38658355 Pedia Abnormal

8/8/2019 38658355 Pedia Abnormal

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CONGENITAL HEART DISEASE

1.Acyanotic diseasesPulmonary circulation and systemic circulation are not connected

If there is a connection, the pressure is higher in the left side than in theright sidePATENT DUCTUS ARTERIOSUS

Most common congenital heart defectSymptoms depend on size of the vessel and age of the childMay have no symptoms; indication may be a murmurIncreasing dyspnea, full bounding pulse, wide pulse pressureSpontaneous closure after infancy rarely occursWithout treatment life expectancy shortATRIAL SEPTAL DEFECTS

10% of all forms of congenital heart diseaseAllows oxygenated blood returning from the lungs to pass into the rightatriumVENTRICULAR SEPTAL DEFECT

constitute 20% of all forms of CHDallows systemic venous and oxygenated arterial blood to mixmay produce no symptoms at allrequire no specific treatment and often close spontaneouslyCOARCTATION OF THE AORTA

narrowing of the lumen of the aortamay be an isolated defect or associated with other cardiac malformationAssessment: measure BP in both arms and a leg and to assess thepulsein both upper and lower extremitiessurgical repair only permanent treatment; usually deferred until 3 yearsof agePULMONIC VALVE STENOSIS

usually do not produce symptoms; typical murmur

AORTIC VALVE STENOSIS


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mild to moderate asymptomatic; typical murmurCYANOTIC DISEASESTETRALOGY OF FALLOT

1.VSD2.

Overriding of the aorta3.Pulmonary valve stenosis4.Enlarged right ventricular wallAssessment:

Primary sign cyanosisHypoxic spells usually initiated by crying

Faintingdue to cerebral hypoxia

Stunted growth, clubbed fingers and toesSquatting position characteristic position to relieve dyspneaImplementation


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Decrease hypoxic spells do not permit child to cryPlace in knee chest positionO2 as needed

TRANSPOSITION OF THE GREAT VESSELS

aorta arises from the right ventricledegree of cyanosis depends on the abnormal connectionsAssessment:

cyanotic at birthdevelop polycythemia

risk for emboli and thrombosesmay develop CHF or pulmonary vascular obstructiveImplementation:

require emergency medical treatmentcardiac catheterizationballool atrial septostomyTRICUSPID ATRESIA

condition in which tricuspid valve did not formno opening between the right atrium and right ventricle2% of congenital heart defectsprofound cyanosis and dyspnea at birthemergency catheterization with balloon atrial septotomyTOTAL ANOMALOUS VENOUS RETURN

condition in which all the pulmonary venous blood returns to the heartcyanosis and severe respiratory distressemergency surgical interventionTRUNCUS ARTERIOSUS

single vessel arising from the ventricles just above a large VSDretarded growth; enlarged liver and heart

usually infants die within the first yearCARDIOVASCULAR DEFECTS


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Monitor vital signs closelyMonitor respiratory statusAuscultate breath sounds for crackles, ronchi or rales

if respiratory effort is increased, place child in reverse Trendelenburgpositionadminister humidified oxygenMonitor for hypercyanotic spells:1.Place infant in a knee chest position2.Administer 100% oxygen by mask3.Administer morphine as ordered

4.IVF as prescribedAssess for signs of CHFAssess peripheral pulsesKeep child stress free as possible; allow maximal restCARDIAC SURGERYPostoperatively:

monitor for signs of discomfort

monitor for signs of sepsis (fever, lethargy, diaphoresis, altered LOC)


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Monitor lines, tubes or catheters ; remove promptlyadminister pain medications; note effectivenessencourage rest periods

facilitate parent-child contact as soon as possibleHome Careomit activities in which child could fall for 2-4 weeks

avoid crowds for 2 weeks after dischargeno added salt dietdo not put creams, lotions or powders on the incision sitechild may return to school 3rd week after dischargeno physical education for 2 monthsfollow up after 2 weeksavoid immunizations, invasive procedure and dental visits for 2 monthsadvise parents regarding importance of dental visit every 6 monthsinform dentist of cardiac problem

instruct parents to call MD if with coughing, tachypnea, cyanosis,diarrheaCONGESTIVE HEART FAILURE

inability of the heart to pump sufficiently to meet the metabolic demandsof the bodyinfants most commonly caused by congenital heart defectscombination of both left sided and right sided heart failuregoal of treatment: to improve cardiac function, remove accumulated fluidand sodium, decrease cardiac demands, improve tissue oxygenationAssessment:

tachycardiatachypneaprofuse scalp sweating especially in infantsfatigue and irritability

sudden weight gainrespiratory distress


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Implementation:monitor vital signs closely and for early signs of CHFmonitor for respiratory distressmonitor I and O; weigh diapers

Monitor daily weight to assess for fluid retention; weight gain of 0.5 kg( 1 lb /day)monitor for facial or peripheral edema , auscultate lung soundselevate HOBmaintain neutral thermal environment to prevent cold stress in infantsadminister cool, humidified oxygen

organize nursing activities to allow uninterrupted sleepmaintain adequate nutritional statusprovide rest; decrease environmental stimulifeed when hungry and soon after awakeninginfant should be well rested before feedingprovide small, frequent feedingsadminister sedation

administer digoxincheck with physician parameters for witholding digoxinNote that infants rarely receive more than 1 mL (50 ug or 0.05 mg) ofdigoxinAdminister diuretics; monitor for hypokalemiaadminister potassium supplements


