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178 Cards in this Set
- Front
- Back
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Top Childhood Cancers - What are they |
1. Leukemia (ALL)2. CNS tumors3. Lymphoma4. Neuroblastoma
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Neuroblastoma -What is itAssociated with?
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Embryonal tumor of neural crest cell originMC cancer in infantsmore than 1/2 kids < 2 y/oassociations - neurofibromatosis Hirschsprung's n-myc oncogene
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Neuroblastoma -Hx/PE
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Can occur anywhereSx vary with locationnontender abdom mass (may cross midline)Horner's syndromeHTNcord compressionanemiaFTTfeversite-specific mets can cause-proptosisperiorbital bruisingsubq tumor nodulesbone pain with pancytopeniaopsoclonus/myoclonus"dancing eyes, dancing feet"
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Neuroblastoma -Dx
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Abdom CT24-hr urinary catecholaminesassess extent of disease -CXRbone scanCBCLFTsBUN/Crcoag panel
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Neuroblastoma -Tx
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■ Excision - localized tumors■ chemo includes - cyclophosphamide doxorubicin■ adjunctive radiation - if tumor spread beyond origin■ prognosis improved for kids with low-risk dis. - < 1 y/o no N-myc amplification localized
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Wilms' Tumor -What is itAssociated with?
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Embryonal cancer of kidneyMC renal tumor in kids2-4 y/oassociated with - family Hx Beckwith-Wiedemann syndrome WAGR neurofibromatosis
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Wilms' Tumor -Hx/PE
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Painless, palpable abdom mass does not cross midlinen/vfeverweight losshematuriaHTN
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Wilms' Tumor -Dx
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Abdom CT or US - intrarenal masscheck for metas -CXRchest CTCBCLFTsBUN/Cr
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Wilms' Tumor -Tx
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■ Transabdom nephrectomy■ postsurgical chemo - vincristine dactinomycin■ flank irradiation (for some)■ prognosis good - depends on staging & histo
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RDS -What is it
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MCC of resp failure in premiessurfactant deficiency => inc. surface tension (poor lung compliance) and alveolar collapse (atelectasis)surfactant made by T2 pneumocytes mainly ~35th weekdipalmitoyl phospha- tidylcholinerisk factors -maternal diabetesmales2nd born of twins
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RDS -Hx/PE
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Presents in 48-72 hrs of lifeRR > 60/minintercostal retractionsexpiratory gruntingnasal flaringcyanosisprogressive hypoxemia
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RDS -Dx
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ABGsCBC (to r/o infection)BC (to r/o infection)CXR - bilat diffuse atelectasis causing ground-glass appearance with visible air bronchogramslecithin:sphingomyelin ratio < 2
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RDS -Tx
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CPAP or intubation and mech ventartificial surfactantto prevent - mom gets corticosteroids monitor fetal lung maturity (L:S ratio)
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RDS -Complications
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Persistent PDAbronchopulmonary dysplasiaretinopathy of prematurityintraventricular hemorrhageNEC
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Intussusception -What is it
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MCC of bowel obstruction in 1st 2 yrs. of lifemales > femalesusu prox to ileocecal valvecause - idiopathicin older kids -mass or intest. abnormality triggers the telescoping:adenovirus or rotavirus parasitesCFceliac disease polypsintestinal lymphomaMeckel's diverticulumHenoch-Schonlein purpura
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Intussusception -Hx/PE
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Hx -colicky abdom pain in apparently healthy kidsn/vtoo young to talk - cry draw knees up to chest dyspnea with painadvanced signs -red "current jelly" stoollethargyfeverPE -abdom tendernesspos. stool guaiacpalpable "sausage-shaped" RUQ abdom mass
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Intussusception -Dx
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Abdom XRabdom USair contrast barium enemaCBC
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Intussusception -Tx
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Correct vol & electrolytescheck CBCair-contrast barium enema - diagnostic and therapeuticsurgical reduction or resection (if gangrenous)
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Pyloric Stenosis -What is it
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Hypertrophy of pyloric sphincter1st-born males more affected
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Pyloric Stenosis -Hx/PE
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1st 2 wks - 4 mos. of lifenonbilious emesis=> projectile emesis after each feedingso, babies feed well init=> malnutrition & dehydrationpalpable olive-shaped, mobile, NT epigastric massvisible gastric peristalsis
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Pyloric Stenosis -Dx
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Abdom US - diagnosticbarium studies - string sign pyloric beakhypochloremic, hypokalemic metabolic alkalosis
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Pyloric Stenosis -Tx
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First -hydrationcorrect acid-base & electrolyte abnormalitiesNG tube - possiblelong. pyloromyotomy
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Child Abuse -What is it
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Neglectphysical abusesexual abuseemotional abusesuspect - if Hx doesn't match physical findings if there was a delay in getting medical care
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Child Abuse -Hx/PE
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Infants may have apnea, seizures, FTTExam findings include -. cutaneous - ecchymoses of varying ages patterned injuries. skeletal - . spiral fractures of femur and humerus in kids < 3 = abuse unless prove else . epiphyseal/metaphyseal injuries - can happen in infants from pulling/twisting limbs . rib injuries < 2 y/o. sexual abuse - STDs or genital trauma
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Child Abuse -Dx
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R/o conditions that mimicskeletal survey & bone scan -can show fractures in various stages of healingif sexual abuse suspected -test for gonorrhea, chlamydia and HIVto r/o shaken baby syn -check for retinal hemorrhagesCT for subdural hemorrhagesMRI for white matter changes
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Child Abuse -Tx
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Document injuriesnotify child protective svcshospitalize if nec.
