were 25.18 with standard deviation of 1.25 year. 44.2% of the items were males and the others females; the average score of them was 4.87 with SD of 1.73. In addition, the minimal score was zero and the maximum score was 10. The age, sex, and duration of education do not have any effect on the interns’ knowledge. Conclusion: The results of this study comparing with similar studies indicated that the level of intern’s knowledge to pediatric oral-teeth health is not optimum and it is necessary to conduct more studies and researches to determine unknown factors which are effective in dearth knowledge level of interns. We suggest that it needs to hold workshops within internship courses and add some training credits in intern’s educational course to rectify these inabilities. So it is necessary to include these subjects in curriculums to take effective steps in protecting and promoting social health and at the end, examine oral health knowledge in different target groups especially in pediatrician groups. Keywords: knowledge, interns, pediatric, oral–dental health

An unusual case of perforated appendicitis Mohammad Hosain Towhidifar, Mohammad Ali Dehghan, Hosian Afrand Social Security Organization Background: Appendicitis is a relatively rare cause of chronic or recurrent abdominal pain in children. Appendicitis can usually be diagnosed on Completion of a history and physical examination (abdominal pain, vomiting, right lower quadrant tenderness and guarding), although laboratory evaluation with a urinalysis and White blood cell count can be of assistance. In the few cases where doubt remains, plain films of the chest and abdomen can be helpful for differential diagnosis, but none of them can prove the appendicitis. Difficulties in early diagnosis particularly in children often led to complications such as perforation with mean time about 36 hours from the onset of symptoms .The shorter time period is seen in younger children which might be as little as 6 hours in children younger than 5 years. Thus most children younger than 5 years have perforated appendicitis at operation. Actually complications like abscess formation and appendix perforation will to be decreased in light of useful and diagnostic procedures like abdominal computed tomography scanning (abdominal CT scan). We here report

a patient with chronic recurrent abdominal pain whose evaluation led to appendicular abscess. Case presentation: A 4 year old girl presented to our emergency department with a history of abdominal pain which started from 20 days ago; fever, anorexia, constipation, nausea, vomiting, dysuria with bad odor urine were some other signs that led to seeking medical attention. She took some drugs with diagnosis of UTI (urinary tract infection) and viral gastroenteritis but without improving. She referred to us because of getting worse condition and admitted in our ward. Physical examination revealed low grade fever, conjunctivitis, with abdominal tenderness localized in right lower quadrant without radiation and rebound tenderness. A mass also was palpated in RLQ during abdominal examination. The results of hematological and biochemical analysis; including complete blood count and differential, alkaline phosphatase, aspartate aminotransferase, alanin aminotransferase, Na, K, urea, creatinine, blood sugar, urine analysis, erythrocyte sedimentation rate, C reactive protein, as well as abdominal ultrasound(US) and plain X-rays were not significant. After consultation with surgery attendant, intestinal intussusception is suggested with consideration of radiography evident and barium enema carried out for patient. While barium enema showed mechanical obstruction, the patient underwent laparotomy via lower midline incision considering intussusceptions. The appendix was perforated and appendicular abscess mimicking intestinal obstruction was detected. Conclusion: It seems that appendicitis must be kept in the differential diagnosis of any child who presents with abdominal pain. The child who has been sick for a week may well have a large appendiceal abscess walled off from the peritoneal cavity. In our case when the abscess began to leak into the free abdominal cavity, the child showed signs of extreme toxicity, oliguria, mottling of the skin, evidence of gram negative septicemia and a falling platelet. Radiographs may show signs of paralytic ileus or ever partial small bowel obstruction. This type of patient is most at risk for a disaster. Keywords: perforated appendicitis, appendicular abscess, abdominal pain, children

Congenital face malformation: Classification and embriology

Cleft lip and palate

Bernard Pavy AP-HP, Paris, France We présent the classification of embryological facial malformation : Paul TESSIER Classification and the modification from B.PAVY and Delmas. Cleft lip and palate description, antenatal diagnosis, evolution, principe of treatment are showed. Long term result of surgical and orthodontic treatment réalised by Hopital Saint Vincent de Paul multidisciplinary team.

80

Epidemiology of rheumatic diseases in children who referred to pediatric rheumatology clinic of

Imam Khomeini Yahya Aghighi, Seyed Reza Raeiskarami, Feridon Azizi Department of Pediatrics, Tehran University of Medical Sciences Background: Today a significant number of patients are referred to outpatient clinics for pediatric rheumatology clinics, because of rheumatologic problems and according to importance and extent of this group of diseases, evaluation of the clinical and epidemiological characteristics of pediatric rheumatology diseases is important. Methods: This project is a cross-sectional study on children who was 2 months and older and referred to pediatric rheumatology clinic of Imam Khomeini. All information data had been gathered from patients' records. The information data were analyzed with using SPSS software (these data are included age, sex, disease type, clinical features, laboratory findings). Findings: In this study, 852 patients were evaluated. Average age of female patients was 7.16±6.30 years and was 7.29±3.59 years in male patients; average age of all patients was 7.23±5.04 years. The most common ages were 6-10 years (39.6%). The frequency of JCP was 39.4%, frequency of JCA-P was 52.3% and for JCA-S was 8.3%. The incidence of JIA did not have show significant differences with patients' age (P=0.1) and patients' sex (P=0.6). The incidence of KD was 3.4%, for HSP was 9.2%, for BHS was 20.8% and incidence of JIA was 55.16%. The prevalence of positive RF in patients with different types of JIA (470) was 4.0%. The frequency of positive ANA in patients with different types of JIA (470) was 2.3%. Most common symptoms were arthralgia (38.7%) and arthritis (24.8%). Conclusion: This project can help to identify the correct diagnosis of pediatric rheumatology diseases and comparison of the epidemiological differences in Iranian population. Keywords: Epidemiological, rheumatic diseases, children and Imam Khomeini hospital, RA

Epidemiologic Features and Clinical Study of

Kawasaki Disease

Yahya Aghighi, Seyed Reza Raeiskarami, Parvin Akbari Asbagh, Aidin Tabrizi, Saharnaz Talebian Department of Pediatrics, Tehran University of Medical Sciences Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown etiology and the major cause of pediatric acquired cardiac disease worldwide, particularly in developed countries. This study characterizes epidemiologic and clinical features of KD in the Pediatric Rheumatology Department service in Imam Khomeini General Hospital. Methods: We investigated the epidemiologic and clinical features with coronary artery involvement of KD cases treated at the Pediatric Rheumatology Department service in Imam Khomeini General Hospital between

1990 and 2009. Frequency of many parameters including age, sex, season, clinical and laboratory findings, response to treatment and complications of the patients determined. Findings: During our 20-year study period, 120 KD patients < 15 years of age were admitted for KD. Among patients, 39.2% were at the extremes of the age spectrum, with 2.5% < 6 months and 36.7% > over 5 years of age with the male/female ratio of 1.3:1 and the classic KD/incomplete KD ratio of 3.1:1. KD recurred in 5% of all cases. KD occurred most frequently in the winter and least frequently in the summer. Coronary artery abnormalities (CAA) occurred in 4.2% of all KD cases. Conclusion: Kawasaki disease should be considered in any pediatric patients with a prolonged refractory febrile illness. Male to female ratio of patients was 1.3:1. The most common season at diagnosis was winter. The incidence of CAA was 0.04. Keywords: Kawasaki, coronary artery abnormality