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monitor serum electrolytesrestrict fluid in acute stagescheck with physician regarding sodium restriction; infant formulas have

slightlymore sodium than does breast milkHome care Instructions for Administering Digoxin

administer 1 hr before or 2 hrs after feedingdo not mix medication with food or drinkif the child vomits, do not administer a second doseif more than 2 consecutive doses have been missed, notify MD; do not

increase or double the dose for missed doseskeep the medication in a locked cabinetif the child becomes ill, notify MDRHEUMATIC FEVER

an inflammatory autoimmune diseaseaffects connective tissue of the heart, joints, subcutaneous tissues andblood vessels of the CNS

most serious complication RHD affecting the cardiac valvespresents 2-6 weeks following an untreated Group A beta hemolytic strepJones criteriaAsssessment:

signs of carditis: SOB, edema of the face, abdomen or ankles, precordialpainsigns of polyarthritis: edema, inflammation of the large joints, joint painerythema marginatum: macular rash on trunk and extremitiessubcutaneous nodulesfeverelevated ASOelevated ESRelevated CRPImplementation:

Assess vital signs


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Control joint pain and inflammation with massageFebrile phase provide bed restlimit physical exercise in child with carditis

Administer antibiotics (penicillin) as prescribedadminister salicylates and anti-inflammatory agentsInstruct parents about the importance of follow up and need forantibiotic prophylaxis for dental work, infection, invasive proceduresadvise child to inform the parents if anyone in school develops astrep throat infectionKAWASAKI DISEASE

known as mucocutaneous lymph node syndromeacute systemic inflammatory illnessunknown causecardiac involvement most serious complicationAssessment:

feverconjunctival injection

red throatSwollen hands, rash, enlargement of the cervical lymph nodes


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Implementation:

monitor temperature frequentlyassess heart sounds and rhythm

assess extremities for edema, redness, desquamationmonitor mucus membrane for inflammationweigh dailyadminister IV immune globulininstruct parents in the administration of prescribed medsaspirin need to monitor bleedingGASTROINTESTINAL SYSTEM

Physiology of DIGESTIVE SYSTEM

Mechanical functions of digestion immature at birthNo voluntary control over swallowing until 6 weeksStomach capacity decreasedRelaxed sphincter contributes to tendency to regurgitate

Liver functions immature throughout infancyGastric acidity low in infants

rises until age 10Digestive processes are mature by toddlerhoodAssessment:

Presenting problema.Vomitingb.Abnormal bowel habits: diarrhea, constipation, bleedingc. Weight loss, failure to thrived.PainNutritional historyPhysical Examination:

General appearance:a.Height and weightb.Measure mid arm circumferencec. Observe color: jaundice

Mouth: dentition


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Abdomena.Skin integrityb.Abdominal distension; visible peristaltic wavesc. Inspect for herniasd.

Auscultate bowel sounds ( every 10-30 seconds)e.Palpate for tendernessf. Liver span (inferior edge palpated 1-2 cm below RCM)g.Spleen (felt on inspiration 1-2 cm below left costal margin)DISORDERS OF THE TEETH

DENTAL CARIES

erosion of the enamel and dentine of teethresults from combination of fermenting sugars and starchedand acid forming organismsPrevention:


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dental hygieneregular dental hygienegood nutrition

fluoridationCLEFT LIP AND CLEFT PALATE

1 in 1000 birthsmultifactorialcommon among children with chromosomal abnormalities

results from failure of the embryonic structures of the face to unitemay occur separately or may combine to produce a single unilateralor bilateral cleft from the lip through the soft palateinterfere with the childs capacity to meet oxygenation andnutritional needsmay seriously hamper normal bonding process of children with parentsAssessment:

facial abnormality visible at birth

-cleft lip or palate or both, unilateral or bilateraldifficulty suckinginability to form airtight seal around nippleformula/milk escapes through nose in infants with cleft palatepredispose to infection communication between mouth and nosedifficulty swallowingabdominal distension air swallowingManagement:

team approachotolaryngologist, audiologist, pediatrician, dentist, orthodontist, speechtherapist

Surgical correction:early correction prevent speech defects

Cheiloplasty


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correction of cleft lipunite edges to allow lips to be both functional; aesthetic reasonsperformed usually at age 2 monthsCleft palate repair

not done until age 18 months anticipation for speech developmentchild should be weaned and able to take liquids from a cup beforepalate repairNURSING INTERVENTION:

Cleft Lip (PreOP)


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feed in upright positionburp frequentlypress cleft lip together with fingers to encourage sucking and to

strengthen musclesif unable to suck, use a rubber tipped syringe; drip onto sideof mouthfinish feeding with water to wash away formula in palate areaprovide small, frequent feedingsprovide emotional support for parentsPost OP: Cleft Lip repair

maintain patent airwaymonitor amount of swallowing to detect hemorrhagedo not place in prone position or with pressure on cheeksavoid any tension on suture lineavoiding straining on suture lineprevent crying

keep child comfortable and contenteduse elbow restraintkeep suture line clean clean after each feeding with saline,peroxide or water to remove crustspain reliefNursing Intervention: Preop cleft palate repair

prepare parents to care fro child after surgeryinstruct concerning feeding methods and positioningPost Op cleft palate repair

position on side for drainage of bloodhave suction availableprevent injury or trauma on suture line1.Use cups only for liquids; no bottles2.

avoid straws, utensils, popsicle sticks, chewing gum3.provide soft toys


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4.Use elbow and wrist restraints5.Provide liquid diet initially soft normal diet6.

