NUTRITION

Protein Energy Malnutrition (PEM) Etiology:

1. An inadequate intake of food both in quantity and quality2. Severe or frequent Infections like:

Diarrhoea Respiratory Infections Measles Intestinal worm infestation

3. Deficient Breat feeding and weaning:4. Malabsorption states5. Immunodeficiency states (HIV)

Syndromal Classification1. Kwashiorkor2. Nutritional Marasmus3. Marasmic Kwashiorkor4. Pre kwashiorkor5. Nutritional Dwarfing6. Under-weight

IAP classification: It is based on percentage of weight for age Nutritional Grade Percentage of standard weight for ageNormal > 80%Grade 1 71 – 80%Grade 2 61 – 70%Grade 3 51 – 60%Grade 4 Less than 50%

Wellcome Trust Classification (1969) Weight between 80 and 60% of expected by Harvard Std

With oedema KwashiorkorWithout oedema Undernutrition

Weight below 60% of expectedWith oedema Marasmic Kwashiorkor

Without oedema Nutritional KwashiorkorWeight-for-Height and Height-for-Age Classification (Waterlow)

Nutritional statusStunting

(% of height/age)Wasting

(% of weight/height)NormalMildly impairedModerately impairedSeverely impaired

>95%87.5 – 95%80 – 87%<80%

>90%80 – 90%70 – 80%<70%

Arnold classification (1969) Based on MACNutritional status Mid-upper Arm Circumference

Normal/SatisfactoryMild to moderate malnutrition

>13.5 cm12.5 – 13.5 cam

Severe malnutrition <12.5cm

Gomez’ classification: It is based on weight retardation using Harvard std.Normal nutritional status Between 90 and 110%1st degree, mild malnutrition Between 75 and 89%2nd degree, moderate malnutrition Between 60 and 74%3rd degree, severe malnutrition Under 60%

Clinical features of PEM FeaturesClinical

Marasmus Kwashiorkor

Muscle wasting Obvious Sometimes hidden by edema and fatFat wasting Severe loss of subcutaneous fat Fat often retained but not firmEdema None Present in lower leg and usually in face and

lower armsWeight for height Very low Low but may be masked by edemaMental changes Sometimes quiet and apathetic Irritable, moaning, apatheticAppetite Usually good PoorDiarrhea Often OftenSkin changes Usually none Diffuse pigmentation, sometimes flaky paint

dermatosisHair changes Seldom Sparse, easily pulled outHepatic enlargement

None Sometimes due to accumulation of fat

Diagnosis of Malnutrition:Nutritional anthropometry:This is a valuable index of assessment of nutritional status. 2/3 of children with PEM does not present with clinical signs but are diagnosed by anthropometry.

1. Weight for age: Most sensitive method when recorded serially. A decrease in weight gain / loss in weight can be seen within 1 month. It indicates wasting.

2. Height for age: Compares the child's height with the expected height for the age. This would detect stunting.

3. Weight for height: This compares a child's weight with the expected weight of the same height. It is useful for differentiating between acute and chronic malnutrition.

4. Mid upper arm circumference (MUAC): Normal MUAC for a child between 1-5 years of age is greater than 13.5 cm. If the MUAC is 12.5-13.5, the child has mild to moderate malnutrition and if it is less than 12.5 cm it is suggestive of severe malnutrition. This is useful for screening a large number of children but less useful in long term growth monitoring.

5. Chest/ Head circumference In PEM, chest circumference is less than Head circumference even after 2 years of age.

6. Body mass index: BMI is calculated as weight in Kg/height in M²; E.g. a 6 year old girl with BMI of 21 is overweight whereas 16 year old girl with BMI 21 is just above the 50th percentile

7. The most important measurement for malnutrition is the growth curve: weight for age is plotted as percentiles curves and growth is monitored over a period of time; it is also called road to health chart

Early detection of PEM: 1. Growth Chart:

a. The first indicator of PEM is underweight for age. b. The most practical method to detect this is to maintain growth charts. c. Flattenning of growth curve is the earliest sign.

2. MAC of between 125mm (12.5cm) and 135mm (13.5cm), YELLOW COLOUR, indicates that the child is at risk for acute malnutrition

Marasmus features:1. It results from inadequate intake of energy or both energy and proteins2. Usual age is less than 1 year infants who are breastfeeding when the amount of milk is

markedly reduced and those who are artificially fed3. It is a form adaptation syndrome to chronic underlnutrition; hence it is a non oedematous

form 4. Clinical:

1. There is severe wasting skin becoming wrinkled and loose2. Buccal pad of fat is lost giving a wise man’s appearance3. Infant have constipation or starvation diarrhoea4. Flat or distended abdomen with visible peristalsis5. Subnormal temperature and low pulse

Kwashirokor features:1. Lethargy, apathy, irritability are the initial features2. Muscle wasting and edema 3. Hepatomegaly4. Skin:

a. Depigmentataionb. Dry and crackling: flaky paint and paddy field dermatitis

5. Hair signs: a. Easy pluckabilityb. Brownishc. Flag sign

6. Theories of Causes of edema:a. Reduced albumin synthesisb. Aflatoxin poisoningc. Impaired renal functiond. Decreaed Na- K ATPase acivitye. Increased free radicals; methionine levels are low which is a precursor of glutathione

an antioxidantManagement of PEM:

Management of mild to moderate PEM: This is managed at home

1. Recommendations: 1. Energy 120-150 kcal/kg/day2. Protein 2-3 gm/kg/day

3. Foods advocated: 1. Double or triple mixes of cereals and pulses like dal, rice, khichdi, seasonal green

leafy vegetables, root vegetables, sugar, jaggery, milk, milk products and nuts like groundnuts.

