GI/bilirubin

Question 1

What is one thing associated with congenital pyloric stenosis and one thing associated with acquired pyloric stenosis

Answer 1

congenital - SLO

acquired – PGE

Question 2

What three random syndromes are associated with Hirschprung’s

Answer 2

SLO, Waardenburg, and congenital hypoventilation syndrome

Question 3

Intrahepatic causes of cholestasis?

Answer 3

idiopathic hepatitis

Alagille (paucity of intrahepatic bile ducts)

Question 4

Extrahepatic causes of cholestasis?

Answer 4

Biliary atresia
Sclerosing cholangitis
choledochal cysts
Bile duct plug/stenosis

Question 5

What presents similarly to DA

Answer 5

annular pancreas (when ventral pancreas is supposed to rotate around duodenum but doesn’t, it compresses the duodenum) - can be associated with other duodenal anomalies but not systemic anomalies like DA

Question 6

Do premies have higher or lower gastric pH?

Answer 6

Higher pH (more alkalotic) because of decreased secretion of HCl

Question 7

Labs in GALD/hemochromotosis? What must it be differentiated from and how can you tell the difference?

Answer 7

See elevated LFT’s and coags, but also ferritin! Those are the same as HLH (hyper-activated T cells and macrophages), but HLH will also have high temp, high TG, low neutrophils, low NK cells, hemophagocytosis, and lucent/lytic lesions of bones

Question 8

3 stages of bilirubin encephalopathy?

Answer 8

stage 1: decreased activity, poor suck, hypotonia
stage 2: hypertonic, rigid extension of 4 limbs, tight fists, irritable high-pitched cry, retrocollis, opisthotonos
stage 3: worse opisthotonos, stupor, coma, shrill cry

with opisthotonos, irreversible damage has occurred

Question 9

wavelength and irradiance for phototherapy?

Answer 9

460-490 and >30

Question 10

more common between duodenal vs. jejunal atresia

Answer 10

jejunal-ileal atresia more common