Give water after each feeding to clean suture line7.Hold and cuddleESOPHAGEAL ATRESIA and TRAACHEOESOPHAGEAL FISTULA

Esophageal atresia

congenital defect; upper segment of the esophagus ends in a blindpouchTEF

defect in which embryonic structures fail to divide into a separateesophagus and tracheaopening between the two structures

usually occur together


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Assessment:

copious oral and nasal secretions first sign of a defectchoke or cough

when suctioning or gavage is attempted catheter cannot passinto stomachMedical and Surgical intervention

prevention of aspirationdrainage tube may be placed in the blind pouch to suction secretionsesophageal atresia medical emergency-end to end anastomosesfeeding tube maybe inserted into the stomach through gastrostomy untilrepair healsNursing intervention:

provide nutrition1.Provide gastrostomy tube feeding until anastomosis site has healed2.start oral feedings when infant can swallow wellpromote respiratory function

1.position properlyPYLORIC STENOSIS

narrowing of the outlet of the stomachcaused by excessive growth of circular muscles that surrounds thepylorushypertrophy develops over 4-6 weeks of life when symptoms begin toappearmore common in Caucasian; first born, full term boysAssessment:olive size bulge under right rib cage

vomiting projectile; non bilousperistaltic waves during and after feedingfailure to thrive

dehydrationdiagnostic tests:


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a.UGIS narrowing of diameter of pylorusb.Decreased serum Na, K, Clc. Increased Hctd. Metabolic alkalosisNursing intervention: Preop

replace fluids and electrolytesprevent vomiting1.Give thickened feedings2.High Fowlers3.Place on right side after feeding4.Minimize handling

5.Strict I and O, daily weights, urine sp gravityNursing Intervention: post Op

Advance diet as toleratedPlace on right side after feeding


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Observe incision for signs of infectionProvide client teaching and discharge planningINTUSSUSCEPTION

telescoping of bowel into itselfileocecal regionedema, necrosis of bowel, obstructionmost common at age 6 monthsmore in boys than in girlsassociated with cystic fibrosis

Assessment:

piercing crysevere abdominal pain (pulls leg up)vomiting of bile stained fluidbloody mucus in stoolcurrant jelly stoolManagement:

1.barium enema2.surgeryNursing Intervention:

1.provide routine pre and post op care2.monitor for peritonitisHIRSCHSPRUNGS DISEASE

absence of autonomic parasympathetic ganglion cells in large intestinesresults in decreased motility in that portion of the colon and signs offunctional obstructionAssessment:

failure or delay in passing meconiumabdominal distension

failure to pass stooltemporary relief following rectal exam


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loose stools only liquid can pass thruribbon like stoolsnausea, anorexia, fecal vomiting

weight loss, failure to growvolvulus bowel twists upon itselfdiagnostic tests: rectal biopsy confirms presence of aganglionic cellsNursing intervention:

enema as orderedA.Mineral oil or isotonic saline

B.Do not use water or soap suds water intoxicationC.Use volume appropriate to weight of childinfants 150-200mlchildren 250-500 mladminister TPN as ordered


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Provide low residue dietprovide client teaching and discharge teachingColostomy care

Low residue dietIMPERFORATE ANUS

during embryonic life membrane that separates the rectum and anus isabsorbedabsorption fails to occur -> imperforate anusanus appear as a dimple

fistula may be presentrectovaginal, rectourethralAssessment:

inspection of anus, check rectum for patencyinsert rectal thermometerabsence of stool during 1st 24 hoursdevelopment of a distended abdomenIntervention:

surgeryanoplasty if possibletemporary colostomyif with distension nasogastric suctionNursing intervention:

keep operative site cleanobserve infant for signs of hypovolemic shock and infectionif anoplasty heals regular dilatation of anus to prevent stricturecolostomy carea.Kept cleanb. zinc oxideafter surgery side lying or prone with hips elevatedCELIAC DISEASE

malabsorption syndrome characterized by intolerance of gluten,

found in rye, oats, wheat and barleyfamilial disease


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more common in Caucasianscause unknownflat mucosal surface and atrophy of villi of the intestine

reduced absorptive surface -> marked malabsorption of fatsAssessment:

steatorrheachronic diarrheafailure to thrivedistended abdomen

abdominal pain, irritability, listlessness, vomitingsymptoms of ADEK deficiencydiagnostic tests:


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1. pancreatic enzymes2. jejunal or duodenal biopsiesManagement:

diet therapygluten free dietTPN in childrenNursing Intervention:

gluten free diet (BROW Barley, Rye, Oatmeal, Wheat)supplemental fat soluble vitamins

client teaching1. gluten free diet2. importance of reading the label3. avoidance of infection4. adhering to diet even if symptoms are controlled5. importance of long term follow upAPPENDICITIS

inflammation of the appendixischemia, gangrene, rupture, peritonitis if untreatedschool age children most commondue to mechanical obstruction or anatomic defectsAssessment:

diffuse pain RLQnausea, vomitingguarding of abdomenrebound tendernessdecreased bowel soundsfeverdiagnostic tests:

1. CBC2. elevated acetone in urineNursing Intervention:


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antibiotics/ antipyretics as orderedno enemasno heating pads

routine preop carepost op care:1. semi-Fowlers position2. Monitor NGT3. Monitor penrose drainsPARASITISM

roundworms, pinwormsAssessment:

pinworms anal itching, disturbed sleeproundworms colic , abdominal pain, lack of appetite, weight loss


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Nursing intervention:

obtain stool culture

observe all excreta for worms

Scoth tape swabinstruct parents to change clothing, bed linens, towels and launder in hotwaterinstruct all family members to scrub hands and fingernails prior to eatingand after using toiletDIABETES MELLITUS

most common endocrine disease of childrenonset maybe any agetype 1 IDDMrisk of complication is high retinopathy, neuropathy, nephropathy,skin changes, predisposition to infectionAssessment:

rapid onset

polyuria, polydipsia, polyphagia, fatigueweight lossketoacidosis-Dry flushed skinManagement:

insulindiet therapyexerciseprevention of complicationsNursing intervention:

administer insulin as orderedforce fluids without sugarmonitor blood glucose levels daily

observe for hypoglycemia: behavior changes, sweating


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provide client teachinga.Daily regimen for home careb.Urine and blood glucose monitoringc. Nutrition managementd.