2. Oil / ghee or butter is added to increase calories.3. Frequent small feeds with calories and proteins distributed proportionately are

encouraged.4. Zinc supplements

5. Miscellaneous: 1. Parents should be educated regarding the hygienic way of preparing and

handling food; personal hygiene and importance of safe and clean drinking water.

2. Basic advice regarding ORS (oral rehydration solution)use for diarrhoea, vitamin supplements, treatment of infections and infestations should be given

3. Child must be immunized as per schedule. Severe PEM: Hospital Management: (Severe acute malnutrition- SAM)

1. Resuscitation : 6 - 24 hours2. Acute Phase : 1 day - 1 week3. Rehabilitation : 2nd - 3rd weeks

1) Resuscitation (Treatment of Complications):Dehydration:

Assessment: Fluid and sodium given should not exceed 75% of allowances calculated on the basis of weight.

Hypothermia: The child is kept warm Hypoglycemia:

Blood sugar of less than 50 mg/dl; more common in Marasmus; 1-2 ml/kg of 10% dextrose as bolus; then maintenance with 10% dextrose and saline over 24 hours.

Infection:Broad-spectrum antibiotics are given.

Anemia:5-10ml/kg of packed red blood cells is transfused if hemoglobin is less than 5 mg/dl.

Xerophthalmia:If child is > 1 year or > 10 kg, vitamin A 100,000 units are given IM on days 1, 2 and 28.If child is less than 1year or less than 10 kg, 1/2 the above dose is given.

Congestive Cardiac Failure:It is common after day 3 of the acute phase usually in Kwashiorkor. Oxygen and Diuretics must be given.

Hypomagnesemia: It is corrected with 1 ml of magnesium sulfate given IM every 12 hours for 1-3 days.

Hypocalcemia: It is corrected with 1-2 ml/kg of calcium gluconate IV.2) Dietary Management:

Rule 1:

1. Calculations are to be based on actual weight (not the expected weight). 2. Initially 80 cal/kg/day and 0.7 gm/kg/day of protein.

Rule 2:Offer small feeds at frequent intervals (to avoid vomiting, hypoglycemia and hypothermia).

Rule 3:1. Step up intake gradually to 100 kcal/kg/day and 1 gm/kg/day of protein. 2. Intragastric / NG tube feeding or total parenteral nutrition may be required. 3. Fluid volume: 120 ml/kg/day.

High Energy Feeding:1. After the initial phase, Protein: 1.5 - 2 gm/kg/day and calorie to 150 - 180

kcal/kg/day. 2. Semisolids and solids are increased.

3) Rehabilitation: a. Cereals, pulses and vegetables, Bengal gram, groundnuts and green leafy vegetables

are added.b. Oils, sugar, fruit and vitamins and minerals are also added.

Recovery And Follow Up:1. Recovery is assessed by:

1. Improvement of general condition, alertness and smile.2. Return of appetite.3. Gain in weight of 50-70 gms/day.4. Disappearance of edema (7-10 days) and hepatomegaly.5. Rise in serum albumin over the first 2 weeks of therapy6. Recovery is complete when the child reaches his/her standard weight, which

usually takes 6-8 weeks.Complications of PEM:

Early Complications of PEM:1. Infections

Common: ARI, Diarrhea, TB, helminthiasisSevere Gram —ye sepsis, septic shock, DICOpportunistic Candidiasis

2. Hypothermia3. Metabolic

i. Hypoglycemiaii. Hypocalcaemia

iii. Hypomagnesemia iv. Dehydrationv. Hypokalemia

vi. Hypernatremia4. Lactose intolerance5. Severe anemia & other nutrient deficiencies eg.Pellagra; Vit.A deficiency6. Congestive cardiac failure7. Late (Recovery) complications:

Diarrhea (lactose intolerance)

CCF (high protein & solute diet)Unmasking of subclinical vit/mineral deficiencies

8. Nutrition Recovery syndromes:a. The refeeding syndrome is a potentially lethal complication of refeeding in patients

who are severely malnourished from whatever cause. b. Salt and water retention leading to oedema and heart failure, c. Hypokalaemia due to rapid cellular uptake of potassium d. Hypophosphataemia due to increased phosphorylation of glucose, e. Rapid depletion of thiamine, a cofactor in glycolysis, leading to wernicke’s

encephalopathy and cardiomyopathy, f. Hypomagnesaemia due to cellular uptake of this mineral.g. Kahn’s recovery syndrome is characterized by sudden onset of tremors and

encephalopathy, due to unmasked deficiency of gamma aminobutyic acid (gaba) h. Gomez recovery syndrome is characterized by progressive abdominal distension,

ascites, hepatomegaly and diarrhea, due to secondary malabsurption and hypokalemia.

i. Management: i. Monitoring of plasma glucose, sodium, potassium, phosphate, and

magnesium before and during refeeding. ii. Before refeeding correction of electrolyte imbalances, vitamin and trace-

element deficiencies iii. Volume restored gradually over12 to 24 hours. iv. A gradual increase in calories and proteins v. Thiamine should be given 30 minutes before refeeding,

Prognosis:Mortality 10-30%PEM may rcecur as socioeconomic conditions remains the same.Long term sequelae: Irreversible stunting and mental impairment.