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Epiglottitis -What is it
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Serious, rapidly progressiveinfection of supraglotticbefore immunization - from H influ type Bnow - Streptococcus nontypable H flu viral agents
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Epiglottitis -Hx/PE
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Sudden-onset high feverdysphagiadroolingmuffled voicesoft stridorcyanosis"sniffing dog" position"tripod" positioninsist on sitting up in beduntreated - life-threatening
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Epiglottitis -Dx
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ClinicalDON'T EXAMINE THROAT unless anesthesiologist presentdefinitive Dx - direct fiberoptic visual of cherry-red, swollen epiglottis & arytenoidslat XR - thumbprint sign
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Epiglottitis -Tx
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Emergencycall anesthesiologisttransfer pt. to ORendotrach intubation or trachIV ABx - ceftriaxone or cefuroxime
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Croup (Laryngotracheobronchitis) -What is it
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Inflammation of larynx and upper airway, mainly subglottic space=> narrowing of airwaykids 3 mos. - 3 yrs.MCC - parainfluenza virus 1also - PIV-2, PIV-3, RSV, rubeola, influenza, adenovirus, Mycoplasma pneumonia
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Croup (Laryngotracheobronchitis) -Hx/PE
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Prodrome - URI Sxs 1-7 daysstridor - worse by agitationfever - low gradehoarsenessbarking cough
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Croup (Laryngotracheobronchitis) -Dx
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ClinicalXR - steeple sign
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Croup (Laryngotracheobronchitis) -Tx
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Mild - cool mistmoderate - oral corticosteroidssevere -(resp. distress at rest)admitnebulized racemic epi
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Bronchiolitis -What is it
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Acute inflammation of smallest airwaysacute viral bronchiolitisMCC - RSVinfants & kids < 2 y/ocan progress to resp. failurerisk for severe RSV - < 6 mos. old premies heart or lung dis. immunodeficiency
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Bronchiolitis -Hx/PE
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Hx -low-grade feverrhinorrheacoughapnea - young infantsPE -tachypneawheezinghyperresonance to percussion
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Bronchiolitis -Dx
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CXR - hyperinflation of lungsinterstitial infiltratesatelectasisELISA of nasal washings for RSV - hi sens & spec
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Bronchiolitis -Tx
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Mild -outpt.fluids, nebulizers, O2 if ndadmit if -marked resp distressO2 saturation < 95%toxic appearancedehydration/poor oral feedingpremie (< 34 wks)< 3 mos. oldunderlying cardiopulmon dis.unreliable parentsinpatients -contact isolationhydrationO2ribavirinRSV prophylaxis -RespiGam or Synagishigh-risk pts. in winter
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Otitis Media -What is itRisk Factors
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Middle ear infectionMCC - #1 - S. pneumoniae #2 - H. flu #3 - Moraxella catarrhaliskids predisposed - eusta tuberisk factors - viral URIs trisomy 21 CF immunodeficiency smoke exposure day-care attendance bottle feeding cleft palate prior otitis media
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Otitis Media -Hx/PE
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Feverear tugginghearing lossirritabilityErythemabulgingdecreased mobility of tym membloss of light reflex and bony landmarkstym memb may be perforated
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Otitis Media -Dx
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Clinical
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Otitis Media -Tx
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Amoxicillin - 10 daysTx failure after 3 days - switch to amoxicillin-clavulanic acid, ceftriaxone or cefuroxime
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Otitis Media -Complications
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Mastoiditismeningitishearing losscholesteatomatympanosclerosischronic suppurative OM
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Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) -What is it
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Multisystem acute vasculitisusually kids < 5 y/oesp. Asianat risk for coronary artery aneurysms => MI
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Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) -Hx/PE
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"CRASH and Burn"Conjunctivitis - b/lRashAdenopathy - cervical lymphStrawberry tongueHands and feet - swollen, red, desquamationfever > 40C for > 5 days
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Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) -Dx
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Clinicalthrombocytosis - wk 2 or 3inc. ESR
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Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) -Tx
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High-dose aspirinIVIG - to prevent aneurysmscorticosteroids are contraindicated (they may inc. aneurysms)
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FTT -What is it
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Persistent weight below 3rd to 5th percentileor falling off growth curveorganic - medical conditionnonorganic - psychosocial nonorganic is MCrisk factors - chronic illness poverty low maternal age chaotic envi genetic dis. (CF) inborn errors of metabolism HIV