Effects of infection and exercise on CHO metabolisme.Prevention of acute and chronic complicationsRESPIRATORY SYSTEM

EPIGLOTTITIS

inflammation of the epiglottisH. influenzae Type b ; Strep pneumoniae

age 2-5abrupt onset; winterconsidered an emergency situationAssessment:fever


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sore, red and inflamed throatdrooling, difficulty of swallowinginspiratory stridor

muffled voicenasal flaringImplementation

maintain patent airwayassess respiratory status and breath soundsuse of accessory muscles; presence of stridorassess temperaturedo not attempt to visualize the posterior pharynx or obtain throat culturelateral neck filmdo not force child to lie downdo not restrainadminister antibiotics; IV fluids

cool mist oxygenhigh humidificationhave resuscitation equipment availableimmunizationLARYNGOTRACHEOBRONCHITIS (LTB)

inflammation of the larynx, trachea, bronchimost common type of croup; maybe viral or bacterialparainfluenza virusgradual onset ; preceded by URIAssessment:

feverirritability and restlessnesshoarse voice

inspiratory stridor and suprasternal retractions


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crackles and wheezingcyanosisImplementation:

patent airway

vital signselevate HOBhumidified oxygen; IVFnebulized epinephrineresuscitation equipments availableBRONCHITIS

infection of the major bronchiassessment: cough, worse at night; becomes productive in 2-3 daysImplementation:1.Monitor for respiratory distress2.Increased fluid intake3.AcetaminophenBRONCHIOLITIS/RESPIRATORY SYNCYTIAL VIRUS (RSV)

Inflammation of the bronchioles; production of mucus that occludesbronchiole tubes and small bronchiRSV highly communicable, usually transferred by hands


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especially at nightAssessment:

episodes of wheezing, breathlessness, dyspnea, chest tightnessSOB, cough, wheezing

child speaks in short, broken phrasesretractionsexercise induced bronchospasmsevere spasm or obstructionImplementation

continuously monitor respiratory statusadminister quick-relief (rescue) medicationinitiate an IV line; prepare to correct dehydration, acidosis or electrolyteimbalanceMedications

1.Quick reliefTo treat symptoms and exacerbationsShort acting b2 agonists

Anticholinergics for relief of acute bronchospasm (ipratropium bromide)


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2.Long term controlCorticosteroidsLong acting b2 agonist

Long acting bronchodilator3.Nebulizer, MDIUsed to deliver many medicationsNon-CFC (chlorofluorocarbon) albuterol4.Chest physiotherapyIncludes breathing exercises

Home care measures:

instruct in measures to eliminate allergensavoid extremes of environmental temperatureavoid exposure to individuals with a viral infectioninstruct the child in how to recognize early symptomsinstruct the child in the cleaning of devices used for inhaled medications

keep immunization up to dateOTITIS MEDIA

Infection of the middle ear occuring as a result of a blocked eustachiantube, preventing normal drainagecommon complication of ARIinfants and children more prone ET shorter, wider, straighterAssessment:

feverirritability and restlessnessrolling of head from side to sidepulling or rubbing the earearache; signs of hearing losspurulent ear discharge

otoscopic examIntervention


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Encourage fluidsupright position when feedingavoid chewing increases pain

have the child lie with the affected ear downinstruct on appropriate technique to clean drainage from the ear withsterile cotton swabsadminister analgesics and antibiotics (10-14 days)screening for hearing lossotic medications1.If younger than age 3 auditory canal is straightened by pulling the pinna

down and back2.If older than 3 years pull pinna up and backMyringotomy

Insertion of tympanoplasty tubes into the middle ear to equalize pressureand keep ear aeratedKeep ears dryEarplugs should be worn during bathing, shampooing, swimmingTONSILLECTOMY AND ADENOIDECTOMY

Assessment:


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persistent or recurrent sore throatenlarged bright red tonsils ; with exudates at timesdysphagia

mouth breathing; unpleasant mouth odorfever; coughPreop:

assess for presence of infectionassess bleeding and clotting studiesassess for loose teethprepare the child for a sore throatPostoperatively:

Position prone or side lying to facilitate drainagehave suction equipment availablemonitor for signs of hemorrhagemay give apply ice collar

discourage coughing or clearing the throatprovide clear, cool, noncitrus and non carbonated drinksavoid milk products intially will coat the throatavoid red liquidsdo not give child any straw, forks, or sharp objectsadminister paracetamol as orderedinstruct parents to notify MD if bleeding, persistent earache or feveroccursinstruct parents to keep child away from crowds until healing occurredHEMATOLOGIC SYSTEM

LEUKEMIA the most frequent type of childhood cancerBrain tumors 2nd

Etiology:

1. Environmental2.

Viruses3.Familial/genetic


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4.Host factorsSTAGES OF TREATMENT

1.INDUCTION

Goal: to remove bulk of tumorMethods: surgery, radiotherapy, chemotherapy, BM transplantEffects: often the most intensive phaseSide effects are potentially life threatening2.CONSOLIDATIONGoal: to eliminated any remaining malignant cells

Methods: chemo/radioSide effects will still be evident3.MAINTENANCEGoal: to keep the child disease freeChemotherapy


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This phase may last several years4.OBSERVATIONGoal: to monitor the child at intervals for evidence of recurrent

disease and complications of treatmentMethod: treatment is completeChild may continue in this stage indefinitely5.LATE EFFECTS OF TREATMENTImpaired growth and developmentCNS damage

Impaired pubertal developmentDevelopment of secondary malignancyPsychologic problems related to living with a life threatening disease andcomplex treatment regimen

NURSING INTERVENTION

1.Help child cope with intrusive procedures

Provide information geared to developmental level and emotionalreadinessExplain what is going to happen, why it is necessary, how it will feelAllow child to handle to handle and manipulate equipmentsAllow child some control in situations-positioning, selecting injection site2.Support child and parentsMaintain frequent clinical conferences to keep all informedAlways tell the truthAcknowledge feelings and encourage child/family to express themProvide contact with another parent or support group3.Minimize side effects of treatmenta.Skin breakdown

Keep clean and dry; wash with warm water; no soaps or creamsDo not wash off radiation marks


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Avoid all topical agents with alcoholDo not use heating pads or hot water bottleb.Bone marrow suppression

Provide frequent rest periodsAvoid crowdsEvaluate any potential site of infectionMonitor temperatureAvoid use of aspirinSelect activities that are physically safec.