Preventive Measures: 1. Promotion of breast-feeding.2. Development of low cost weaning food: 3. The child should be made to eat more food at frequent intervals.4. Nutrition education: Promotion of correct feeding practices.5. Home Economics6. Family planning and spacing of births7. Family environment: tender loving care(TLC) 8. Food fortification9. Immunization 10. Early diagnosis of any lag in growth.11. Early diagnosis and treatment of infections and diarrhoea.12. Development of supplementary feeding programmes during epidemics.13. Deworming of heavily infested children.

WEANING FOOD

(Complementary feeding)

1. Definition:a. Introduction of traditional family diet to an infant after 6 mo of age who is so far

depenadant on breastfeeding2. Rationale:

a. By the age of 6 months, birth weight is doubled and child is becoming more active. b. Exclusive breastfeeding is no longer sufficient c. To supply Iron which is insufficient in breastmilk d. Infant is developmentally ready for other foods e. The digestive system is mature to digest the starch, protein and fat in a non-milk

diet.f. Oral cavity has developed to handle semi-solid food

3. Important Principles for Weaninga. Avoid foods with high allergenic potential (eg. cow's milk, eggs, fish, nuts, soybeans). b. Iron-containing foods (meat, iron-supplemented cereals) are required. c. Zinc intake should be encouraged with foods such as meat, dairy products, wheat,

and rice. d. Phytates ntake should be low to enhance mineral absorption. Grains, legumes, nuts

& seeds contain phytates.e. Breast milk should continue to 12 mo; formula or cow's milk is then substituted.f. Give no more than 24 oz/day of cow's milk. g. Give no more than 4–6 oz/day of fruit juices.

4. Feedings:a. Beginning at 6 months, an infant can eat mashed or semi-solid foods.b. By 8 months most infants can also eat finger foods. c. By 12 months, children can eat the same food as consumed by the family

5. Good complementary foods are:a. Rich in energy, protein and micronutrients (particularly iron, zinc, calcium, vitamin A,

vitamin C and folate);b. Not spicy or salty;c. Easy for the child to eat;d. Liked by the child;e. Locally available and affordable

6. Five keys to safer fooda. Keep cleanb. Separate raw and cookedc. Cook thoroughlyd. Keep food at safe temperaturese. Use safe water and raw materials

Nutritional advice for 1 year child:

Childs expected weight is 10 kg; actual weight is 7 kg; Gr II malnutrition (IAP)Nutrition requirement is calculated for expected weight and the recommended diet is slowly stepped up to this level as per tolerance of the child Calories: 10x100= 1000

Proteins: 2x10=20 gmDiet plan for 1 year old child:

Calories ProteinEarly Morning: 100 ml of milk 60 3Breakfast: 1 idli 50 2

Dhall 3 tsp 30 1.5Ghee/oil 1 tsp 40 -

Noon 2 biscuits 40 1100 ml milk 60 3

Lunch: ½ cup rice 85 2Dhall 3 tsp 30 1.5Ghee/oil 1 tsp 40 -

Evening: ½ egg or 1 vada 40 3100 ml milk 60 31 banana 40` -

Night: ½ cup rice 85 2Dhall 3 tsp 30 1.5Ghee/oil 1 tsp 40 -

Total 730 23Breast milk: Not included >270 calories

BREAST FEEDING

Colostrum and mature milk 1. Secreted in the first 2–3 days after delivery. about 40–50 ml on the first day2. Colostrum is rich in:

1. white cells and 2. antibodies, especially Ig A, 3. larger percentage of protein,minerals and fat-soluble vitamins(A,E and K)

3. Colostrum is a baby's first immunization against many bacteria and viruses4. Colostrum is laxative, and helps the baby to pass meconium

Mature milk types:1. On the third day, an infant is normally taking about 300–400 ml per 24 hours, and on the

fifth day 500–800 ml.2. From day 7 to 14, the milk is called transitional, and after 2 weeks it is called mature

milk. 3. Foremilk is the milk that is produced early in a feed. It provides:

a. Plenty of protein, b. Lactose, other nutrients and c. Water.

4. Hindmilk is the milk that is produced later in a feed. a. Contains more fat.

b. Provides much of the energy c. Satietyd. Sleep

5. Preterm milk1. Preterm breast milk, has more protein,2. Higher levels of some minerals including iron, 3. More immune properties than mature milk,

Four key points for attachment (latching):1. Baby’ chin touching the breast2. Nipple and lower areola inside the mouth 3. some upper areola visible4. Lowe lip turned outside

Four key points for good position 1. The baby’s head and body should be in a straight line2. His face should face the breast, with his nose opposite the nipple3. His mother should hold his body close to hers4. Mother should support his bottom, and not shoulders and head

Reliable Signs of “NOT ENOUGH MILK” :1. Almost all mothers can produce enough breastmilk for one or even two babies, provided

the baby suckles effectively and breastfeeds as often as he wantsthe amount of milk the breasts produce is determined by the amount that the baby takes, and increases when the baby takes more

2. Possible signs of not enough milk are:a. Poor weight gain (<500gm/month) b. Passing small amount of urine (<6times/day= wetness test) c. Concentrated urined. Hard stools e. Frequent crying?f. No milk comes out when the mother expresses g. Breasts did not enlarge (during pregnancy) h. Milk did not "come in" (after delivery)

Reasons for not enough milk1. Delayed initiation of breastfeeding2. Poor attachment3. Infrequent feeds-8 upto 8 weeks; 6 upto 6 months4. Short feeds5. Using bottles or pacifiers6. Giving other foods or drinks7. Stressed mother8. Sick mother9. Oral pill ; pregnancy10. Sick baby

Lactation management of not enough milk:1. Help the mother practically 2. Sit quietly and privately or with a supportive friend.