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FTT -Hx/PE
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Low weight for age and heightminimal weight gain or weight lossplot on growth chartck for signs of systemic dis.diet Hxobserve caregiver-child interaction
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FTT -Dx
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Calorie countCBCelectrolytesCralbumintotal proteinsweat chloride testUA/UCstool cultureO&Passess bone age
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FTT -Tx
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Tx depends on causesupplement nutrition if breastfeeding inadequateadmit if - neglect severe malnourishment
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Atrial Septal Defect (ASD) -What is it
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Opening in atrial septumlets blood flow bet. atriaL to R shunting due to lower R pressureblood flow to lungs inc.
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Atrial Septal Defect (ASD) -Hx/PE
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Hx -usu. presents in late childhd or early adultonset & severity dep. on sizelarge defect -tire easy (DOE)freq. resp. infectionsFTT=> CHF => cyanosisPE -RV heavewide, fixed split S2systolic ejection murmur - upper left sternal border
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Atrial Septal Defect (ASD) -Dx
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Echo with color flow Doppler - diagnosticECG - rt-axis deviationCXR - cardiomegaly and inc. pul vascular markings
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Atrial Septal Defect (ASD) -Tx
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. Small defects may close spontan (no Tx needed). ABx prophylaxis before dental procedures. surgical closure - infants with CHFpts. > 2:1 pul to sys bld flow correct early to prevent - arrhythmia RV dysfunction Eisenmenger's syn
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Ventric Septal Defect (VSD) -What is it
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MC congenital heart defectMore common in pts. with - Apert's syn Cri-du-chat Trisomies 13 & 18
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Ventric Septal Defect (VSD) -Hx/PE
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Hx -Sxs dep. on degree of shuntingsmall def usu asymp at birthlarge def - CHF freq resp infections FTTPE -pansystolic murmur - lower lt sternal borderloud pulmonic S2in severe defects - systolic thrill cardiomegaly crackles
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Ventric Septal Defect (VSD) -Dx
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Echocardiogram - diagnosticEKG - may show RVH or LVH normal with small VSDs
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Ventric Septal Defect (VSD) -Tx
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. Most small defects closespontan (no Tx needed). ABx prophylaxis before dental or pulmonary procedures. surgical closure -correct early to prevent -Eisenmenger's syn, et al. Tx CHF & resp infections
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Patent Ductus Arteriosus (PDA)What is it
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Failure of DA to close=> L-to-R shunt (aorta to pulmonary artery)risk factors - high altitude (low O2) 1st trimester rubella in mom premies females (more common)
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Patent Ductus Arteriosus (PDA)Hx/PE
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Hx -typically asymp (small PDA)slowed growthrecurrent lwr resp infectionslwr extremity clubbingCHF SxsPE -wide pulse pressurecontinuous machine murmurloud S2bounding periph pulses
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Patent Ductus Arteriosus (PDA)Dx
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Small - often no signs of cardiomegLarge -echocardio - LA & LV enlargedEKG - LVHCXR - cardiomegalycolor-flow doppler- diagnostic
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Patent Ductus Arteriosus (PDA)Tx
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Indomethacin(unless need PDA for survival)if indomethacin fails or child > 6-8 mos. old - surgical closure preferred
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Coarctation of the Aorta -What is it
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Narrowing prox or distal to DA=> inc. flow above,dec. flow below coarctationmore common in malesTurner's25% have bicuspid aortic valve
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Coarctation of the Aorta -Hx/PE
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Hx -often presents in childhood with asymp HTNheadachesyncopeepistaxisDOEclaudicationPE -systolic BP higher in upr extmay be gtr in right armfemoral pulses weak or delayedlate systolic murmur in left axillaapical impulse forcefuladvanced cases - well-developed upper body lwr ext wasting
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Coarctation of the Aorta -Dx
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EKG - LVHechocardiographycolor-flow dopplerCXR - "3" signrib notchingaortography - diagnostic (cardiac catheterization)
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Coarctation of the Aorta -Tx
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Surgery orballoon angioplastyendocarditis prophylaxis
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Transposition of the Great Arteries -What is it
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Pulmon & sys circ in parallelaorta connected to RVpulmon artery connected to LVincompatible with life unless septal defect or PDArisk factors - babies of DM moms Apert's syn Down's cri-du-chat Trisomies 13 & 18
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Transposition of the Great Arteries -Hx/PE
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Critically illcyanosis immed after birthtachypneaprog. resp failureCHF (some pts.)