Evaluation of serum adenosine deaminase changes

before and after treatment in patients with systemic lupus erythematosus, Henoch-Schonlein purpura

and Juvenile rheumatoid arthritis Vahid Ziaee, Ali Amiran, Mohammad-Hassan Moradinejad Department of Pediatrics,Tehran University of Medical Sciences Background: Adenosine deaminase (ADA) is involved in purine metabolism and plays a significant role in the mechanisms of the immune system. The aim of this study was to investigate the changes of serum adenosine deaminase before and after treatment in patients with systemic lupus erythematosus, Henoch-Schonlein purpura and Juvenile rheumatoid arthritis. Methods: The study comprised 103 patients with JRA, 17 with SLE and 30 Henoch-Schonlein purpura. The serum ADA were measured of all patients before and after treatment. Findings: During the disease activity (before treatment), the mean serum ADA in patients with JRA was 15.1±10.9 U/l, vs. 12.6±4.5 U/l in patients with Henoch-Schonlein purpura and 18.8±11.95 U/l in patients with SLE (P=0.1). The mean serum ADA in patients with Oligoarticular JRA was 12.1±4.3 U/l, vs. 15.9±8.9 U/l in Polyarticular JRA and 27.7±23.8 U/l in Systemic JRA during the disease activity (before treatment) (P=0.0001). There was no significant differences in changes of ADA levels between disease activity and control (p= 0.1) in SLE. In patients with JRA, there was significant differences in changes of ADA levels between disease activity and control (p= 0.01). There was no significant differences in changes of ADA levels between disease activity and control (p= 0.2) in Henoch-Schonlein purpura. Conclusion: Determination of ADA serum levels is as Noninvasive method, reliable and easy for diagnosis of rheumatoid arthritis and other inflammation diseases and could be used as alternative parameters representing disease activity. Also determination of ADA serum levels can be helpful as an indicator during the follow up course of treatment for this patients. Keywords: Adenosine deaminase, systemic lupus erythematosus, Henoch-Schonlein purpura, Juvenile rheumatoid arthritis

Rheumatology

81Proceeding of the 23rd International Congress of Pediatrics – Oct 2011

Rheumatology

Investigation the most common symptoms of Kawasaki in yazd hospitals during 5 years

Hamideh Azizkhan, Ryhaneh Mosaipoor Shohadaye Kargar Hospital, Social Security Organization, Yazd Background: Kawasaki disease is systemic acute vasculitis associated with high fever with unknown causes and approximately 20-25% of untreated patients experience coronary abnormalities, including aneurysms. This disease is most commonly seen in children younger than 5 year old. Prolong fever more than 5 days with four criteria from five typic criteria of disease will be proved disease recognition. But Kawasaki patients don’t refer to doctor with the typical signs of disease and sometimes they are atypical. Hence, we decided to survey the most prevalent typical signs of Kawasaki disease, because by this way rapidly recognition and on time treatment prevent from creation of heart disease. Methods: In this descriptive study after diagnosing Kawasaki disease in children in the hospitals in yazd province, we provided some questionnaires based on criteria on Nelson Textbook's table for prevalent signs of Kawasaki disease. The questionnaires were completed by pediatricians based on the survey of patients' dossier. Findings: In this study, we surveyed 100 patients with diagnosis of Kawasaki. The average age of patients was 3.33 and 44% of the patients were females and 56% were males. Regards to gained results, fever (100%), bilateral non-exudative conjunctivitis (41%), Iymphadenopathy (30%), rash (29%), and strawberry tongue(28%) were the most prevalent signs. 81 patients were afflicted to typical Kawasaki with the most prevalent signs included digestive signs (diarrhea, vomiting) (45.8%) and neurologic signs (headache, seizure) (11.1%) as well as reducing of conscious level. Conclusion: Despite Kawasaki is an infrequent disease, in patients who have prolonged fever, rash, bilateral non-exuative conjunctivitis as well as diarrhea, vomiting, headache and seizure the possibility of Kawasaki should be considered. Keywords: Kawasaki, prolonged fever, heart disease

The Six Categories, a new Guidance for

treatment of children with JIA Reza Shiari Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences Recent advances in the treatment of patients with juvenile idiopathic arthritis (JIA) have significantly improved outcomes of children with chronic arthritis. Treatment of JIA has dramatically changed with the widespread use of methotrexate and introduction of biologic agents. The ACR Therapeutic recommendations are nonsteroidal antiinflammatory drugs (NSAIDs), intra-articular glucocorticoid injections, nonbiologic disease modifying antirheumatic drugs (DMARDs), biologic DMARDs, and systemic glucocorticoids for treatment of systemic features of systemic arthritis. Because of the broad international input, the recommendations are expected to inform and benefit physicians throughout the world and to be a “useful guide to clinicians at all levels of experience with the treatment of JIA. Five treatment groups are identified in the 2011 ACR recommendations,

which differs from the most recent ILAR (International League of Associations for Rheumatology) classification criteria that names six categories. The five treatment groups are as followings: • Patients with a history of arthritis of four or fewer joints throughout the history of their disease course. This group can include patients with persistent oligoarthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis. • Patients with a history of arthritis of five or more joints throughout the history of their disease, which can include those with extended oligoarthritis, rheumatoid factor (RF-)– negative polyarthritis, RF-positive polyarthritis, enthesitis-related arthritis, and undifferentiated arthritis. • Patients with active sacroiliac arthritis, which includes children with clinical and imaging evidence of active sacroiliac arthritis. This group likely will include patients with enthesitis-related arthritis and psoriatic arthritis, as well as patients from any of the ILAR JIA categories. • Patients with systemic arthritis with active systemic features (and without active arthritis). An example would be a child whose arthritis resolved spontaneously or rapidly upon initiation of NSAIDs therapy but who had persistent fever. • Patients with systemic arthritis with active arthritis (and without active systemic features). An example would be a child whose systemic features resolved spontaneously or rapidly upon initiation of NSAIDs therapy but whose arthritis remained active. Each treatment group has its own features of poor prognosis, and the presence of one of these features qualifies the patient as having a poor prognosis. Keywords: juvenile idiopathic arthritis, chronic arthritis, RF

Incidence of limping and limb length discrepancy

following treatment of septic hip in children Mehrnosh Hassas Yeganeh1, Hamid Reza Seyyed Hossein Zadeh Ardebili2 1. Imam Khomeini Hospital, Tehran University of Medical Sciences 2. Akhtar Orhtopaedic Hospital, Shahid Beheshti University of Medical Sciences Septic arthritis of the hip in childhood is a serious disease affecting a growing hip. It has multiple sequelae and according to its sequelae, may result in severe disability. There are different classifications for these residual deformities in hip joint, all of which are based on radiographic findings. We assessed the clinical consequences of septic hip disease by evaluating the limping and leg length discrepancy in the affected children. We reviewed 18 children with 18 hips with sequelae of septic arthritis of the hip who were treated in Akhtar hospital. Septic arthritis occurred in all of these hips before the age of 6 years. 12 were males and 6 females. All children were treated by arthrotomy and appropriate antibiotic therapy. The mean time elapsed from the septic arthritis to our final clinical evaluation was 42 months (range 23 – 61). We evaluated the limping by testing for terendlenburg sign in the affected hip. We also measured the true limb length in the affected side and compared with the normal side. In 12 of 18 affected children, limping was evident all of whom had grade Ib and II radiographic sequels according to Forlin classification. In 13 of 18 children we found an average 19 mm (range 8 – 31) LLD by clinical evaluation all of whom had grade Ib and II deformity according to Forlin classification. Since all these children had some degrees of flexion contracture, some of this LLD may be attributed to

82 Proceeding of the 24th International Congress of Pediatrics – Oct 2012 Rheumatology

this flexion contracture. 11 of these 13 children had been treated by delayed arthrotomy. In one of the children, we found 8 mm overgrowth in the affected side, who had a grade Ia deformity according to Forlin classification. In all the rest of children who had grade Ia deformity (Forlin classification), limping and LLD was not evident. These findings show a correlation between radiographic consequences of septic hips and their clinical presentations. So treating this significant disease in an appropriate way can result in better clinical results.