3. Hold her baby with skin –to – skin contact if possible 4. Take a warm soothing drink5. Stimulate her nipples6. Massage or stroke the breasts lightly 7. Ask a helper to rub her back

Breastfeeding &Working Mother1. If working mother she should take maternity leave 2. xpress milk before leaving for work and somebody can feed using paladai3. Reverse the feeding pattern

Maternal Nutrition during Breastfeeding1. The average increase in calories to support breastfeeding is 750 calories/day2. Recommendations are to eat an additional 500 calories/day and let fat reserves

accumulated during pregnancy provide the rest3. Fluid

1. Fluid needs increase with breastfeeding2. Women need to drink 8 to 12 cups of fluid/day to prevent dehydration and

milk production Duration of breastfeeding:

• Exclusive breastfeeding for 6 months• Continue breastfeeding for 2 years with supplementary feeding (home food)

Breast milk contents

1. Vitamins/minerals:1. Breast milk normally contains sufficient vitamins for an infant 2. Vitamin D also reflects it’s level in mother.3. The vitamin K content of human milk also is low 4. Iron and zinc are present in relatively low concentration, but their bioavailability and

absorption is high. 2. Anti-infective factors:

a. Immunoglobulin A, coats the intestinal mucosa b. White blood cells kill micro-organisms;c. Whey proteins (lysozyme and lactoferrin) can kill bacteria, viruses and fungi;d. Oligosacccharides prevent bacteria from attaching to mucosal surfaces.

3. Other bioactive factors 1. Bile-salt stimulated lipase facilitates the complete digestion of fat2. Epidermal growth factor stimulates maturation of the lining of the infant’s intestine

4. Fat:1. Breast milk contains about 3.5 g of fat per 100 ml. 2. Breast-milk fat contains long chain polyunsaturated fatty acids (docosahexaenoic acid or

DHA, and arachidonic acid or ARA) which are important for the neurological development of a child.

5. Carbohydrate:a. Breast milk contains about 7 g of lactose per 100 ml, b. Oligosaccharides provide important protection against infection

6. Protein: a. The concentration of protein in breast milk is 0.9 g per 100 ml b. Breast milk contains less of the protein casein, c. Among the whey, or soluble proteins, human milk contains more alpha-lactalbumin;

7. Antibodies:1. Immunoglobulin A prevents bacteria from entering the cells; 2. Maternal live white blood cells in intestine of baby can kill micro-organisms; 3. Whey proteins (lysozyme and lactoferrin) can kill bacteria, viruses and fungi; 4. Oligosacccharides prevent bacteria from attaching to mucosal surfaces

Exclusive breast feeding

Definition: Exclusive breastfeeding means that an infant receives only breast milk from his or her mother or a wet nurse, or expressed breast milk, and no other liquids or solids, not even water, with the exception of oral rehydration solution, drops or syrups consisting of vitamins, minerals supplements or medicines

ADVANTAGES OF BREAST MILK-For baby:

1. Natural food available at the proper temperature and requires no preparation time. 2. It is fresh and free of contaminating bacteria, 3. Has the protective effects of breast milk against enteric and other pathogens 4. Free from allergy which may cause diarrhea, intestinal bleeding, occult melena, "spitting

up," colic, and atopic eczema.5. Secretory IgA prevents microorganisms from adhering to the intestinal mucosa. 6. It also contains substances that inhibit growth of many common viruses. 7. Antibodies provide lower incidence of diarrhea, otitis media, pneumonia, bacteremia, and

meningitis 8. Macrophages in human milk may synthesize complement, lysozyme, and lactoferrin. 9. Lactoferrin has an inhibitory effect on the growth of Escherichia coli in the intestine. 10. The lower pH of the stool protects against infections 11. Bile salt-stimulated lipase kills Giardia lamblia and Entamoeba histolytica. 12. passive transfer of T-cell immunity against TB13. Less chances for asthma and other atopic conditions, type 1 diabetes, celiac disease,

ulcerative colitis and Crohn disease and childhood leukaemia.14. obesity in later childhood and adolescence is less common among breastfed children 15. Less chances for increased blood pressure, altered blood cholesterol levels and

atherosclerosis in later adulthood. 16. Increased duration of breastfeeding has been associated with greater intelligence in late

childhood and adulthood, which may affect the individual’s ability to contribute to society.For Mother:

1. The psychologic advantages of breast-feeding; a feeling of being essential and a sense of accomplishment

2. The risk of postpartum haemorrhage may be reduced by breastfeeding immediately after delivery

3. the risk of breast and ovarian cancer is less among women who breastfed4. Breastfeeding mother tends to develop less obscity 5. Exclusive breastfeeding can delay the return of fertility - Lactation amenorrhea

For Family:

1. The cost of rearing a child by buying formula or cow’s milk is reduced2. The cost of frequent visits to hospital is reduced due to less incidence of illnesses 3. Lactation amenorrhea helps planning the family size in the first year 4. Infanticide is unknown if mother starts breastfeeding and develops bonding

Ten steps to successful breast feeding in health facilities:

1. Have a written breastfeeding policy that is routinely communicated to all health care staff.

2. Train all health care staff in skills necessary to implement this policy.3. Inform all pregnant women about the benefits and management of breastfeeding.4. Help mothers initiate breastfeeding within one half-hour of birth.5. Show mothers how to breastfeed and maintain lactation, even if they should be

separated from their infants.6. Give newborn infants no food or drink other than breastmilk, unless medically

indicated.7. Practice rooming in - that is, allow mothers and infants to remain together 24 hours a

day.8. Encourage breastfeeding on demand. 9. Give no artificial teats or pacifiers (also called dummies or soothers) to breastfeeding

infants.10. Foster the establishment of breastfeeding support groups and refer mothers to them

on discharge from the hospital or clinic.

VITAMINSVitamin A

Introduction 1. Vitamin A is made up of a family of compounds called the retinoids 2. There are essentially 3 natural forms of vitamin A:

1. Retinols: most active form and is mostly found in animal sources of food.2. Beta carotenes: provitamin A, is the plant source of retinol; provides twice the

vitamin A activity3. Carotenoids: exist in a free alcohol or in a fatty acyl-ester form. α-, β-, and γ–

carotenes; and β-cryptoxanthinsAbsorption:

1. It is a fat soluble vitamin 2. Bile is necessary for for its absorption in intestines3. Stored in liver4. Released from the liver into the circulation as retinol bound to retinol-binding protein (RBP),

which binds to the thyroid hormone transport protein, transthyretin; this complex delivers retinol (as well as the thyroid hormone) to tissues.

Derivatives of Vit.A 1. All-trans-retinol, the alcohol form is the regular Vit.A; is present only in animal tissues;

2. Retinyl palmitate: The storage form 3. Retinal: the aldehyde form; functions in vision; enables rhodopsin to mediate vision4. Retinoic acid: The physiologically most important acid derivative, functions at the gene

level as a ligand for specific nuclear transcription factors; thus, regulate many genes involved in fundamental biologic activities of the cell; is not reversible to other forms

Functions :1. Genomic role: retinoic acid regulates cell division, cell death, and cell differentiation2. physiologic role: reproduction, growth, embryonic and fetal development, and bone

development, respiratory, gastrointestinal, hematopoietic, and immune functions.3. Vision: retinal, is the prosthetic group on both visual proteins. low-intensity light isomerizes

11-cis retinal, to all-trans- retinal resulting in visual sensation4. Epithelium: Maintains the integrity of epithelium of skin, conjunctiva, cornea, urinary tract,

vagina, pancreatic and salivary ducts Deficiency manifestations:

1. “keratinizing mataplasia” of epithelium 2. Incresaed incidences of diarrhea and respiratory infections3. bronchial obstruction-bronchiectasis4. UTI5. Dry scaly skin6. Cystitis- hematuria and pyuria7. Growth retardation due to recurrent infections8. Anemia, apathy, mental retardation9. Increased intracranial pressure with & pressure on the optic nerve.

Risk factors for Vit.A deficiency: 1. Children with measles, respiratory disease, tuberculosis diarrhoea 2. Protein—energy malnutrition, which interferes with the storage transport, and utilization of

the vitamin3. Intestinal infestations and infections 4. NB born to mother with vit.A deficiency 5. Denial of Colostrum and early breast milk which are concentrated sources of vitamin A . 6. Bottle-fed children are often at even greater disadvantage7. patients with chronic malabsorption

Epidemiology of Vit.A deficiency:1. Boys are frequently at greater risk of xerophthalmia2. Xerophthalmia may be more prevalent at certain times of the year (measles, famine,

harvest)3. Clustering of cases ( cultural practices)4. 1-4 years more prone for corneal involvement5. 2-8 years: conjunctival xerosis

Serum levels 1. vitamin A normal serum retinol levels are above 1.0 to 1.4 μmol / litre. 2. Below 0.35 μ mol / litre is considered deficient.

WHO Classification of xerophthalmia:1. XN - Night blindness2. X1A - Conjunctival xerosis

3. Xl B - Bitot’s spot4. X2 - Corneal xerosis5. X3A- Corneal ulceration / keratomatacia < 1/3 corneal surface6. X3B- Corneal ulceraiuon / keratomalacia >1/3 corneal surface7. XS - Corneal scar8. XE - Xerophtbalmic fundus

Diagnosis. 1. Dark adaptation tests 2. Conjunctival impression cytology, 3. Relative dose response 4. Levels of retinol (pure vitamin A) in the blood.5. Levels of retinol-binding protein— simple diagnostic procedures

TREATMENT 1. Xerophthalmia is a medical emergency carrying a high risk of corneal destruction and

blindness, and/or sepsis and death2. concomitant treatment of underlying systemic illnesses and PEM6. Oral administration is preferred, because it is safe, cheap, and highly effective; 200000 IU

vitamin A is administered by mouth immediately upon diagnosis and the dose is repeated the following day; An additional dose is commonly given 1-4 weeks later; Children less than 6 – 11 months half of the oral dose

7. Intramuscular injection:1. 100,000 IU IM water soluble retinyl palmitate when child can not swallow Severe

vomiting or malabsorption Vit.A Requirements in normal status:

1. 1 IU is equal to 0.3 μg of retinol and 0.6 μg of β-carotene 2. Daily requirements: Infants 500 μg older children 600-1500 μg

Vit.A prophylaxis program in India - 20071. 100000 IU at 9 months with measles immunisation2. 200000 IU at 16-18 months, with DPT booster3. 200000 IU every 6 months, up to the age of 5 years.4. Thus a total of 9 mega doses are to be given from 9 months of age up to 5 years.