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Transposition of the Great Arteries -Dx
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EchocardiographyCXR - "egg-shaped sihouette" "egg on a string" "apple on a string"
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Transposition of the Great Arteries -Tx
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Prostaglandin E1 (PGE1) - to keep PDA openballoon atrial septostomyarterial or atrial switch op
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Tetralogy of Fallot -What is it
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VSDpulmonary stenosisRVHoverriding aortart-to-lt shunting => early cyanosisrisk factors - Down's cri-du-chat Trisomies 13 & 18
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Tetralogy of Fallot -Hx/PE
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Hx -cyanosisdyspneafatigabilityprofound cyanosis = tet spellsquatting for reliefhypoxemia => FTT, mental status changesPE -SEM at left sternal borderRV liftsingle S2CHF signs possible
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Tetralogy of Fallot -Dx
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EchocardiographycatheterizationCXR - boot-shaped heart dec. pulmon vascular markingsEKG - rt-axis deviation RVH
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Tetralogy of Fallot -Tx
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PGE1 - keep reopen PDAfor cyanotic spells - O2 propanolol knee-chest position fluids morphineballoon atrial septostomy before surgical correction
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Cerebral Palsy (CP) –What is it
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Group of nonprogressive, nonhereditary neurological d/od/o in movement and postureMC movement d/o in kidsMCC unknown - prenatal, perinatal and post insultsrisk factors –prematurityperinatal asphyxiaintraut. growth retardationearly infection or traumabrain malformationneonatal cerebral hemorrh
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Cerebral Palsy (CP) –What are the categories
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Spastic (pyramidal) -spastic paresis of any limb75% of casesMR up to 90%athetoid -extrapyramidal, b. gangliauncontrollable jerkingwrithingworse with stressdisappears during sleepataxic -cerebellumhard to coordinate movementwide-based gaitmixed
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Cerebral Palsy (CP) –Hx/PE
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May be associated with - seizure d/o behavioral d/o hearing or vision impaired learning disabilities speech deficitshyperreflexiaBabinskiinc. tone/contracturesweaknessunderdevelopmenttoe walkingscissor gaithip dislocationsscoliosis
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Cerebral Palsy (CP) –Dx
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Clinicalr/o metabolic d/o, cerebellar dysgenesis, spinocerebellar degenEKG (if seizures)
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Cerebral Palsy (CP) –Tx
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Special edphysical therapybracessurgical rel of contracturesfor spasticity -diazepamdantrolenebaclofenfor severe contractures -baclofen pumpsposterior rhizotomy
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Febrile Seizures -What is it
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In kids 6 mos. - 6 y/ono evidence of intracranial infection or other causerisk factors - rapid rise in temp Hx in close relative
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Febrile Seizures -Hx/PE
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Most are simple seizuressimple - high feverfever onset within hrs of szgeneralized seizurelasts < 15 min.1 in a 24-hr periodcomplex -low-grade feverfever for several days before seizure onsetseizure has focal featurescan have postictal paresislasts > 15 min.> 1 in a 24-hr period
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Febrile Seizures -Dx
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Find source of infectionLP - if signs of CNS infectionNo labs if presentation consistent with febrile szAtypical presentation - electrolytes glucose BC UA CBC with diffEEG & MRI - complex seizures
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Febrile Seizures -Tx
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Simple -aggressive antipyreticstx underlying illnesscomplex -thorough neuro examchronic anticonvulsants may be necessary
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Febrile Seizures -Complications
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Febrile Sz will recur in 30%no inc. risk of epilepsy, developmental, intellectual or growth abnormthose with complex seizures - 10% risk of dev. epilepsy
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Neonatal Jaundice -What is itWhat are the typesWhat is kernicterus
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Inc. serum bilirubinfrom inc. production or dec. excretionconjugated - always pathologicuncon - patholog or physiologphysiologic jaundice -not present until 72 hrs after birthbilirubin peaks < 15 mg/dLresolves by 1 wk in termresolves by 2 wks in premiespathologic jaundice -present in 1st 24 hrs of lifebilirubin rises to > 15 mg/dLpersists past 1 wk in termpersists past 2 wks in premieskernicterus -unconjug hyperbilirubinemiabilirubin deposits in pons, basal ganglia, cerebellumirreversiblecan be fatalrisk factors - premies asphyxia sepsis