Nausea and vomitingAdminister antiemetic at least half an hour before chemotherapyEat light meal prior to administration of therapyAdminister IVF if needed


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d. AlopeciaReduce trauma of hair lossBuy wig before hair falls out

Discuss various head coveringsAvoid exposing head to sunlight4. Nutrition deficitsEstablish baselineProvide high calorie, high protein5. Developmental delayFacilitate return to school as soon as possible

Discuss limit setting, disciplineLEUKEMIA

most common form of childhood cancerpeak 3-5 yearsproliferation of abnormal wbc that do not mature beyond the blast phaseblast cells infiltrate other organs liver, spleen, lymph tissueTypes:

1. Acute Lymphocytic leukemia (ALL)80-85%Acute75% chance of surviving2. Acute nonlymphocytic leukemiaIncludes granulocytic and monocytic types60-80% will obtain remission30-40% cure rateAssessment:1. anemia, weakness, pallor, dyspnea2. Bleeding, petecchiae, spontaneous bleeding, ecchymoses3. Infection, fever, malaise4. Enlarged lymph node5. Enlarged spleen and liver6. Bone painManagement:

1. diagnosis: blood studies, BMA2. Treatment stages

a. Inductionb. CNS prophylaxisc.


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MaintenanceNursing Intervention:

1. Provide care for the child receiving chemo and radiotherapy2. Provide support for the family/child3. Support child during painful proceduresa. Use distraction, guided imagery


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b.Allow the child to retain as much control as possiblec.Administer sedation prior to procedure as orderedSickle Cell Disease

hemoglobin A is partly or completely replaced by abnormal sicklehemoglobin (HgbS)HgbS sensitive to changes in the oxygen content of the red blood cellRisk factors: African AmericanInsufficient oxygen causes the cells to assume a sickle shape and thecells become rigid and clumped together, obstructing capillary blood flowThe sickling response reversible under adequate oxygenationSickle cell crises vaso-occlusive crisis, splenic sequestration, aplasticcrisisAssessment:

1.Vaso-occlusive crisisMost common type of crisisCaused by stasis of blood with clumping of thecells in microcirculation, ischemia and infraction

Fever, pain and tissue engorgement2.Splenic sequestrationPooling of blood in the spleenProfound anemia, hypovolemia, shock3.Aplastic crisisCaused by the diminished production and increased destruction of RBCs,triggered by viral infection or the depletion of folic acidProfound anemia and pallorImplementation:

Administer oxygen and blood transfusionsadminister analgesicsmaintain adequate hydration and blood flow with IV normal saline asprescribed and with oralfluids

Assist the child to assume a comfortable position so that the child keepsthe extremities extendedto bed no more than 30 degrees


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avoid putting strain on painful jointsencourage consumption of a high calorie, high protein diet with folic acidsupplementationadminister antibiotics as prescribed

Monitor for signs of increasing anemia and shock (pallor, vital signchanges)IRON DEFICIENCY ANEMIA

Iron stores are depleted, resulting in a decreased supply of iron for themanufacture for themanufacture of hemoglobin in RBCsResults from blood loss, increased metabolic demands, syndromes of GImalabsorption, dietary

inadequacyAssessment:

pallor


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weakness and fatigueirritabilityImplementation:

Increase the oral intake of ironInstruct the child and parents in food choices that are high in ironAdminister iron supplements as prescribedLiquid iron prep stains stains teeth; with strawside effects of iron therapyAPLASTIC ANEMIA

a deficiency of circulating erythrocytes resulting from the arresteddevelopment of RBCs within the bone marrowcauses: exposure to toxic agents, viruses, infection, autoimmunedisorders, allergic statesdefinitive diagnosis: BMATherapeutic management: blood transfusions, splenectomy,corticosteroids, immunosuppressive therapy, bone marrow transplantationAssessment

PancytopeniaPetecchiae, purpura, bleeding, pallor, weakness, tachycardia andfatigueImplementation:

Blood transfusioncorticosteroids and immunosuppresivessplenectomybone marrow transplantMedic Alert braceletHEMOPHILIA

x linked recessive traitHemophilia A deficiency of Factor VIIIHemophilia B deficiency of factor IX

Males inherit hemophilia from their mothers and females inherit thecarrier status from their


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fathersAssessment:

prolonged bleeding after minor injury1.At birth after cutting cord

2.Following circumcision3.Following IM immunization4.Increase bruising as child learns to crawl and walkabnormal bleeding in response to traumajoint bleeding pain, tenderness, swelling limited range of motiontendency to bruise easilyprolonged PTTnormal BT, PT, platelet countImplementation:

prepare to administer Factor VIII concentrate /cryoprecipitatea.Thaw slowlyb.Gently rotate bottle


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c.Infuse immediately; deteriorates at room temperaturemonitor for bleedingmonitor for joint pain; immobilize the affected extremity if joint pain

occursassess neurological status ( child at risk for IC bleed)monitor urine for hematuriaControl bleeding by immobilization, elevation, application of ice; applypressure (15 mins)for superficial bleedinginstruct parents regarding activities of the child

avoidance of contact sportsBETA THALASSEMIA MAJOR

Cooleys anemiaautosomal recessive disordercharacterized by reduced production of one of the globin chains in thesynthesis of hemoglobinincidence highest in Mediterranean descentAssessment:

severe anemiapallorfailure to thrivehepatosplenomegalymicrocytic, hypochromic RBCsImplementation

