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Step III
Step III - Peds 7
| Question | Answer |
|---|---|
| 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 |