Evaluation of adrenal function after

withdrawal long term-low dose corticosteroid therapy in patients with

juvenile rheumatoid arthritis Payman Sadeghi, Yahya Aghighi, Heshmat Moaieri, Mohammad Hasan Moradinejad Department of Pediatrics, Tehran University of Medical Sciences Background: Juvenile rheumatoid arthritis (JRA) is the most common chronic rheumatologic disease in children. Glucocorticoids are one of the common drugs that used for JRA treatment. The aim of this study is evaluation of the adrenal function after cessation of long term and low dose glucocorticoids therapy in patients with JRA. Methods: 36 patients with JRA entered in a cohort study. They were treated by less than 7.5 mg prednisolone daily which tapered gradually after clinical remission for a total period of 18 months. 2-4 months after discontinuing prednisolone, ACTH stimulation test was done in all pateints for diagnosing of adrenal insuficiency. Findings: 25 patients (69.4%) were females and 11 patients (30.6%) were males. Mean of age was 8.2±3.4 years. 32 patients (88.9%) had pauciarticular and 4 patients (11.1%) had systemic JRA. Adrenal function was normal in 32 patients (88.9%) and adrenal insuficiency was detected in 4 patients (11.1%) by ACTH stimulation test. Repeating ACTH test showed normal adrenal function in that 4 patients after three months follow up. Conclusion: Our finding showed that adrenal insufficiency is not common in the patients whom treated by long term and low dose glucocorticoids. More future studies are necessary for final judgments. Keywords: juvenile rheumatoid arthritis, adrenal function, low-dose corticosteroid therapy

Spectrum of mutations and carrier frequency of familial Mediterranean fever

gene in the Iranian population Tayebeh Sabokbar, Ali malayeri, Yahya Aghighi, Vahied Ziaee, Reza Ries karami, Abas shakoori Garekani Tehran University of Medical Sciences Background: MEFV gene is about 15kb of DNA and has 10 exons and encodes a 781-aminoacid protein most of the mutations located in exon 2and10. Our study was based on clinical picture of recurrent attacks of fever and muscles. A sharp increase in reactions of acute phase was already reported. A positive history of similar clinical

picture among siblings and close relatives, and a good response to colchicin were the key points for establishing the diagnosis. Methods: The samples for study were gathered from the patients who were referred to the genetic section of Imam Khomeini hospital for genetic confirmation of diagnosis of FMF.All of the patients were interviewed directly by pediatric Rheumatologist with a predesigned indicated form. Ethnicity, age of onset of signs and symptoms history of admission to hospitals, and a good response to colchicin were the key points for establishing the diagnosis. Findings: 58 patients with clinical features of FMF have been studied for mutations. In 12 patients (20%) no mutation could be detected. 62.7 percent of patients were from Azari ethnic group and 37.3 percent were from other ethnic groups. In 34 (57.6%) of patients age of onset of the disease was under 3 years. The time interval between disease onset and genetic diagnosis was difrent from 1 to 15 years. Most of mutations were detected in exon 10 in codons: M694V 27.3%, M694I 16.6%, M680I 7.1%, V725A7.1%, R761H8.3% and exon 2 with only one mutation in codon E148Q 15.4%. Here we found 2 rare mutations in patients from zanjan province in exon 10 in codons: A744S, K695R with 1.1% of frequency for each. Conclusion: The most frequent mutations were detected in 3 codons: M694V and M694I in exon 10 with 27.3% and 16.6% respectively and codon E148Q in exon 2 with 15.4%. Keywords: FMF, MEFV Gene, Azeri ethnic

MEFV gene mutations (M694V, V726A, M680I,

and A744S) in Iranian children with Henoch-Schönlein Purpura

Ahmad-Ali Nikibakhsh, Mohammad Houshmand, Morteza Bagheri, Hashem Mahmood Zadeh, Isa Abdi Rad Urumia University of Medical Sciences

Background: Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. Several risk factors play important role in pathogenesis of HSP. We aimed to study the MEFV gene mutations (M694V, V726A, M680I, and A744S) in Iranian children with HSP. Methods: 50 unrelated pediatric cases were studied regarding M694V, V726A, M680I, and A744S mutations using ASO-PCR method. Findings: 24% of cases had a mutation. 22% of cases had M694V mutations. One out of 50 (2%) patients had V726A mutation. In 76% of cases no mutation was determined. In other hand, 13 out of 100 alleles (13%) were carrier for one mutation. 12 out of 100 alleles had M694V mutations (%12) and 1 out of 100 alleles had V726A mutation (%1). In 87 out of 100 alleles no mutation was detected. M680I and A744S mutations were not found in tested group. Mutation study and analysis demonstrated that the most frequent mutation was M694V (22%). Frequency of alleles were 0.12, 0.01,0,0,0.13, and 0.87 regarding M694V, V726A, M680I, A744S, total mutation, and wild type alleles, respectively. Our findings imply that M694V was a dominant mutation. Conclusions: This report as the first investigation of its kind in Iranian Azeri Turkish patients implying that M694V mutations are more frequent in tested group in comparison with general population. So it is suggested that investigation of M694V mutations should be considered as genetic test for diagnosis of HSP among Iranian Azeri Turkish patients. Keywords: MEFV gene, M694V, V726A, M680I, A744S, mutations, Iranian, HSP

83

The effects of secondhand smoke exposure on infant growth, a prospective cohort study

Azam Baheiraei1, Azar shams2, Afshin Mohsenifar3, Anoshirvan Kazemnejad4, Mehrnaz Geranmayeh 5, Zinat Hatmi6, Mohammad Milani7, Ali Keshavarz 8 1,5Department of Reproductive Health. 2Department of Maternal and Child Health, 3Department of Toxicology. 4Department of Biostatistics, 6Department of Community Medicine. 7Department of Pediatrics. 8Nutrition and Biochemistry Department. 1,2,5,6,7,8Tehran University of Medical Sciences. 3,4 Tarbiat Modares University Background: There is no study in Iran evaluating the impact of cigarette smoke on infant growth. The purpose of this study was to determine the effects of cigarette smoke exposure on infants’ anthropometric development. Methods: This study was conducted on 51 cigarette smoke exposed infants and 51 non-exposed infants. They were evaluated for weight, height and head circumference three times; five to seven days, two months and four months after birth. Urine samples were also collected in each turn. Exposure to secondhand smoke was assessed through questionnaires and urinary cotinine levels. Findings: The weight and height of the exposed group were significantly lower than the non-exposed group (p<0.001) at two and four months after birth. Conclusion:The results showed that exposure to secondhand smoke during infancy may lead to weight and height growth reduction in the first four months of life. Keywords: Cigarette smoke exposure, Infant, Growth, Cotinine