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Neonatal Jaundice -Hx/PE
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Hx -child breastfed or formula?intrauterine drugsfam Hx of - hemoglobinopathies enzyme def. RBC defectsSxs -abdom distentiondelayed passage of meconiumlight-colored stoolsdark urinelow Apgar scoresweight lossvomitingkernicterus -lethargypoor feedinghigh-pitched cryhypertonicityseizuresjaundice may be cephalopedalcheck for signs of - infection congen malformations cephalohematomas bruising pallor petechiae hepatomegaly
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Neonatal Jaundice -Dx
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CBC periph blood smearblood type mom and babyCoombs' testbilirubin levelsdirect hyperbilirubinemia -LFTsbile acidsBCsweat testtests for aminoacidopathies & a1-antitrypsin deficiencySepsis w/u and ICU -jaundicefebrilehypotensiveand/or tachypneic
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Neonatal Jaundice -Tx
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Tx underlying causeunconjugated -severe - exchange transfusionif mild - phototherapy start earlier for premies (start at 10-15 mg/dL)
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Down Syndrome -What is it
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Trisomy 21MC chromosome d/o#2 cause of congen MRrisk inc. with mom's age, but 80% of kids are born to women < 35 y/oflat facial profileprominent epicanthal foldssimian creasedec. levels of AFPbrushfield spotsduodenal atresia - double bubble on US/XRcongen heart disease -septum primum-type ASD due to endocardial cushion defectAlzheimer's > 35 y/oinc. risk of ALLmeiotic nondisjunction
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Edwards' Syndrome -What is it
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Trisomy 18 (election age=18)severe MRrocker bottom feetlow-set earsmicrognathiacongen heart disclenched handsprominent occiputdeath usu < 1 y/o
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Patau's Syndrome - What is it
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Trisomy 13 (puberty=13)severe MRmicrophthalmiamicrocephalycleft lip/palateabnorm forebrainpolydactylycongen heart diseasedeath usu < 1 y/o
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Klinefelter's Syndrome -What is it
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XXY (male)inactivated X (Barr body)1 of MCC of male hypogonadismtesticular atrophyeunuchoid body shape, longlong extremitiesgynecomastiafemale hair distribution
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Turner' Syndrome -What is it
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XO (No Barr body)short statureovarian dysgenesiswebbing of neck cystic hygromacoarctaton of aortaMCC of primary amenorrhea
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Double Y males -What is it
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XYYphenotypically normalvery tallsevere acneantisocial behavior
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Phenylketonuria (PKU) -What is it
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Phenylalanine => tyrosineIn PKU, dec. phenylalanine hydroxylase or tetrahydrobiopterin cofactortyrosine becomes essentialphenylalanine builds up=> excess phenylketonesphenylketones -phenylacetatephenyllactatephenylpyruvateMRfair skineczemamusty body odorscreened for at birthTx - dec. phenylalanine (in Nutrasweet) and inc. tyrosine in diet
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Fabry's Disease -(lysosomal storage disease)What is it
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X-linked recessivedef. of a-galactosidase Aceramide trihexoside accumsrenal failure
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Krabbe's Disease -(lysosomal storage disease)What is it
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AR (autosomal recessive)def. of B-galactosidasegalactocerebroside accums in the brainoptic atrophyspasticityearly death
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Gaucher's Disease -(lysosomal storage disease)What is it
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ARdef. of B-glucocerebrosidaseglucocerebroside accums in brain, liver, spleen, bmGaucher's cells - "crinkled paper" enlarged cytoplasmType I - more common, compa- tible with normal life span
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Niemann-Pick Disease -(lysosomal storage disease)What is it
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ARdef. of sphingomyelinasesphingomyelin and cholesterol build up in reticuloendo- thelial & parenchymal cellscherry-red spot on maculadeath by age 3
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Tay-Sachs Disease -(lysosomal storage disease)What is it
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ARabsence of hexosaminidase AGM2 ganglioside accumscherry-red spot on maculadeath by age 3MC lysosomal storage disease that causes MR
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Metachromatic leukodystrophy -(lysosomal storage disease)What is it
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ARdef. of arylsulfatase Asulfatide accums in brain, kidney, liver, periph n.