Instruct the administration of folic acid (Vit B9)administer blood transfusion as prescribedMonitor for iron overloadiron overload chelation therapy with deferoxaminegenetic counselingIDIOPATHIC THROMBOCYTOPENIC PURPURA

increased destruction of platelets with resultant platelet count less than100,000/mm3


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characterized by petechiae and ecchymoses of skinexact mechanism unknownoften preceded by a viral illness

spleen not enlargeAssessment:

petechiae: spider web appearance of bleeding under the skin due tosmall size of plateletsecchymosisblood in any body secretions, bleeding from mucus membranes,nosebleeds

diagnostic test: platelet count decreases, anemiaManagement:

steroids and immunosuppressivesplatelet transfusionsurgery; splenectomyNursing Intervention


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control bleeding1.Administer platelet transfusion as ordered2.Apply pressure to bleeding sites as needed

3.Position bleeding part above heart level if possiblePrevent bruisingProtect from infectionMeasure normal circumference of extremities for baselineAdminister meds orally, rectally or IV rather than IM; hold pressure on sitefor 5 mins

Avoid aspirinProvide client teaching and discharge planning1.Pad crib and playpen2.Provide soft toys3.Provide protective headgear during toddlerhood4.Use soft toothbrush5.Avoid contact sports

INTEGUMENTARY SYSTEM

ECZEMA

atopic dermatitis - often the first sign of an allergic predisposition in achildusually manifests during infancyAsssessment:

erythema, weeping vesicles that rupture and crustssevere pruritus; scratching causes thickening and darkeningdry skin, sometimes urticariaIntervention:

topical steroidsantihistaminescoal tar preparation

colloid baths


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diet therapy: elimination of offending foodNursing Intervention:

avoid heat and prevent sweatingcheck materials in contact with childs skin (sheets, lotions, soap)

avoid frequent bathsavoid use of soapprovide lubricant immediately after bathadminister topical steroids as ordereduse cotton instead of woolkeep childs nails short; use elbow or glove restraints if neededapply wet saline or Burrows solution compressesDIAPER RASH

contact dermatitisplastic/rubber pants and linings of disposable diapersexacerbate the condition by prolonging contact with


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moist, warm environment

skin further irritated by acidic urineAssessment:

erythema/excoriation in the perineal areairritabilityNursing intervention:

keep area clean and dry; clean with mild soap and water after each stooland soon as child urinatestake off diaper and expose area to air during the day

client teaching:1.Proper hygiene2.Avoid use of plastic pants or disposable diapers with a plastic lining3.Avoid commercially prepared diaper wipes containing alcohols4.Avoid cornstarch; good medium for bacteriaMUSCULOSKELETAL SYSTEM

Care of the child with a Cast

if cast is of plaster will remain wet for at least 24 hrsuse only the flats of their hands to move childrencasts must remain open to the air until drycasted extremities are elevated to help blood return and reduce swellingInitial chemical hardening reaction may cause a change in an infantsbodyChoose toys too big to fit down castdo not use baby powder near cast medium for bacteriaprepare for anticipated casting by having child help apply cast in a dollDiagnoses and interventions:

1.Potential for alteration in tissue perfusion related to constriction of castCheck color, sensation and motion distal to the cast every half hourCheck pedal or radial pulse

Check for tightness by slipping finger under edge; if impossible cast istoo tight


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Ask child to move toes or fingersElevate casted extremity2.Potential for alteration in skin integrity

Remove plaster flakes from skinHandle wet cast carefully so as not to cause indentationsExpose wet cast to air to hasten dryingSupport heavy cast with sling or pillow to decrease pressure of cast edgesCheck cast for foul or musty odors3.Potential for fear and loneliness

Encourage expression of feelingsProvide diversional playEncourage friends and family to visit children as oftenProvide educational opportunity for children confined for long periods


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4.Potential for knowledge deficit of familyEncourage discussion of feelings and fearsProvide information and reassurance as appropriate

Involve family in childs care in hospitalPrepare family for some emotional regressionCONGENITAL HIP DISLOCATION

displacement of the head of the femur from the acetabulumpresent at birth although not always diagnosedfamilial disorderunknown cause; may be fetal position in uteroacetabulum is shallow and the head of femur is cartilaginous at birthAssessment:

maybe unilateral or bilaterallimitation of abduction (cannot spread legs to change diaper)Ortolanis clicka.

With an infant supine, bend knees and place thumb on bent knees,fingers at hip jointb.Bring femur 90degrees to hip, then abductc.Palpable click dislocationBarlows testa.With infant on back, bend kneesb.Affected knee will be lower because the head of the femur dislocatestowards the bed of gravityadditional skin folds with knees bentwhen lying on abdomen, buttocks of affected side will be flatterTrendelenburg test if child can walka.Have child stand on affected leg onlyb.Pelvis will dip on normal side as child attempts to stay erectManagement:

Goal : to enlarge and deepen the socket


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Early treatment: positioning the hip in abduction with the head of thefemur in the acetabulum and maintaining it in position for several monthsTraction and casting (hip spica)SurgeryNursing intervention:

Maintain proper positioning: keep legs abducted1.Use triple diapering2.Use Frejka pillow splint (jumperlike suit to keep legs abducted)3.Place infant on abdomen with legs in frog position4.Use immobilization devices

Provide adequate nutritionProvide sensory stimulationClient teaching and discharge planning:CLUBFOOT (Talipes)

abnormal rotation of foot at ankleVarus inward rotation; bottom of feet face each otherValgus outward rotationCalcaneous upward rotation; would walk on heelsEquinas downward rotation; would walk on toes


