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Step III

Step III - Peds 7

QuestionAnswer
Tx for Cleft lip Surgery by or AT 3mos
Tx for Cleft palate Special nipple for feeding then repair at 6mos
Failure to thrive, N/V, Cataracts, Large liver Galactosemia
Palpable kidneys, Can’t pee, UT obstruction, h/o oligohydramnios Posterior urethral valve
Diagnostic test for posterior urethral valve Voiding cysto
LOW: Sugar, Ca+, Platelets, Mg. Other: RDS, Polycythemia, HCM, Premature delivery, ↑bili Infant of DM mother
Sudden decline motor and cognitive fxn. Looks like autism. FEMALES by 18mos. Grinding teeth, Flapping hands, Scoliosis Rett syndrome
Kidney + hearing loss Alport dz
Blue sclera + hearing loss OI
Male, Abdominal muscles are abnl, Renal dysplasia, Dilated/tortuous ureters, BIG bladder, Heart + GI, Cryptoorchidism, Small prostate, Pulm HYPOplasia Prune belly syndrome
Prune belly syndrome c/w Prader Willi syndrome Hypotonia, cryptoorchidism, feeding problems
Port wine V1/V2, Glaucoma, MR,Seizures Sturge weber
HYPOmelanotic macules “ash leaf”, Facial angiofibromoas, Cutaneous nevi Tuberous sclerosis
HYPERpigment 6+ café au lait spots, Freckling @ GROIN, AXILLA, Hamartoma iris (LISCH nodules) NF1
BILAT acoustic neuromas NF2
Clear cell renal CA, Pheo, Hemangioblastomas, Von Hippel Lindau
Failed passage of meconium w/i 48hrs, Bilious vomiting, XR: obstruction Hirschsprung
HSM, Petechiae, Low birth wt, Microcephaly, Direct bili,  platelets, IC calcifications CMV from in utero
Mental retardx, Aniridia, 3-4yo, Ab pain, Flank mass Wilm’s tumor aka Nephroblastoma
WAGR syndrome = Wilms, Aniridia, GU, Mental retardation
Radio eval of Wilm’s tumor Ultrasound then CT
Tx of Wilm’s Tumor Nephrectomy, Chemo, Radx, Tumor in kidneys
Wilms tumor in association with organomegaly, macroglossia and neonatal hypoglycemia = Beckwith-Weidemann syndrome
Abdominal mass in 2-4 yo w/  catecholamines Neuroblastoma (adrenal gland)
Cells of origin for Wilm’s tumor mesodermal
Prognosis of Wilm’s tumor excellent
Cells of origin for neuroblastoma Neural crest
gold standard for confirming reflux in neonate 24hr pH monitoring
gold standard for confirming pyloric stenosis US
Tetra Fallot presents w/ VSD, RVH, overiding aorta, (R) outflow obstruction/pulmonary stenosis
Irritable, Turning blue, Syncope, Kid will start to squat during spells, Systolic murmur (L) up radx to back Tetra Fallot
Boot shape heart = tetra fallot
Tx for tetra Fallot O2, β (-), PGE1 infusion if blue at birth
When is surgery done for tetra fallot Surgery @ 4-12mos
Cyanosis in tetra fallot is d/t R to L shunting
What are the cyanotic CHD Tetra fallot / transposition of great vessels / total anomalous pulm venous return / truncus arteriosus / tricuspid atresia
Cyanosis Within HOURS of birth Transposition great vessels
“egg on string” XR Transposition great vessels
Tx for Transposition great vessels PE1, 2 (keep PDA open)
1 vessel for RV+LV w/ VSD, Large heart, Fluid in lungs Truncus arteriosus
4 pulm veins drain to RA/IVC/SVC Total anomalous pulm venous return
“snowman” XR Total anomalous pulm venous return
Requires ASD/foramen ovale to exist Total anomalous pulm venous return
Large RA Tricuspid atresia
Older <14 yo c/o HIP pain, Obese kid SCFE
Tx for SCFE Surgical pinning
4-10yo male, MC: 7yo MALE c/o HIP pain, Short stature, Referred pain to knee, thigh, groin Legg Calve Perthes
Pathophys of Legg Calve Perthes Avascular necrosis of fem head
Tx for Legg Calve Perthes Splint + decr WB
Created by: DrINFJ