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Hurler's Syndrome -(lysosomal storage disease)What is it
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ARdef. of a-L-iduronidasecorneal cloudingMR
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Hunter's Syndrome -(lysosomal storage disease)What is it
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X-linked recessivedef. of iduronate sulfatasemild form of Hurler'sno corneal cloudingmild MR
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Fragile X Syndrome -What is it
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X-linked3rd MCC of MRFMR1 gene affectedanticipation triple repeat of CGGautismlarge testes, jaw, earsfloppy/prolapsed mitral valve
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APGAR Score -Chart
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Appearance -skin color0 = blue all over1 = blue at extremities2 = normalPulse -0 = none1 = < 1002 = > 100Grimace -reflex irritability0 = none1 = grimace, feeble cry2 = sneeze, cough, pull awayActivity -muscle tone0 = none1 = some flexion2 = active movementRespiration -0 = none1 = weak or irreg2 = strong
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APGAR Score -What do total scores mean
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Score at 1 min. after birththen at 5 min.score 8-10 -good cardiopulm adaptationscore 4-7 -possible need for resusobservestimulatepossible need for vent supportscore 0-3 -resus immed
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Erythema Infectiosum -(Fifth Disease)CauseCharacteristics
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Cause - parvovirus B19prodrome nonefever absent or low-grade"slapped cheek" erythematouspruriticmaculopapular rashgoes to armsspreads to trunk and legsworse with fever and sun
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Erythema Infectiosum -(Fifth Disease)Complications
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Arthritishemolytic anemia -aplastic crisis in sickle cellencephalopathyassociated c hydrops fetalis
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Measles -CauseCharacteristics
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Paramyxovirusprodrome -low-grade feverconjunctivitiscoryzacoughKoplik's spots -buccal mucosa after 1-2 daysmaculopap rash from ears down
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Measles -Complications
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Giant cell pneumoniaotitis medialaryngotracheitisrare - subac scleros. panencephalitis
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Rubella -CauseCharacteristics
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Rubella virusprodrome -asymp ortender, generalized lymphadenopathyerythematous, tender, maculopapular rashslight feverpolyarthritis in adolescents
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Rubella -Complications
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Encephalitisthrombocytopeniacongen infections associated with congen anomalies
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Roseola Infantum -CauseCharacteristics
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HHV-6prodrome - acute onset of high fever no other Sxs for 3-4 daysmaculopap rash as fever breaks starts on trunk => face and extremitiesoften lasts < 24 hrs
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Roseola Infantum -Complications
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Rapid fever onset =>febrile seizures
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Rotavirus -Characteristics
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Primary cause of diarrhea in kids < 2fever and vomitingthen diarrheaupper resp Sxslasts < 1 wkinfection confirmed by Elisaoral rehydration sufficient
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Varicella -CauseCharacteristics
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VZVprodrome -mild feveranorexiamalaiseprecedes rash by 24 hrsgeneralized, pruritic, "teardrop" vesicular rashstarts on trunkspreads to peripherylesions often at different stages of healinginfectious from 24 hrs before eruption til lesions crust over
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Varicella -Complications
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In immunocompromised kids -progressive varicella with meningoencephalitis and hepatitiscongen infections =>congen anomalies
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Varicella Zoster -CauseCharacteristics
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Prodrome -reactivation of Varicella infectionstarts as pain along affected sensory n.pruritic "teardrop" vesicular rash in dermatomal distributionuncommon unless immunocomp
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Varicella Zoster -Complications
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Encephalopathyaseptic meningitispneumonitisTTPGuillain-Barrecellulitisarthritis
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Hand-Foot-and-Mouth Disease -CauseCharacteristics
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Coxsackie Aprodrome -feveranorexiaoral painrash -oral ulcersmaculopap vesicular rash on hands, feet, buttocks
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Hand-Foot-and-Mouth Disease -Complications
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None(self-limited)
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Tracheoesophageal Fistula -What is it
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Tract between trachea & esophassociated with esoph atresia & VACTERL anomalies - vertebral anal cardiac tracheal esophagus renal limb
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Tracheoesophageal Fistula -Caused byPresentation
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Polyhydramnios in uteroinc. oral secretioninability to feedgaggingresp distress
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Tracheoesophageal Fistula -Dx
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CXR after NGTair in GI tractbronchoscopy - to confirm
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Tracheoesophageal Fistula -Tx
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Surgical repair
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Congenital Diaph Hernia -What is it