Necessity of DNA banking from patients in

the post genome era Sirous Zeinali Pastur Institute of Iran Background: Research in the past several decades has shown that most conditions have a genetic underlying basis. New advances in high throughput sequencing (HTS) have paved the way for accurate and rapid diagnosis of genetic cause of a condition. Genetic analysis is mostly performed to know the molecular defect causing the disease. The information can help the clinicians to provide better treatment and also to help the family for prenatal diagnosis and PGD. New technologies have enabled us to perform genetic analysis with very small amount of DNA. For most genetic analysis there is a need for patients’ DNA. The DNA mostly comes from peripheral blood. In pediatric practice we face death of lots of children in need of genetic test. We at the Molecular Medicine Network of Iran have advised our network member to provide DNA banking for their patients. The more permanent and large scale is making cell line for each patient but this method is a difficult one and requires special equipment and facilities. DNA banking using specialized filter paper such as FTA (Whatman, USA) or DBC (KBC, Iran) can aid this approach by providing inexpensive yet reliable method for

storing patients’ DNA in the hospital, patient’s file in the clinic or at home. The paper is of small size and can hold enough amount of DNA to perform several times of molecular tests including HTS. We are now routinely banking our patients’ blood from every admission and have found it be very necessary approach to provide better medical genetic service to families. We suggest that every pediatric ward and pediatricians bank their patients’ blood for future uses. This is more important when there is higher possibility of losing the patient for various reasons including death. Keywords: DNA banking DBC blood

Evaluation of clinical presentation and polysomnographic findings of sleep apnea in

obese children referred to Qods hospital, Qazvin , 2011

Shiva Lotfi Darabadian1, Shabnam Jalilolghadr2, Azita Tavasoli3 1,3Tehran University of Medical Sciences. 2Qazvin University of Medical Science

Background: Sleep apnea is a life-threatening condition. The prevalence of sleep apnea in children has been reported between 0.7% to 10.3%, but in obese it is between 13% to 66%. The purpose of this study was evaluation of clinical presentation and polysomnographic findings of sleep apnea in obese children referred to Qazvin Children Hospital, Iran. Methods: The descriptive-prospective study was conducted by selecting the overweight and obese children aged 6 to 18 years. After parent’s allowance, the CSHQ and BEARS questionnaires were filled. Subjects underwent full night PSG for evaluation of sleep. Parameters scored manually by sleep subspecialist. The collected data were analyzed using statistical methods. Findings: 30 children (14 boys and 16 girls) were evaluated by mean age 11.18± 2.87 years. 21 cases had BMI over than 95% and 9 cases had BMI between 85% to 95%. The nocturnal symptoms were: sweating (43.3%), snoring (36.6%), talking (33.3%) and frequent awakening during sleep (26.6%).The daily symptoms were: daytime sleepiness, loss of attention, hyperactivity, morning headache and depression respectively. Children slept an average of 346.94±87.82 minutes. The mean of sleep efficiency was reported %75.71±15.42. The incidence of sleep apnea in obese children was reported 90%. The mean of apnea-hypopnea index was 12.55 times per hour. A significant correlation was observed between BMI and Arousal Index (P<0.001). Most of respiratory events occurred in N2 than the other stages of sleep (P<0.03). There was significant drop in arterial oxygenation correlate with apnea hypopnea events (P<0.004). Conclusion: Sleep apnea- hypopnea is surprisingly high in obese children. It is advised that all obese children evaluated for sign and symptoms of OSAHS in routine health care visits and, in case of strong clinical suspicion, assessed with PSG. Keywords: Polysomnography, apnea, obesity

Miscellaneous Abstracts


Miscellaneous Abstracts

Family interference on diagnostic or

therapeutic process in hospitalized children

Manoochehr Mahram, Fatemeh Teimouri, Shabnam Jalilolghadr Faculty of Medicine, Qazvin University of Medical Sciences and Health Services Background: Interference of hospitalized children’s family on diagnostic or therapeutic process is frequently observed which inhibits appropriate intervention of medical team. This qualitative study was performed to determine the main types and causes of family interference on diagnostic or therapeutic process in hospitalized children in Qods Pediatrics Hospital (Qazvin, Iran). Methods: All parents who inhibited some performances by medical team for their hospitalized children or discharged their patient through a written consent during March 2010-March 2011, were interviewed by the staffs of assistance unit and/or related nurses to obtain the main causes and to assess the ways of decreasing the interferences. Registered data were analyzed.

Findings: Average inhibition of hospitalization or continuance of therapy through giving written consent by the family was monthly 104 (about 11% of admissions) among which relief of symptoms of the disease(62%), personal/family problems(14%), dissatisfaction of hospital or treatment process(7%), tendency to transport the patient to other medical centers(7%) and worry about costs or no insurance coverage(6%) consisted the main causes. The most diagnostic procedure which about 50% of the parents inhibited was Lumbar Puncture (LP) and the cause was fear of paralysis or other harms following the procedure. Patiently advice and consultation by the physician to parents was effective in half of them to be convinced. Conclusion: More public education is needed to remove wrong believes about some medical procedures and to complete therapeutic course regardless relief of clinical signs and symptoms. Some national interventions to increase health insurance coverage can decrease worry in families. Absence of any committee or society to protect of children’s rights, especially in critical situations such as sickness has permitted some parents to neglect their children, for which establishment of such societies is recommended. Keywords: Child, Hospitalized, Interference, Diagnostic, Therapeutic

85

Survey the effects of mothers’ quality of relationship in social development of autistic

children Mahboobeh Abbasian, Alzahra University Mahdis Maghsudloo, Tarbiat Modares University Background: As we know, people interpret and are influenced with their social relationship. Quality of it may affect many domains of family and social environment. The aim of this study is assessing the effects of mothers’ perception of social support, depth of relationship and social conflict on social development of their autistic children. Method: 18 mothers of autism children filled quality of relationship questionnaire and Vineland social development scale. Also Autism children separated to 2 groups (3-5 years and 6-8 years). Dates analyzed with paired sample t test. Findings: Findings show that there is a significant relationship between social support and depth of relationship with social development in both groups (p<0/001). And there were no significant relationship between social conflict and social development in both groups. Conclusion: we can understand that mothers’ supportive relationship can directly affect the treatment schedule of autistic children and with regards to this reality that social relationship in autistic children is so weak, and mother has a main role in interpersonal communication so we must pay attention to mother satisfaction of her social and familial relationship. Keywords: quality of relationship, autism, social development

Comparison of sensory over responsivity in

autistic boys versus autistic girls Mahdis Maghsudloo, Tarbiat Modares UniversitY Mahboobe Abasian, MA in psychology, Alzahra University Ebrahim Pishyare, University of Social Welfare and Rehabilitation Sciences Background: Atypical responses to sensory stimulation are frequently reported to co-occur with Autism diagnosis. We measure sensory over responsivity in 7 domains and compare them between two group of boys and girls. Method: Parents of 18 autistic children (9 boys and 9 girls, ages 3-8 years) filled sensory over reponsivity questionnaire. datas analysed with T sample test. Findings: Findings show that there is a significant relationship between tactile subscale and boys group (p<0/001). Findings alsow show that there is a significant relationship between movement subscale and girls group (p<0/001). Conclusion: Our results suggest that not only sensory over responsivity occurs comorbid with autism but also we must take care about the severity of each domains and control it from childhood. Keywords:sensory over responsivity, autism