Dietary sources of Vit.A1. Lightly cooked green leafy vegetables, including leaves of the “drumstick” tree (moringa

oleifera), the various amaranths, cassava leaves, etc, 2. Red palm oil, and coloured fruits, such as papaya and mango3. Adding a small amount of edible oil will enhance the absorption of the b-carotene

Eye care 1. In the presence of corneal involvement, broad-spectrum antibiotic eye ointment should be

applied every 8 hours to reduce the risk of secondary bacterial infection 2. Until the causative agent is identified, antibiotics that cover a wide range of organisms,

especially Staphylococcus and Pseudomonas, should be chosen

Bb Preventable causes of blindness in children:

Condition Associated Factors Prevention Treatment

Cataract Genetic disease Counseling SurgeryMaternal rubella Rubella vaccineInjuries and inflammation Injury prvention

Corneal scarring Vitamin A deficiency Vitamin A supplementation twice a year

AntibioticsSurgery for advanced stages

Measles Measles vaccineNeonatal conjunctivitis Antibiotics and control of sexually-

transmitted infectionsHarmful traditional eye remedies

Health education

Trachoma Facial cleanliness and environmental improvement

Microfilaria Onchocerciasis (river blindness)

Insecticide spraying of affected area Single annual dose of ivermectin

Refractive error (blurred vision)

GeneticsEnvironmental factors

None Optical correction

Retinopathy of prematurity (ROP)

Oxygen therapy Limited use of low dose oxygen Follow up examination and optholmic referral

HYPERVITAMINOSIS – A:1. Toxicity can be induced in adults and children with chronic daily intakes of 15,000 μg and

6,000 μg, respectively. 2. Symptoms subside rapidly on withdrawal of the vitamin.

Signs of subacute or chronic toxicity may include 1. Headache; vomiting; anorexia; 2. Dry, itchy desquamating skin; 3. Alopecia and/or coarsening of the hair; 4. Bone abnormalities; swelling of the bones; hyperostosis affecting several long bones,

especially in the middle of the shafts.5. Enlargement of the liver and spleen; 6. Diplopia; increased intracranial pressure; irritability; stupor; 7. Desquamation of the palms and the soles of the feet is common. 8. Hypercalcemia and/or liver cirrhosis may be present.

In young children 1. Vomiting and bulging fontanelles. 2. Anorexia, pruritus, and a lack of weight gain. 3. Drowsiness; diplopia, papilledema, cranial nerve palsies, and 4. Other symptoms suggestive of pseudotumor cerebri.

Pregnancy 1. Severe congenital malformations occur in infants of mothers who consumed therapeutic

doses during the 1st trimester of pregnancy for treatment of acne 2. This results in a high incidence (>20%) of spontaneous abortions and birth defects

Excess b-carotenes (carotenemia)1. Excessive intake of carotenoids is not associated with toxicity,

2. But may cause yellow coloration of the skin that disappears when intake is reduced;

RICKETS

Vitamin D:1. Two forms are important in humans:

a. Ergocalciferol (vitamin D2) synthesized by plantsb. Cholecalciferol (vitamin D3) synthesized by humans from 7-dehydrocholesterol

in the skin when it is exposed to ultraviolet B (UVB) rays from sunlight.2. Metabolism:

a. In Liver it si converted to 25-hydroxyvitamin D (25-OH-D) by the action of 25-hydroxylase

b. 25-OH-D undergoes a second hydroxylation in the kidney to become 1,25-dihydroxyvitamin

3. Functions of Vit.D: a. It is a Steroid hormone b. Active form: 1,25-dihydroxy vitamin D3 (Calcitriol) c. Action via intracellular receptor d. Regulates Ca++& Phosphorus homeostasis e. Essential for normal absorption of calcium from the gut

4. Vit.D in diet: a. Animal: fish, eggs, fortified milk, and cod liver oilb. Vegetable source: mushroom

Rickets: 1. It is a disease of growing bone before fusion of the epiphyses; 2. Uncommon in PEM as their growth is restricted.3. The mineralization of matrix at the growth plates does not occur as Ca is not availble due to

Vit D deficiency.4. Increase in the circumference of the growth plate and the metaphysis causes widening of

the wrists and ankles. 5. General softening of the bones leads to a variety of bone deformities.6. Osteomalacia is a disorder of mineralization of newly formed matrix in adults

Etiology of rickets: a. Vitamin D disorders, b. Calcium deficiency, c. Phosphorous deficiency, and d. Renal causes

Classification of Rickets:A. VITAMIN D DISORDERS

1. Vit.D deficiency:1. Nutritional2. Congenital3. Secondary4. Malabsorption5. Increased degradation

2. Decreased liver 25-hydroxylase3. Vitamin D–dependent rickets type 1&24. Chronic renal failure

B. CALCIUM DEFICIENCY1. Premature infants (rickets of prematurity)2. Malabsorption3. Primary disease4. Dietary inhibitors of calcium absorption5. Low intake