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Most common talipes equinovarus

Assessment:

foot cannot be manipulated by passive exercises into correct position

Management:

exercisescastingDenis Browne splint (bar shoe)surgery and castingNursing Intervention:

perform exercises as orderedprovide cast carechild who is learning to walk must be prevented from trying to stand;apply restraints if necessaryprovide diversional activitiesprovide skin careclient teaching

SCOLIOSIS

lateral curvature of the spinemost commonly in adolescent girlsfamilial pattern; associated with other nueromuscular conditionidiopathic majorityAssessment:

failure of curve to straighten when child bends forward with kneesstraight and arms hanging down feetuneven bra strap marksuneven hipsuneven shouldersasymmetry of rib cagexray: reveals curvature

Management:


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stretching exercisesMilwaukee brace worn 23 hours/day for 3 yearsplaster jacket vestspinal fusion

Nursing Intervention:

teach/encourage exerciseprovide care for the child with Milwaukee bracea.Child wears brace 23 hours/dayb.Monitor pressure pointsc.

Promote positive body image with braceProvide cast careAssist with modifying clothing for immobilization devicesAdjust diet with decreased activityProvide client teaching and discharge instructionsa.Exerciseb.Cast care

c.Correct body mechanicsd.Alternative education for long term hospitalizatione.Availability of community agencies


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JUVENILE RHEUMATOID ARTHRITIS

systemic, chronic disorder of connective tissueautoimmune reaction

results from eventual joint destructionaffected by stress, climate and geneticsTypes:

1.Monoarticular JRAFewer than 4 joints involved (usually legs)AsymmetricGood prognosisMild signs of arthritis2.Polyarticular JRAMultiple joints affectedSymmetricalInvolvement of TMJ

Remissions and exacerbationspoor prognosis3.Systemic disease with polyarthritis (Stills disease)Fever, rash, LADP, anorexia, weight lossExacerbations and remissionsAssessment:

No specific diagnostic testsESR, ASO, RF- not specificIntervention

Drugs: ASA, corticosteroidsNSAIDSPhysical therapy strengthening muscles, preventing deformitiesSplints used for knees, wrists and hands to reduce pain and prevent

or reduce flexion deformitiesNursing intervention:


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infection

UNDESCENDED TESTES (Cryptorchidism)

unilateral or bilateral absence of testes in scrotal sac

testes normally descend at 8 months of gestationincreased incidence in those with genetic disorder75% will descend spontaneously by age 1 yearAssessment:

unable to palpate testes in scrotal sacManagement:

if testes remains in abdomen, damage to testes is possible because ofincreasedchorionic gonadotropinorchipexy: surgical procedure to retrieve and secure testes placement;performed between ages 1-3 yrs

Nursing Management:

provide treatment options

support parents if surgery will be donepost op: avoid disturbing the tension mechanismavoid contamination of incisionHYPOSPADIAS

urethral opening located anywhere along the ventral surface of penisAssessment:

urinary meatus misplacedinability to make straight stream of urineManagement:

minimal defects no interventionneonatal circumcision delayed, tissue maybe needed for corrective repairsurgery at age 3-9 monthsNursing Intervention:

diaper normally


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provide support for parentspost op: check pressure pressure dressingmonitor catheter drainagePHIMOSIS

an abnormal narrowing of the foreskin so that it cannot be retracted overthe glans penismay be present at birth or may develop as a result of poor hygiene withaccumulation of smegmaManagement:

Prevention regular pulling the foreskin back and cleaning

circumcisionNursing intervention:

circumcision care:1.Close observation for bleeding


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2.Ice application3.Administration of analgesicsENURESIS

involuntary passage of urine after the age of control is expected (4 years)types:1.Primary in children who have never achieved control2.Secondary: in children who have developed completecontrolMay occur anytime but most frequent at night

More common in boysNo organic causeEtiologic possibilities:1.Sleep disturbances2.Delayed neurologic development3.Immature development of bladder4.Psychologic problems

Assessment:

PE normalHistory of repeated voluntary urination management:bladder retention exercisesdrug therapy1.TCA imipramine2.AnticholinergicsNursing Intervention:

Provide information/counseling to family:1.Confirm that this not conscious behavior and that the child is notpurposely misbehaving2.Assure parents that they are not responsible and that this is a relativelycommon problem

Involve child in care; give praises and support with small accomplishments1.Age 5-6 years can strip wet beddings


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2.Age 10-12 years can do laundry and change bedAvoid scolding and belittling the childEXSTROPHY OF THE BLADDER

congenital malformation ; nonfusion of abdominaland anterior walls of the bladder during embryologicdevelopmentanterior surface of bladder lie open on abdominal wallAssessment:

Associated structural changes1.Prolapsed rectum2.

Inguinal hernia3.Widely split symphysis4.Rotated hipsAssociated anomalies1.Epispadias2.Cleft scrotum or clitoris3.Undescended testes

4.Chordee (downward deflection of the penis)


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Management:

reconstructive surgeryurinary diversion

delayed until 3-6 monthsNursing intervention: Preop:

Provide bladder care; prevent infectiona.Keep area as clean as possibleb.Change diaper frequently; keep loose fittingc.Wash with mild soap and water

d.Cover exposed bladder with vaseline gauzePostop:

Design play activities to foster toddlers need for autonomy-child will be immobilized for extended period of timePrevent trauma; as child gets older and more mobile,trauma is more likelyNEPHROTIC SYNDROME

autoimmune processstructural alteration of glomerular membraneresults in increased permeabilityb to plasma proteinscourse: exacerbations and remissions over a period of months to yearscommonly affects preschoolers; boys>girlsAssessment:

1.Proteinuria2.Hypoproteinemia3.Hyperlipidemia4.Dependent edemaPuffiness around the eyes in morningAscitesScrotal edema

Ankle edema5.