Evaluation of association between allelic frequency of single nucleotide polymorphism rs662799 of APOA5 gene and fasting glucose

levels in children affected with metabolic syndrome

S. Ghazaleh Fatemi1, Modjtaba Emadi-Baygi12, Parvaneh Nikpour3,4, Roya Kelishadi5,6, Mahin Hashemipour5,6 Shahr-e-Kord University of Medical Sciences Background: Metabolic syndrome shows a group of risk factors that arise together. Indeed, this syndrome indicates a group of heterogenic metabolic diseases that insulin resistance is the central component. This syndrome involves several metabolic paths such as dyslipidemia, central obesity, microalbuminuria and blood pressure. Among the genetic risk factors in development of metabolic syndrome are APOA5 gene polymorphisms. This gene has various single nucleotide polymorphisms that -1131T>C is the most frequent one in different populations. Methods: In this study, the association of this polymorphism and increased fasting blood sugar levels, as a risk factor for the metabolic syndrome, has been investigated. Totally, 50 patients and 50 healthy individuals were included in the study, according to ATPIII criteria. Allelic frequencies determined by Mismatch PCR-RFLP and sequencing. Findings: In patients, 80% were TT, 6. 7% were CT and 13. 3% were CC while in healthy individuals 70% were TT, 23. 3% were CT and 10% were CC. Furthermore, the fasting glucose levels in patients with -1131C allele and healthy individuals with -1131T allele were as follows: 96. 33±8. 11 mmol/l and 98. 36±13. 98 mmol/l, respectively. Conclusion: Statistical analysis (ANOVA) did not show any association between the fasting blood sugar levels and APOA5 genotypes (p= 0. 312). Keywords: Metabolic Syndrome, APOA5, single nucleotide polymorphism, Mismatch PCR- RFLP

Current Status of Zoonotic Cutaneous Leishmaniasis in a Hyperendemic Focus in

Central Iran

A Veysi 1, AA Akhavan 1,, H Vatandoost 1, MR Yaghoobi-Ershadi 1, MH Arandian 3, R Jafari 3, Y Rassi 1

1 Department of Medical Entomology and Vector Control, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran 2Institute for Environmental Research (IER), Tehran University of Medical Sciences, Tehran, Iran 3 Esfahan Training and Health Research Center, National Institute of Health Research, Tehran University of Medical Sciences, Esfahan, Iran

Background: Zoonotic cutaneous leishmaniasis (ZCL) is a major health problem in many rural areas of Iran, which has been reported in rural areas of 17 out of 31 provinces of the country. Due to improvement of reporting system by the Ministry of Health and Medical Education of Iran, natural disaster such as earthquake, urbanization,

Young Researchers Abstracts



constructions of buildings near colonies of the rodents, immigration of non-immune people to the endemic areas, a sharp increase of cases have been reported since 2004. Methods: The present study was carried out in 3 villages 45 to 50 km far from Esfahan City, Esfahan Province, Iran from November- December 2011. Totally two hundred and twenty seven households whose buildings were located next each other were examined and corresponding forms were completed for each household during active case-finding. The presence or absence of scars or ulcers of ZCL were indicated on the forms. Each individual was examined for scar(s) or ulcer(s) and the date and place of acquiring the disease, age, sex, number of ulcer(s) or scar(s), site of ulcer(s) or scar(s) were recorded. Findings: A study of prevalence among 794 inhabitants from 227 households in three villages showed a rate of 1.39 for active lesions. The most highly infected age group was 0-4 yr with a rate of 9.80, children under 10 yr of age had a rate of 5.50 for active lesions. The rate of the disease was 0.73 for those more than 10 yr old. The scar rate was 13.76 for individuals under 10 yr of age and 71.68 for those over 10 yr of age. The proportion of males and females were 422 and 372 respectively. Conclusion: It seems that the disease in these areas is hyperendemic and children are the most at risk group to be infected. Keywords: Hyperendemic, Zoonotic Cutaneous Leishmaniasis, Iran

Frequency of anti toxocara antibodies in schoolchildren with chronic cough and eosinophilia in rural area of the shiraz

A. Ghorbani Ranjbry1, N. Ghorbani Ranjbry2, Z. Ghorbani Ranjbry2, P. Cheraghi3 1.The Veterinary Medicines Student of Islamic Azad University of Kazeroon(the member of young scientific research club of Islamic azad university of kazeroon) 2. Khormshahar marine Science,Technology University, Iran (the member of young scientific research club of Islamic azad university of kazeroon) 3. Department of Food Hygiene , Faculty of Veterinary facuty, kazeroon branch, Islamic Azad University Background: Toxocara cat is the cause of Toxocariasis in cats that might lead to death of cats and also it is mainly the most prevalent reason causes by visceral larva migrants in children. Also the secondary stage larvae causes ocular, respiratory and neurotic disorders. The aim of this study was to determine the frequency of anti-toxocara antibodies in children with chronic cough. Methods: In a cross sectional study during a tuberculin survey rural area of the Shiraz, a city in southwest Iran a total of ۶10 children selected by randomized two-stage cluster sampling. Children with chronic cough were investigated for toxocariasis. Initially, the absolute eosinophil count was determined. Next, children with eosinophilia were tested for toxocara-IgG - ELISA kit (Ibl, Hamburg). Other epidemiological data collected by a questionnaire and analyzed with 95% confidence interval using SPSS 17 software. Findings: Of total 80 children with chronic cough, 12 (15%) were eosinophilic, among them 6.25% were hypereosinophilic, 9 (11.25) were ELISA-IgG positive for T. cati. No correlation between intestinal parasites and toxocariasis was detected (P>0.05).There was

also no significant age, gender, and living site difference in toxocara antibody frequency (P>0.05). Conclusion: In conclusion, the presence of eosinophilia and the risk factors evaluated in this population were frequently associated to human toxocariasis. Anti toxocara antibody was detected in more than half of children with chronic cough. Keywords: Toxocariasis, Toxocara cati, Anti-toxocara antibody, Eosinophilia, Chronic cough The effect of neurofeedback training on EEG

and balance performance in children with reading disorder

N. Sadeghi, MSc M. A. Nazari, Ph. D M. Alizade, Ph. D M. Kamali, Ph. D Tehran University of Medical Sciences Background: reading disorder is a neurodevelopmental disorder with deficits in cognition and motor skills. neurofeedback as a new treatment, can improve function in this disorder through regulating electroencephalogram (EEG) abnormalities. The purpose of this study was to investigate the effectiveness of neurofeedback balance protocol on improving EEG and balance performance in children with reading disorder. Methods: the study was conducted in a single subject design. Participants were 4 children aged between 8-12 years old who completed twenty 30-min neurofeedback sessions. Repeated measurements were performed during the baseline, treatment and follow-up by means of 2th subtest of BOTMP for balance performance and EEG for brain waves changes. Findings: The results showed that the effect of treatment on balance performance was high in all subjects but there was no significant changes in absolute power of brain waves (beta and theta). Conclusion: the results of this study indicated that neurofeedback balance protocol can improve balance in children with reading disorder and maybe longer treatment sessions are needed for significant brain waves changes. Keywords: neurofeedback ,readingdisorder, balance, EEG