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GI tract segment protrudes thru diaph into thorax90% are post. lt. Bochdalek
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Congenital Diaph Hernia -Presentation
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Resp distress from - pulmonary hypoplasia pulmonary HTNsunken abdomenbowel sounds over lt. hemithorax
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Congenital Diaph Hernia -Dx
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US in uteropostnatal CXR - to confirm
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Congenital Diaph Hernia -Tx
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Hi-freq ventilation or extracorporeal membrane oxygenation (ECMO) (to manage pulmonary HTN)surgical repair
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Gastroschisis -What is it
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Herniation of intestine thru abdom wall next to umbilicus (usually on right) with no sac
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Gastroschisis -Caused byAssociated with
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Polyhydramnios in uterooften premieassociated with - GI stenoses GI atresia
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Gastroschisis -Tx
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Surgical emergencysingle-stage closure possible in only 10%
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Omphalocele -What is it
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Herniation of abdom viscera thru abdom wall at umbilicus into sac covered by peritoneum and amniotic memb
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Omphalocele -Caused byAssociated with
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Polyhydramnios in uterooften premieassociated with other GI & cardiac defects
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Omphalocele -Tx
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C-section - to prevent sac ruptureif sac intact -postpone surg correction until pt. fully resuscitatedkeep sac covered/stable with petroleum & gauzeintermittent NG suction -to prevent abdom distention
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Duodenal Atresia -What is it
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Complete or partial failure of duodenal lumen to recanalize during gestational wks 8-10
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Duodenal Atresia -PresentationCaused byAssociated with
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Bilious emesis within hrs after 1st feedingpolyhydramnios in uteroassociated with - Down's other cardiac/GI anomalies: annular pancreas malrotation imperforate anus
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Duodenal Atresia -Dx
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Double-bubble sign on XR(prox to site of atresia)
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Duodenal Atresia -Tx
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Surgical repair
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Meckel's Diverticulum -What is it
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MC congen GI tract anomalyvestigial remnant of omphalomesenteric ductrule of 2's -2x's as many males2 ft. from ileocecal valve2% of people affected2 types of mucosa - gastric pancreatic
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Meckel's Diverticulum -Presentation
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MC presentation - painless rectal bleedingpainful diverticulitisintest. obstruction from - intussusception or volvulus
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Meckel's Diverticulum -Dx
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Meckel's scan -for ectopic gastric mucosauses IV technetium pertechnetate
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Meckel's Diverticulum -Tx
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Surgery
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Hirschsprung's Disease -(Congen Aganglionic Megacolon)What is it
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Absence of autonomic innervation of bowel wallinadeq relaxation and peristalsis => intest. obstruction
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Hirschsprung's Disease -(Congen Aganglionic Megacolon)Presentation
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Abdom distentionbilious vomitingfail to pass meconium in 1st 24 hrs of life
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Hirschsprung's Disease -(Congen Aganglionic Megacolon)Dx
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Barium enema - dilated prox segment narrowed distal segmentrectal Bx - to confirm
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Hirschsprung's Disease -(Congen Aganglionic Megacolon)Tx
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Colostomy prior to corrective surgery
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Hypospadias -What is it
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Abnorm urethral opening on ventral surface of penisdue to incomplete dev of distal urethra
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Hypospadias -PresentationAssociated with
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Chordeeassociated with - hernias cryptorchidism
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Hypospadias -Tx
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Circumcision contraindicatedsurgical repair uses preputial tissue
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X-linked Agammaglobulinemia(Bruton's Disease) -What is it
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B-cell def.boys onlymay present < 6 mos. of ageat risk for life-threatening Pseudomonas infections
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X-linked Agammaglobulinemia(Bruton's Disease) -Dx
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No B cellslow levels of all Ab classes
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X-linked Agammaglobulinemia(Bruton's Disease) -Tx
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IVIGprophylactic ABx
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Common Variable Immunodeficiency -What is it
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Ig levels drop in 2nd-3rd decade of lifeinc. risk of lymphomainc. risk of autoimmune dis.