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anorexia, vomiting, diarrhea6.Pallor, lethargy7.HepatomegalyManagement:

drug therapy1.Corticosteroids2.Antibiotics3.Thiazide diureticsNursing Intervention:

Provide bed rest

1.Conserve energy2.Find activities for quiet playProvide high protein, low sodium diet during edema phaseMaintain skin integrityAvoid IM injections meds not absorbed in edematous tissuesObtain morning urine for protein studies

Provide scrotal supportMonitor I and O, vital signs


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Daily weightAdminister as orderedProtect from sources of infection

ACUTE GLOMERULONEPHRITIS

immune complex diseasebeta hemolytic strepMore frequently in boys; 6-7 yearsresolves in 14 daysself limiting

Assessment:

1. History of strep infection (URTI or impetigo)2. Edema, anorexia, lethargy3. Hematuria or dark colored urine4. Fever5. Hypertension6. Diagnostic tests:Urinalysis rbc,wbc,protein, cellular castsUrine specific gravity increased

BUN, crea increasedESR elevatedHgb, Hct decreasedManagement:

antibioticsAntihypertensivesdigitalis if with CHFfluid restrictionperitoneal dialysis if severe renal complication occursNursing Management:

1. Monitor I and O, BP2. Weigh daily3. Provide diversional activity4. Provide client teaching and discharge planningMedication administration

Prevention of infection


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Signs of renal complicationsImportance of long term follow upWILMS TUMOR (NEPHROBLASTOMA)

large, encapsulated tumor that develops in the renal parenchyma,

more frequently in left kidneyoriginates during fetal lifepeak age : 1-3 yearsAssessment:non tender mass, usually midline near liverhypertension

hematuriatest: IVPNursing intervention:1. Do not palpate abdomen to avoid dissemination of CA cells2. Handle child carefully when bathing and giving care


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Surgical Implementation: Hydrocephalus

Goal: to prevent further CSF accumulation by bypassing the blockage anddraining the fluid from the ventricles to a location where it may bereabsorbed

1.

VP Shunt CSF drains into the peritoneal cavity from the lateral ventricle2.AV shunt CSF drains into the right atriumPostOP Care:

1.Keep child flat as prescribed to avoid rapid reduction of intracranial fluid2.Observe increase ICP if present, elevate HOB 15-30 deg3.Monitor for infection4.

Measure HC5.Monitor I and O6.Provide comfort measures; administer medications (diuretics, antibiotics,or anticonvulsants)


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7.Toddler headache and anorexia earliest common signs of shuntmalfunctionSPINA BIFIDA

CNS defect that occurs as a result of neural tube failure toclose during embryonic developmentdefect closure usually done during infancyTypes:

1.Spina bifida occultaPosterior vertebral arches fail to close in the lumbosacral area

Spinal cord intact; not visibleMeninges not exposed on the skin surfaces2.MeningocoeleProtrusion involves meninges and a sac-like cystLumbosacral area3.MyelomeningocoeleProtrusion of meninges, CSF, nerve roots, portion of spinal cord

Sac covered by a thin membrane may rupture or leakNeuro deficit evidenceAssessment:

Depends on spinal cord involvementVisible spinal defectFlaccid paralysis of legsAltered bladder and bowel functionImplementation:

Evaluate sac; measure lesionneuro checkmonitor for increase ICPmeasure HC; assess fontanelles

Protect the sac1.


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Cover with sterile, moist (normal saline) non-adherentdressing2.Change dressing every 2-4 hoursPlace prone position

head is turned to one side for feedingdiapering may be C/I until defect repairedAseptic techniqueWatch for early signs of infectionAdminister antibioticsAdminister anticholinergics improve urinary continence

Administer laxatives , antispasmodicsMENINGITIS

infectious process of the CNS caused by bacteria and virusesacquired as a primary or as a result of complicationsdiagnosis CSF analysis (increase pressure, cloudy CSF, high protein,low glucosebacterial or viralAssessment:

signs and symptoms vary depending of age groupfever, chillsvomiting, diarrheapoor feeding or anorexia


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altered LOCbulging anterior fontanelnuchal rigidity

Implementation:

isolation; maintain for at least 24 hours after antibiotics are initiatedadminister antibiotics as prescribedmonitor VS and neuro statusMonitor I and Oassess nutritional statusdetermine close contacts of the child with meningitisSEIZURE DISORDERS

Sudden transient alterations in brain function resulting from excessivelevels of electrical activity in the brainAssessment:obtain information from parents about the time of onset, precipitatingevents and behavior before and after the seizure

seizure precautions:1.Raise side rails2.Pad side rails3.Place waterproof mattress on bed4.Instruct child to swim with companion5.Alert caregivers to the need for special precautionsEmergency Treatment for Seizures:

Ensure patency of airwaysIf the child is standing or sitting, ease the child down to the floorplace pillow or folded blanket under the childs headloosen restrictive clothingclear area of any hazardsif vomiting occurs, turn child to one side

do not restrain child; do not place anything in the childsmouth


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Remain with the child until fully recoversPrepare to administer medicationsCEREBRAL PALSY

disorder characterized by impaired movement and posture resulting from

an abnormality in the extrapyramidal motor systemspastic type- most commonAssessment:

extreme irritability and cryingfeeding difficultiesstiff and rigid arms and legs

delayed gross developmentabnormal motor performancealterations of muscle toneabnormal posturingpersistence of primitive reflexesImplementattion:

early recognition

PT, OT, speech therapy, eduaction and recreation


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assess the childs developmental level and intelligenceearly interventionencourage communication and interaction with the child on a functional

levelprovide safe environmentposition upright after mealsprovide safe, appropriate toys for age and developmental level


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