Comparative statistics of dental caries in

primary screening examinations of first grade students, under the guidance of academic

health centers No. 2 in 2010-2011 and 2011-2012

Mitra Rezaei- Dr. Mahshid Ahmadian- Dr. Mojgan Talaei- Ashraf Ghanbari Isfahan University of Medical Sciences Background : Tooth decay is the most common of colds and flu, most people experience in their lifetime to understanding the effects of tooth decay can motivation for compliance is effective teeth and prevent tooth decay. Methods: This descriptive study is to compare students based on the first primary, middle and high schools under the city of Isfahan Health Center No. 2 screening examinations performed Findings: According to Screening examinations for first grade students of elementary and junior in two academic years 2010-2011 and 2011-2012 show interventions and health projects bases and training of health workers and increase the awareness and attitudes of family factors

87 Proceeding of the 24th International Congress of Pediatrics – Oct 2012 Young Researchers Abstracts

causing tooth decay, taking a bite of healthy schools, the correct way to brush, this study shows only students in grade school girls about 17.23% of caries has decreased, perhaps due to the sensitivity of the oral health of young girls and decay increases in other categories of interventions, other bases for measuring student enrollment in primary school was the region No. 5, one measure of the examination process, the company has been increased awareness of oral class participants before and after attending the company has about 43.56% increase Composed of A. This increased awareness will lead to good performance in the coming years and also increase the health system's mission and oriented toward preventive activities led that In oral health topics, learning how to properly care for the teeth of prenatal onset strength of teeth and In life, and especially to complement the permanent teeth enamel to increase the strength of the teeth should be strength. Interventions need to be done according to age groups and sex. Keywords: dental caries, screening, examinations, Comparative statistics

The effect of neurofeedback training and neurofeedback with occupational therapy

based on cognitive rehabilitation on executive functions in autistic children

Fateme Vosooghifard1, Mehdi Alizadeh Zarei2, Mohammad Ali Nazari3 1- School of Rehabilitation, Tehran University of Medical Sciences, Tehran, Iran 2- Department of Occupational Therapy, School of Rehabilitation, Tehran University of Medical Sciences, 3- Department of Psychology, Tabriz University of Medical Sciences Background: a lot of key problems in autistic children are related to executive dysfunction. The disorder is due to structural and functional disconnection between brain regions. In this regard, neurofeedback as a new treatment, can improve function in this disorder through regulating the brain waves. But behavioral changes usually appear after some changes in the brain so the purpose of this study was to investigate the overall effectiveness of treatment and behavioral changes by adding occupational therapy based on cognitive rehabilitation. Methods: the study used single system (A-B-A-B+C-A) design. Participants were 2 children with autism spectrum disorder who completed 41 sessions during the study. Repeated measurements were performed during the baseline, transitional, treatment and follow up by Wisconsin cord sorting and tower of London tests for executive functions. Findings: the result showed that the effect of neurofeedback training on executive function was high in all subjects. After adding the occupational therapy intervention the increasing effect was maintained and the rate of shifting attention as a subset of executive function improved. Conclusion: the results of this study indicated that neurofeedback can improve executive functions in autistic children and effects of neurofeedback can be more pronounced if occupational therapy was added and maybe more occupational therapy sessions are needed for significant behavioral changes. Keywords: high functioning autism, executive function, neurofeedback, cognitive rehabilitation

The relationship of maternal stress during pregnancy and childhood leukemia

Mir Saeed Zakeri1, Sina Sadeghi Mehr1, Shima Khan Ahmadi, 1Nazanin Cham Heidar1, Azim Rezamand1, Mahasti Alizadeh2 Address and Affiliations:1 Tabriz Children Hospital, Tabriz university of Medical Sciences, Tabriz, Iran2Department of Community Medicine, Tabriz University of Medical Sciences, Tabriz Iran3 Students research committee,tabriz,iran Background: Acute leukemia is the most common malignancy in childhood and so health care systems are deeply involved to rule out the factors that have effect on the incidence and/or outbreak of the disease, so the multiple costs of the disease can be diminished. The aim of this study was to determine the impact of maternal stress during pregnancy on childhood leukemia . Method: This study was conducted in Tabriz teaching children Hospital and Urmia teaching children hospital. The study of Tabriz area Included 111childhood cancer cases (<15 years of age) and their individually matched 222 control subjects and the Urmia area Included 62 childhood cancer cases (<15 years of age) and their individually matched 222 control subjects. Information determining the maternal stress during pregnancy was gathered by direct interview and mother’s comments by trained interviewers . Findings: The percentages were 32% and 21% in the case and control groups, respectively (p=0. 03, OR=0. 568, CI=0. 339-0. 950) in Tabriz and were 31% and 21% in the case and control groups, respectively (p=0. 044, OR=0. 580, CI=0. 308-1. 090) in urmia. Statistically, there seems to be association between maternal stress during preganancy and the occurrence of childhood leukemia. Conclusion: the findings show that the childhood acute leukemia is correlated with maternal experience of stress during pregnancy. This gives emphasis to the importance of mental maternity health care during pregnancy. More studies are needed to confirm the association of mentioned factors on the occurrence of leukemia in children. Keywords: acute leukemia, stress, fright, mental care Pregnancy assessment of reason to utilization

three-dimensional (3D) sonography in pregnancy

Barzegar M1, Saeedi F2 1- School of Medical Science, Tehran university of Medical Science, Tehran, Iran 2- School of Allied Medical Science, Tehran university of Medical Science, Tehran, Iran Background: Despite the speed, relative safety and reliability of the ultrasound in the advanced societies, this method use only once during pregnancy with the doctor prescription for a genetic disease or delivery mode. In Iran this method mostly uses for unnecessary reasons and genetic diseases evaluation is in the next rank. Researchers found that a significant number of nerve cells didn't move to the appropriate parts of the cerebellar membranes in the brains of fetal mice that repeatedly were used the ultrasound, Correct nerve cell's movement during embryonic period is essential for normal growth of the cerebellum. Goals are evaluation of causes to use type of ultrasound and them accomplish percent.



Methods: we performance the research with the deal some questionnaire among the pregnancy mothers in Tehran, Hamadan, Tabriz and Qom and analysis of the results obtained via SPSS software. Findings: 31% of patients were presented for either three-dimensional (3D) or 4D ultrasound. Statistically Tehran with 71% of the results for ultrasound application has highest awareness in terms of safety and this value in other cities is below 50%. Also 75% of patients with education level below diploma have no good knowledge about this method. The most important reason for more extended using of 3D or 4D ultrasound is that patients are not certain about the performance results of the ultrasound. Generally good level of trust was observed in the ultrasound result. Conclusion: Optimum codes is give awareness and increase the trust among individuals, especially in other cities (except Tehran) and people with education level below diploma. Regarding to rather majority of mothers to follow the fetus's status by family doctor's counselor, we realized to be transferred this awareness through the media, public health centers or family doctors to counsel with them. Keywords: three and four-dimensional ultrasound, genetic diseases, gender identify *Corresponding