C. PHOSPHORUS DEFICIENCY:1. Inadequate intake2. Premature infants (rickets of prematurity)3. Aluminum-containing antacids

D. RENAL Rickets:1. Hypophosphatemic rickets

a. X-linkedb. Autosomal dominant

2. Overproduction of phosphatonin3. Tumor-induced rickets4. McCune-Albright syndrome5. Fanconi syndrome6. Distal renal tubular acidosis

Nutritional Rickets:

Etiology of Nutritional rickets:1) Transplacental: if mother has poor source of vit.D, infant may have congenital rickets2) Breast fed infants has less vit.D (low content) 3) Deficiency of cutaneous synthesis:

i. Lack of exposure to sunlight due to illness or cultural factorsii. High skin pigmentation(dark color) which filters UV light

iii. Prolonged winter months4) Unfortified formula milk 5) Phenobarbital and Phenytoin 6) Aluminum containing antacids interferes with absorption of phosphate from gut7) Malabsorption of vit.D occurs in:

i. Fat malabsorption (liver disorders): celiac disease and cystic fibrosis ii. Chronic diarrhea

iii. Giardiais Clinical features of rickets:

1. Breast-fed infants of vitamin D-deficient mothers may develop rickets within 2 mo, but rickets usually appears toward the end of the 1st and during the 2nd yr of life.

2. Vitamin D-deficient rickets is rare later in childhood;3. It is rare in PEM due to depressed growth

Cranium:1. Craniotabes is caused by thinning of the outer table of the skull

2. The softness of the skull may result in asymmetry of the head. 3. The anterior fontanel is larger than normal, and its closure may be delayed until after the

2nd yr of life. 4. parietal and frontal bosses giving the head a boxlike appearance (caput quadratum or hot

cross bun appearence)Dentition:

1. Eruption of the temporary teeth may be delayed, and there may be defects of the enamel and extensive caries.

Chest:1. Palpable enlargement of the costochondral junctions (the rachitic rosary)2. The sternum with its adjacent cartilage appears to project forward-pigeon breast deformity3. horizontal depression in the lower chest, Harrison groove, develops along the lower border

of the chestPelvis: Deformity of the pelvis in girls add to the hazards of childbirth Limbs:

1. The epiphyseal enlargement at the wrists and ankles 2. Weight bearing produces bending of the softened shafts resulting in bowlegs or knock-knees3. Greenstick fractures in the long bones 4. knock-knees, overextension of the knee joints5. tibial malleolus gives the impression of a double epiphysis (Marfan sign).

Spine: kyphosis and scoliosis Stature: rachitic dwarfism.Muscle tone: hypotonia and delayed walkingTetany: Tetany due to vitamin D deficiency occasionally accompanies rickets. Investigations:

1. Calcium (ionized fraction) is low; becomes normal as parathyroid hormone levels increase.2. Calcidiol (25-hydroxy vitamin D) levels are low, 3. Serum parathyroid hormone levels are elevated; 4. Calcitriol levels maybe normal or elevated because of increased parathyroid activity.5. The phosphorus level is always less than 4 mg/dLlow 6. Alkaline phosphatase levels are elevated. 200 IU/dL in normal children, increases to more

than 500 IU/dL7. Urinary cyclic AMP level is elevated8. A generalized aminoaciduria occurs from the parathyroid activity; aminoaciduria does not

occur in familial hypophosphatemia rickets (FHR).9. Phosphaturia with occasional glucosuria can occur

Imaging:1. Anterior view of the knee reveals metaphyses with cupping and fraying2. Along the shaft, the uncalcified osteoid causes the periosteum to appear separated from the

diaphysis giving a double contour appearance3. Green stick fractures

Treatment of nutritional rickets:I. Vit.D3 oral daily 50-150 μg or 1,25 dihydroxy cholecalciferol .5 to 2 μg

II. Alternatively a single dose of 15000 μg (600,000 U) of Vit.D Prevention:

I. Adequate exposure to UV lightII. Supplemt of 400 Iu vitD daily to breastfed infants

III. Vit D to pregnant and lactating mother to increase the levels in NB

Vitamin D–Dependent RicketsType 1 (renal 1α-hydroxylase deficiency)

1. An autosomal recessive disorder, have mutations in the gene encoding renal 1α-hydroxylase, preventing conversion of 25-D into 1,25-D.

2. These patients normally present during the 1st 2 yr of life 3. May present with symptomatic hypocalcemia. 4. They have normal levels of 25-D but low levels of 1,25-D 5. Renal tubular dysfunction can cause a metabolic acidosis and generalized aminoaciduria.

Treatment: 1. These patients respond to long-term treatment with 1,25-D (calcitriol). Initial doses are 0.25-

2 µg/day, and lower doses are used once the rickets has healed. 2. Especially during initial therapy, it is important to ensure adequate intake of calcium.

Type 2 (defective receptor for 1,25-D)

1. Autosomal recessive2. Mutations in the gene encoding the vitamin D receptor, preventing a normal physiologic

response to 1,25-D. 3. Levels of 1,25-D are extremely elevated 4. 50-70% of children have alopecia, which tends to be associated with a more severe form of

the disease and can range from alopecia areata to alopecia totalis. 5. Epidermal cysts are a less common manifestation.