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Common Variable ImmunodeficiencyDx
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Ig levelsantibody titers
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Common Variable ImmunodeficiencyTx
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IVIGprophylactic ABx
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IgA Deficiency -What is it
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MC immunodeficiencyusually asymptomaticmay have recurrent infections
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DiGeorge Syndrome(Thymic aplasia) -What is it
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CATCH-22Cardiac defectsAbnormal faciesThymic hypoplasiaCleft palateHypocalcemia22 - microdeletions in chrom22tetany in first days of life
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DiGeorge Syndrome(Thymic aplasia) -Dx
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Absolute lymphocyte count,mitogen stimulation responseand delayed hypersensitivity skin testing
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DiGeorge Syndrome(Thymic aplasia) -Tx
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BMT - if severeIVIGthymus transplant
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Ataxia-Telangiectasia -What is it
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DNA repair defectoculocutaneous telangiectasiasprogressive cerebellar ataxia
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Ataxia-TelangiectasiaTx
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No effective Tx for CNS abnormneuro deterioration progressesdeath by 30 y/o
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Severe Combined Immunodeficiency (SCID) -What is it
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Severe lack of B & T cellsfreq. severe bact. infectionschronic Candidiasisopportunistic organisms
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Severe Combined Immunodeficiency (SCID) -Tx
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BMT or stem cell transplantIVIGPCP prophlaxis until BMTgene therapy may be future option
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Wiskott-Aldrich Syndrome -What is it
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X-linked recessiveT & B cell dysfunctionthrombocytopeniasmall-sized plateletseczemahigh IgEhigh IgAlow IgMbloody diarrheableeding gumsprolonged nosebleeds
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Wiskott-Aldrich Syndrome -Tx
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Protective helmetIVIGaggressive ABx for infectionsHLA-identical BMTif no BMT - rarely survive to adulthood
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Chronic Granulomatous Disease-(CGD)What is it
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X-linked or ARdeficient superoxide production by PMNs & M0susual sites of infection - skin lungs (pneumonia) lymph nodes liver (abscesses, hepatitis) bones (osteomyelitis)swollen collections of infected tissue obstruct intestines (IBD) and urinary tract (UTIs)
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Chronic Granulomatous Disease-(CGD)Dx
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Absolute neutrophil count and adhesionassays - chemotaxic phagocytic bactericidaldiagnostic -neg. nitroblue tetrazolium dye reduction test (NBT)
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Chronic Granulomatous Disease-(CGD)Tx
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Daily TMP-SMXjudicious ABx use during infectionsIFN-g - can dec. incidence of serious infection
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Chediak-Higashi -What is it
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ARgiant lysosomal granules dev. in neutrophilslysosomes can't fuse with phagosomes =>ingested bact. can't be lysedoculocutaneous albinismneuropathyneutropenia
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C1 Esterase (Inhib) Def. -(Hereditary Angioedema)What is it
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C1 inhibitor -acute phase proteininhibits proteinases of: complement pathway clotting pathway kinin generator pathway fibrinolytic pathwaydeficiency => her. angioedemaADcan affect - hands & feet - local edema bowel - extreme abdom pain mouth airway - life-threat. edemausually lasts 3 dayscan be precip by trauma, virusaggravated by stress
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C1 Esterase (Inhib) Def. -(Hereditary Angioedema)Dx
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Total hemolytic C' assay- CH50if defect in one component - no CH50 reductionthen det. which component
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C1 Esterase (Inhib) Def. -(Hereditary Angioedema)Tx
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Daily prophylactic danazolpurified C1 esterase and FFP - prior to surgery
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Terminal Complement Deficiency(C5-C9) -What is it
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ARrecurrent N. meningitidis& dissem. gonorrhea infectionsrarely - systemic lupus
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Terminal Complement Deficiency(C5-C9) -Tx
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Meningococcal vaccineappropriate ABx
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Hyper IgM Syndrome -What is it
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MC - def. in CD40 ligand in T Helper=> can't class switchnormal or high IgMlow IgG, IgA, IgEXL recessive (most common)AR (others)severe upr & lwr respiratorydiarrhea - Cryptosporidium
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Hyper IgM Syndrome -Tx
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IVIGPCP prophylaxis - TMP-SMX
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