Antimicrobial susceptibility pattern of kebsiella rhinoscleromatis strains isolated from consumed powdered infant formula

milk in NICU

Mehrnaz Taheripour1, Jalal Mardaneh2,3, Mohammad Mehdi Soltan Dallal2

1Science and Research branch, Islamic Azad University, Arak. 2 Dept. of pathobiology & Microbiology, School of Public Health, Tehran University of Medical Sciences. 3Prof. Alborzi Clinical Microbiology Research Center, Shiraz University of Medical Sciences

Background: K. pneumoniae subsp. rhinoscleromatis is a nonmotile gram-negative coccobacill bacteria in the Enterobacteriaceae family. This organism is found in the environment and is considered to be part of the normal flora of the skin, respiratory and gastrointestinal tracts of humans and animals. Milk and its products might represent important sources of pathogenic Klebsiella species (1,2). The goal of this investigation was isolation and determination antimicrobial susceptibility pattern of K. pneumoniae subsp. rhinoscleromatis strains isolated from consumed powdered infant formula milk in NICU ward. Methods: In this cross-sectional study, total 125 consumed powdered infant formula milk in NICU ward were surveyed for microbial contamination. FDA protocol was used for isolation and identification of K. pneumoniae subsp. Rhinoscleromatis from samples (3,4). Antimicrobial susceptibility test was carried out based on CLSI (2011) recommendations by using the standard disc diffusion method(5). Findings: In this study, K. pneumoniae subsp. rhinoscleromatis was isolated from 3 (2. 4%) of 125 powdered infant formula milk samples. Antimicrobial susceptibility results showed that all isolated strains are sensitive (100%) to amikacin, cefotaxime, nalidixic acid, chloramphenicol, ciprofloxacin, ticarcillin and minocycline. Conclusion: awareness of drug resistant Klebsiella spp. is important for the food industry. In the absence of control

measures, drug resistant organisms might be transmitted to humans by the consumption of milk, powdered infant formula milk and milk products. Keywords: powdered infant formula milk, K. pneumoniae subsp. rhinoscleromatis, antimicrobial susceptibility

The relationship of parental exposure to chemical substances and childhood leukemia

Nazanin Cham Heidar1, Shima Khan Ahmadi1, Mir Saeed Zakeri1, Sina Sadeghi Mehr1, Azim Rezamand1, Mahasti Alizadeh2 1Tabriz Children Hospital, Tabriz university of Medical Sciences, Tabriz, Iran 2 Department of Community Medicine, Tabriz University of Medical Sciences, Tabriz Iran 3 Students research committee Background: leukemia is one of the most common malignancies in children. There are plenty of evidences on the risk factors and determinants of this problem. Parental exposure to chemical substances such as toxins and pesticides is one of the possible factors. The aim of this study is to investigate the effect of parental exposure to such chemicals in East and West Azerbaijan, Northwest of Iran which is an agricultural area. Method: This study was conducted in shahid motahary hospital in urmia and teaching Children hospital in Tabriz. The study was a Case-Control study and it included 111childhood leukemia cases in Tabriz and 62 in urmia (<15 years of age) and 222 Controls. Data were gathered by direct interview by trained interviewers. Findings: The parental exposure to chemical substances in cases in urmia was 22% and in control group was 1% (P<0.0001, OR=0. 112, CI=0. 44-0. 291). While this exposure was 1% and 3% among cases and controls respectively (P=0. 50, OR=1. 7, CI=0. 34-8. 37). Conclusion: This study shows that the childhood leukemia has associated with parental exposure in urmia Whiles we don’t found this association in Tabriz. More studies needed to approach to more information about this relationship. Considering the importance of this topic it is better to do more studies on the relation between childhood leukemia and parental exposure to chemical substances. Keywords: childhood leukemia, parental exposure, chemical substance

Interleukin 6 single nucleotide

polymorphisms in allergic rhinitis

Armin Hirbod-Mobarakeh, Masoud Movahedi, Nima Rezaei, Rasoul Nasiri, Ali Akbar Amirzargar Tehran University of Medical Sciences Background: Allergic rhinitis (AR) is a polygenic inflammatory disorder of the nasal mucosa with an increasing prevalence worldwide. As interleukin (IL)-6 seems to be involved in development of allergic disorders, such as allergic rhinitis, this study was performed to evaluate the association of 2 promotor variants of IL-6 gene in AR. Methods: Ninety eight patients with AR were enrolled in this study. To determine background population allele frequencies, 140 healthy volunteers were recruited as the control group. Genotyping was done for two

89 Proceeding of the 24th International Congress of Pediatrics – Oct 2012 Young Researchers Abstracts

polymorphisms in promoter region of IL-6 gene (G/C at -174, rs1800795 and G/A at -597, rs1800797), using PCR sequence-specific-primers method. Findings: Patients homozygous for the G allele of rs1800795 in IL-6 had a 3.35 fold risk of having AR than those with the C allele. AA genotype in rs1800797 of IL6 was associated with increased risk of developing AR. G/G haplotype for IL6 (rs1800795, rs1800797) was significantly higher in the patient group. The male patient with GC genotype had lower IgE levels and also lower eosinophil count (p<0.05). The patients with AG genotype in rs1800797 had lower IgE levels (p<0.05). In a subgroup of patients who had persistent type of disease, a significant relationship was found between eosinophil percentages and rs1800795 and rs1800797. CC or CG genotypes in rs1800795 had a protective effect on developing a persistent type of AR. The patients with A allele in rs1800797 had less likelihood to have a persistent disease. Conclusion: We found that 2 promotor variants in IL-6, especially rs1800795, were predisposing factors for AR with negative heterosis pattern. These SNPs could also affect the clinical parameters, the nature of sensitivity and persistency of the disease in some subgroups of the patients. Keywords: Allergic rhinitis, Genetic susceptibility, Interleukin 6, Single nucleotide polymorphism

Isolation and molecular identification of anti-

cancer enzyme, L-asparaginase, producing Bacillus species

Saeed Gholamian, Ali Nazemi, Mirsaed Miri Nargesi Department of Microbiology, Tonekabon Branch, Islamic Azad University Background: During recent years, the life time of leukemia patients have had an increase by utilizing more effective anti-cancer drugs. “Asparaginase” is an enzyme that plays a key role in curing acute lymphoblastic leukemia (ALL) -the most common cancer in young children. Due to its antineoplastic property, it is widely used in chemotherapy. The goal of this study was to explore and introduce extracellular L-asparaginase enzymes produced by Basillus spp. which potentially possess more desired serological properties and less side effects comparing to those of common commercial types. The genus Bacillus is chosen because of its significance in producing extracellular enzymes. Methods: The Bacillus spp. isolated from protein rich soils were cultured in modified M9 media. Asparaginase producing colonies were differed based on color change in media. Then the produced enzyme was purified and its activity was evaluated. Also its molecular weight was measured. Those Bacillus spp. which were capable of producing enzymes with desired activity, were identified using molecular method. Findings: New L-asparaginase producer Bacillus strains were identified and general properties of extracted enzymes were logged. Conclusion: As extracted L-asparaginase from different bacteria has shown different anti-cancer effects, exploring for microorganisms that produce this enzyme, is one of the main ways that would lead us to an enzyme with ideal medical aspects. The other way is trough the targeted modifications that apply to currently available enzymes in order to reduce their side effects. Considering the

difficulties and costliness of this method, it is not cost-effective comparing to the method of this study. Keywords:L-asparaginase,Bacillus, Cancer, Leukemia, Children

