Test #3 (surgical, metabolic, NEC, Neuro, GU) Flashcards
What defect is when the umbilical cord in uninvolved and to the right of the umbilicus?
gastroscheisis
What does gastroschisis appear in embryonic life?
4 week
What abd midline defect is associated with heart defects?
omphalocele
Where does an omphalocele defect herniate?
through the umbilical ring
When does omphalocele efect fail to return to abd cavity?
11 weeks
What test should you have done before you do surgery on omphalocele?
ECHO
What should you start IV fluids at 150ml/kg/day on day 1 with abd wall defects?
Bc of continuous sx on og output
What is the % of Cardiac anomalies with TEF/eso atresia?
30%
What defect has an x-ray with a gasless abd?
Eso atresia ONLY
What is another cue on an x-ray with EA?
dilation esophageal pouch
What is an ER surgery for EA/TEF?
gastrostomy for decompression
Long term probs with EA/TEF: stricture/GE reflux, anastamotic leak, esophageal dysmotility all cause ____.
risk of aspiration
What defect shows a double bubble?
Duodenal atresia
Where is the level of obstruction in duodenal atresia?
distal to ampulla of Vater
What is the sign seen on 1st DOL with malroatation/volvulus?
Bilious vomiting
What disease is seen with congenital aganglionic megacolon?
Hirschprung’s disease
What should you think when an infant has a meconium ileus?
Cystic fibrosis (90%)
What abd defect should be repaired ASAP?
gastroschisis
NEC correlates with degree of ____
Prematurity
What is a critical finding in NEC that is uncorrectable?
Dead gut
Free air seen in the abdominal x-ray is ____ with NEC
Pneumoperitoneum
Persistent acidosis suggests what in NEC?
Necrotic bowel
What is removed with surgery with NEC?
Frankly necrotic bowel
What is a normal finding on NBS r/t prematurity?
decrease T4
If left untreated what leads to irreversible brain damage and death?
Hypothyriodism
What is an inborn error of with smelly urine?
Maple syrup urine diesase
An X-ray with a double bubble, what else would you evaluate if the baby had trisomy 21?
Cardiac anomalies
If a baby has galactosemia what formula should you feed?
Isomil
Why in the fetus unaffected with inborn errors of metabolism?
the placenta removes the toxins
What are some clues in family hx with Inborn errors of metabolism?
MR, SIDS, unexplained neonatal death
What are some common s/s inborn errors of metabolism?
vomiting, poor fdg, no wt gain, diarrhea, jaundiace, heptomegaly
When the radiologist says the cecum is riding high think!
Its not in the right place and probably malrotation
When there is an increase in lactate with metabolic acidosis you should think?
Pyruvate metabolism defects
What are the first dx studies for inborn errors of metabolism?
CBC
Urinalysis
CBG
Electrolytes, glucose, ammonia level
With dx studies for inborn errors of metabolism, a urinalysis should be tested for?
reducing substances
What is seen in inborn errors of metabolism with hypoglycemia?
- urine non-glucose reducing substances
- galaxtosemia, tyrosimemia type 1
In 21-OH deficiency what should be given to suppress SCTH and androgen overproduction?
Cortisol replacement - hydrocortisone
what is the medical mgnt for Congential Adrenal hyperplasia?
glucocorticoid therapy
mineralocorticoid therapy
Na supplementation
What is the typical age of onset for NEC?
for late preterm?
2-3 weeks
~7days
What are two major associations with NEC?
preinatal hypoxia
polycythemia
What NEC is diminished bowel sounds, dilation, and pneumatosis.
Stage 2
What NEC shows portal vein gas, metabolic acidosis, and throbocytopenia
Stage 2B
What NEC stage is Neutropenia, abdominal wall erythema, hyoptension
Stage 3A
What NEC stage has perforated bowel, shock, and pneumoperitoneum?
Stage 3B
What is a tale tell sign with NEC?
Hyponatremia and hypothermia
What is when a piece of bowel does not move and can indicate dead bowel?
Sentinel loop
What is the tx for intestinal perf in ELBW along with explo lap?
PPD (peritoneal drainage) pin rose drain
What defines short bowel syndrome?
What intestinal disease that is not assoc. with pneumoatosis, unrelated to feeds?
Spon intestinal perforation
What are 3 signs consistent with acute peripartum event?
APGAR 12
What are some treatment options to prevent severe CND deficits?
Ventilate, prevent hypoxia, treat seizures, stable Vitals
What are the eligibility criteria for therapeutic hypothermia?
Fetal cord gas with pH 16
acute perinatal event,
APGAR
What is the most common type of seizure and most often missed?
Subtle seizure
Seizure or Jitters:
when activity easily elicited with stimulation
Jitterness
What drug and dosage used for seizures?
Phenobarbital: Load with 20mg/kg, sz cont: 20mg/kg
theraputic level 15-40mg
What are the outcomes for lower birth wt infants?
The outcome is worse with more severe neurological disease.
Describe a grade 2 and 3 IVH?
II - IVH with normal ventricles
III - IVH with dilated ventricles
PVL can occure separate from what?
P/IVH
What will you see with a LP in Subarachnoid hemorrhage?
Blood in the LP
what are some disease that cause prerenal failure
asphyxia, CHF, dehydration, hypotension, RDS, CHD
What are some examples of Postrenal failure?
prune belly, renal calculi, blood clots, neurologenic dysfunction, ureterocele
What is a good indicator of renal failure?
Urine SG prerenal >1.015, posrenal
What is a good indicator of renal intrinsic dz?
granulae casts and spitheleal cells in urine
What is the most common abdominal mass?
hydronephrosis
What test can be preformed if there is a vesicourethral reflux?
VCUG
What is the abnormal flow or urine from the bladder back into the ureters?
vesicourethral reflux?
What are three clinical manifestations of ureterocele?
hematuria, abd mass, UTI
What is the most common cause of bladder outlet obstruction in male infants?
Posterior urethreal valve
What is a common sign of Posterior urethreal valves?
palpable distended bladder
What is an autosomal recessive disease that is rare with poor prognosis?
Polycystic kidney disease
What are some preinatal causes of renal vein thrombosis?
toxemia, maternal diabetes, placental insufficiency
What are the most common S/S of renal vein thrombosis?
Mass, gross hematuria, and HTN!!!
When there is a sudden severe blockage of the renal artery?
Renal artery thrombosis - lines
What is a good indication of renal ARTeral thrombosis?
systemic HTN