Treatment 1. Some patients respond to extremely high doses of vitamin D2, 25-D or 1,25-D, especially

patients without alopecia. This response is due to a partially functional vitamin D receptor. All patients with this disorder should be given a 3-6 mo trial of high-dose vitamin D and oral calcium. The initial dose of 1,25-D should be 2 µg/day, but some patients require doses as high as 50-60 µg/day. Calcium doses are 1,000-3,000 mg/day. Patients who do not respond to high-dose vitamin D may be treated with long-term intravenous calcium, with possible transition to very high dose oral calcium supplements.

2. Treatment of patients who do not respond to vitamin D is difficult.

RENAL RICKETS

1. X-Linked Hypophosphatemic Rickets a. The defective gene is on the X chromosome, but female carriers are affected, so it is

an X-linked dominant disorder.b. The gene appears to have an indirect role in inactivating the phosphatonin FGF-23. It

leads to inhibition of phosphate reabsorption in the proximal tubule, and phosphate excretion is increased.

c. FGF-23 also inhibits renal 1α-hydroxylase, leading to decreased production of 1,25-Dd. These patients have rickets, but abnormalities of the lower extremities and poor

growth are the dominant features. Delayed dentition and tooth abscesses are also

common. Some patients have hypophosphatemia and short stature without clinically evident bone disease.

e. Patients have high renal excretion of phosphate, hypophosphatemia, and increased alkaline phosphatase; PTH and serum calcium levels are normal

f. Patients respond well to a combination of oral phosphorus and 1,25-D (calcitriol).2. Autosomal dominant hypophosphatemic rickets (ADHR):

a. It is much less common than XLH. There is incomplete penetrance and variable age of onset

3. Autosomal recessive hypophosphatemic rickets:a. It is an extremely rare disorder due to mutations in the gene encoding dentin matrix

protein 1, which results in elevated levels of FGF-234. Hereditary hypophosphatemic rickets with hypercalciuria (HHRH):

a. It is a rare disorder that is mainly described in the Middle East. This autosomal recessive disorder is due to mutations in the gene for a sodium-phosphate cotransporter in the proximal tubule.

5. McCune-Albright syndrome: a. It is an entity that includes the triad of polyostotic fibrous dysplasia,

hyperpigmented macules, and polyendocrinopathy. Affected patients have inappropriately low levels of 1,25-D and elevated levels of alkaline phosphatise.

6. Fanconi syndrome:a. It is secondary to generalized dysfunction of the renal proximal tubule. There are

renal losses of phosphate, amino acids, bicarbonate, glucose, urate, and other molecules that are normally reabsorbed in the proximal tubule.

7. Distal renal tubular acidosis:a. It usually manifests with failure to thrive. Patients have a metabolic acidosis with an

inability to acidify the urine appropriately. Autosomal recessive and autosomal dominant forms. Rickets is variable, and responds to alkali therapy

HYPERVITAMINOSIS D

Hypervitaminosis D is secondary to excessive intake of vitamin D. It may occur with long-term high intake or with a substantial, acute ingestion. Most cases are secondary to misuse of prescribed or over-the-counter vitamin D supplements, but other cases have been secondary to accidental overfortification of milk, contamination of table sugar, and inadvertent use of vitamin D supplements as cooking oil. The recommended upper limits for long-term vitamin D intake are 1,000 IU for children younger than 1 year old and 2,000 IU for older children and adults.Clinical manifestations:

1) Hypotonia2) Anorexia3) Irritability4) Constipation5) Poydipsia6) Polyuria7) Pallor8) Hypercalcemia and hypercaliuria9) Metatstatic calcification

10) Aortic stenosis11) Osteopetrosis of bones12) Treatmnt: aluminium hyderoxide by mouth

ZINC

Functions: Component of many enzymes and gene transcription factors; plays critical roles in nucleic acid metabolism, protein synthesis, and gene expression; supports membrane structure and function.

Dietary sources: Human milk, meats, shellfish, legumes, nuts, and whole-grain cereals.Causes of Deficiency:

1. Diets low in available zinc (high phytate), unfortified synthetic diets; 2. malabsorptive diseases (enteritis, celiac disease, cystic fibrosis); 3. excessive losses (chronic diarrhea); 4. Inborn errors of zinc metabolism (acrodermatitis enteropathica, mammary gland zinc

secretion defect). 5. Inadequate intake in breast-fed infants after age 6 mos. 6. Prematurity and low birth weight is risk factors.

Clinical Features1. Mild: impaired growth, poor appetite, and impaired immunity.2. Moderate–severe: mood changes, irritability, lethargy, impaired immune function, increased

susceptibility to infection; acro-orificial skin rash, diarrhea, alopecia.3. Response to zinc supplement is gold standard for diagnosis of deficiency; plasma zinc levels

are lowered by acute phase response.Treatment:

1. 1 mg/kg/d of elemental zinc for 3 mo (eg, 4.5 mg/kg/day of zinc sulfate salt), given separately from meals and iron supplements.

2. With acrodermatitis enteropathica, 30–50 mg Zn2+ per day (or more) sustains remission.3. WHO recommendation for Diarrhea:

a. Less than 6 mo: 10 mg OD for 14 daysb. More than 6 mo: 20 mg OD for 14 days