Effects of gentamicin on rabbit newborns seminiferous tubes

Seyedeh Maryam Kameli1, Seyed Mohammad Hosein Noori Mougehi2, 1. Developmental Biology , School of Biological Science, Tehran University of Medical Sciences, 2. Tissue and Embryology, Faculty of Medical Sciences, Tehran University of Medical Sciences Background: Pregnant often faced to inflectional diseases that it is necessary to use injection gentamicin in treatment. The purpose of this study is to investigate the effects of gentamicin teratorgenic in different periods of pregnancy (before differentiation, differentiation and embryonic) Methods: in this study, 40 rabbits six months old divided to four groups of ten (8 female and 2 male) and they pregnant in polygamy method. First group (control) didn’t receive any medication. Second group (before differentiation) and third group (Differentiation period) 8 days and fourth group (embryonic) 20 days after intercourse, gentamicin injected 1.7/mg/kg doses. Then male newborn biopsied in two age groups (3 days and adult) and their testis discharged, they prepared to make color in H&E method and observed in light microscopy. Findings: The investigations shown the medicine before differentiation and during this period caused to simulate pubertal praecox, but there weren’t teratorgenic effects in embryonic period. Conclusion: It is suggested that if necessary this medication don’t use in before differentiation and during this period especially the fetus in male Keywords: Gentamicin, Pregnancy, Pubertal praecox, rabbit

Anticipatory grief reactions in fathers of premature infants hospitalized in NICU

Elaheh Rahininia, Students' Research Committee, Tabriz University of Medical Sciences, Tabriz, Iran Background: Entering to fatherhood state on one hand and the birth of preterm neonates on the other hand puts fathers' mental health at risk. Also, paying attention to fathers as a major supporter for mothers is essential. So, the present study was conducted with the aim of the examination of anticipatory grief reactions among fathers with premature infants hospitalized in neonatal intensive care unit. Methods: The current descriptive study was done on 120 fathers with premature infants hospitalized in neonatal intensive care unit in the hospitals of Tabriz in 2011. Fathers were selected using Convenience method of sampling and data collection was done by the use of Benfield anticipatory grief scale. The data were analyzed by the descriptive statistics and SPSS 13 software. Findings: all the fathers in the present study reported anticipatory grief reaction. The range of scores was between 0 and 3. The average of the obtained scores for the



fathers was 1/47± 0/78. The mostly noted reactions among them was preoccupation thinking or dreaming about infant, and the sadness feelings and to a lesser extent irritability and the feeling of anger, and the least reactions were related to physical reactions (disorders related to sleep and appetite) and guilt feeling. Conclusion: The obtained results show that fathers experience anticipatory grief after the birth of premature infants and their being hospitalized in neonatal intensive care unit. So, it is essential for the medical team to pay more attention to the fathers' cognitive and emotional reactions. Medical team's understanding of the fathers' grief reactions can help them in providing services and practical support and helping them to fit more and better. Keywords: Grief, Neonatal Intensive Care Unit (NICU), Fathers, Premature infant

Evidence based practice: a new way to care for the children

Shahzad Pashaeypoor, Tahereh Ashktorab, Maryam Rasouli, Hamid Alavi majd Shahid Beheshti University of Medical Sciences. Background: Evidence based practice is a framework for conscious, explicit and judging by the current best evidence in making decisions about patient care and it has improved patient outcomes and has reduced health care cost. Method: It was quasi-experimental study, a single group before and after. The sample included 21 nursing students. The importance of breastfeeding, intrauterine growth retardation and prevention of pregnancy hypertension trained by evidence based education based on diffusion of innovation model. A knowledge, attitude and adoption were evaluated before and after training. Findings: The mean knowledge scores, before and after intervention were 31±5.6 and 9.44±3 , a mean attitude scores were 39±6.6 and 9.57±5.5 and mean adoption scores were 2.38 and 33.45 (P=0.000). The results showed that there were significant differences between before and after scores. Conclusion: Evidence based practice is very essential to prevent disease, maintain and improve the health of mothers and children. It can improve health indicators. Keywords : Evidence based education- Children- nurse

Isolation and determination antimicrobial susceptibility pattern of Tatumella ptyseos strains isolated from Consumed powdered

infant formula milk in NICU ward Mehrnaz Taheri Poor1, Mohammad Mehdi Soltan Dallal2, Jalal Mardaneh2,3 1 Science and Research branch, Islamic Azad University, Arak 2Department. of Pathobiology, Microbiology, School of Public Health and Institute of Public Health Research, Tehran University of Medical Sciences, Tehran, Iran. 3Prof. Alborzi Clinical Microbiology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Background: Tatumella ptyseos is a rod-shaped, gram-negative, facultative anaerobic, within the Enterobacteriaceae family .It is an important opportunistic pathogen in human and cause disease in premature and

immunocompromised persons in all age groups. The aim of this study was to isolation and determination antimicrobial susceptibility pattern of Tatumella ptyseos strains isolated from Consumed powdered infant formula milk in NICU ward. Methods: In this cross-sectional study, total 125 consumed powdered infant formula milk in NICU ward were surveyed. Isolation and Identification of microorganisms was carried out according to FDA method. Antimicrobial susceptibility test was performed by using the standard disc diffusion method based on CLSI (2011) recommendations. Findings: Tatumella ptyseos was isolated from 4 (3.2%) of 125 powdered infant formula milk samples. The results showed that isolated strains are sensitive to aminoglycoside antibiotics (amikacin (100%), tobramycin (100%), gentamicin (100%)). Conclusion: Tatumella ptyseos is relatively unknown to many laboratory personals and can easily be missed in the routine diagnosis presses. However it is necessary these laboratory workers will become aware of and look out for Tatumella ptyseos in the different samples. Keywords: powdered infant formula milk, Tatumella ptyseos, antimicrobial susceptibility.

Maternal employment and children's

psychosocial adjustment Mansoureh Ahmadifaraz1, Heidarali Abedi1, Mehrdad Azarbarzin2 1. Department of Nursing & Midwifery, Khorasgan (Isfahan) Branch , Islamic Azad University, Isfahan, Iran. 2. Islamic Azad University, Najafabad Branch, Nursing and Midwifery faculty, Isfahan, Iran. background: Due to social and economic change and increase the presence of women in contemporary society, Need to pay more attention to how the psychosocial adjustment of children and adolescents with working mothers, are necessary. Therefore, this study aimed to explore the experiences of children of working mothers and their compatibility was investigated. Methods: In this study, a qualitative research approach to developmental theory (grounded theory) . data collection is In-depth interviews and unstructured data through field notes were collected from women And sampling continued until data saturation. Comparative Analysis of continuous data was analyzed by Strauss and Corbin. Findings: The main findings of this study are: Compatibility with alternative caregivers, better understanding of situations, independent, successful model, self-esteem and forced adjustment are some of the important factors for acceptance of children of working mothers in the study, which with the integration of these concepts, the main theme of "The psychosocial adjustment" was made. Conclusion: Children and teenagers who has a working mother has more autonomy and social adjustment but what is important is the suitable working hours of mothers for maintaining a good relationship with her child or teenager which has an important role for a healthy relationship of mother and child and increasing their compatibility. Key words: working mothers, Grounded theory, psychosocial adjustment, children

Documents

24 International Congress on Pediatrics