Misc. GI and Liver Dz PART 1

Question 1

What causes physiologic jaundice

Answer 1

liver immature in neonates, low conjugating and excreting bilirubin not fully operational until 2 wks. severe hyperbilirubinemia injures brain, can be tx with UV lights

Question 2

what are things causing conjugated jaundice

Answer 2

obstruction, hepatitis, dubin-johnson

Question 3

what are things causing unconjugated jaundice

Answer 3

hemolysis, hepatitis, gilbert

Question 4

what is biliary atresia?

Answer 4

a progressive inflammatory and fibrosing dz of extra hepatic bile ducts with gradual complete obstruction of bile flow caused by destruction of extrahepative bile ducts

Question 5

what is the most common cause of death from liver dz in early childhood

Answer 5

biliary atresia

Question 6

when does jaundice present in biliary atresia?

Answer 6

3-6 weeks of age, as opposed to immediately with physiologic jaundice

Question 7

how are ggt, alt and ast levels in biliary atresia?

Answer 7

ggt is disproportionately high, ast and alt mild-moderately high

Question 8

is alk phos useful in biliary atresia with kids? why?

Answer 8

no because alk phos in kids is high because they are growing

Question 9

what is alagille syndrome?

Answer 9

syndromic paucity of intrahepatic bile ducts,

Question 10

how do you present with alagille syndrome?

Answer 10

neonatal jaundice, pruritis and cholestasis plus triangular face

Question 11

what is another name for alagille syndrome?

Answer 11

arteriohepatic dysplasia

Question 12

what is the mutation in alagille syndomre

Answer 12

jagged 1 gene for ligand for notch 1 receptor

Question 13

what is dubin-johnson syndrome

Answer 13

hereditary conjugated hyperbilirubinemia, due to defective excretion of bili conjugates and other oragnic anions across hepatocyte canalicular membranes

Question 14

what does dubin-johnson look like on histo?

Answer 14

brown=black pigment in liver with lysosomes containing polymers of epinephrine metabolites

Question 15

what is gilbert’s syndrome?

Answer 15

ar defi of ugt enzyme that conjugated bilirubin

Question 16

what does gilbert’s give a good prognosis for?

Answer 16

colon cancer, cancer mortality, atherosclerotic heart dz

Question 17

what is primary biliary cirrhosis?

Answer 17

ai slowly progressive dz in late middle aged women presenting with pruritis, fatigue hepatomegaly elevated alk phos eventually with jaundice steatorrhea and xanthomas

Question 18

what is the marker for primary biliary cirrhosis

Answer 18

anti-mitchondrial ab

Question 19

what is primary sclerosing cholangitis?

Answer 19

probably ai fibrosing cholangitis of intra and extra hepatic bile ducts

Question 20

what is associated with PSC

Answer 20

inflammatory bowel dz, typically in early middle aged men

Question 21

what does PSC present with

Answer 21

jaundice fatigue pruritis, beading of bile ducts due to strictures and dilatations

Question 22

what is budd-chiari syndrome

Answer 22

portal HTN due to thrombosis of hepatic veins.

Question 23

what dzs are associated with budd-chiari

Answer 23

polycythemia vera, myeloproliferative dz, pregnancy, postpartum state, oral contraception, abdominal cancer

Question 24

what lobe of the liver hypertrophies in budd-chiara

Answer 24

caudate

Question 25

what are complications of acute cholecystitis?

Answer 25

gangrene or rupture

Question 26

in noncalculous cholecystitis, what seriously ill pts are common

Answer 26

postop after major surgery

severe trauma or burn or sepsis

Question 27

chronic cholecystitis is associated with what bacteria

Answer 27

e coli or enterococci

Question 28

what is choledocholithiasis

Answer 28

gallstones in bile ducts

Question 29

what is ascending cholangitis due to

Answer 29

e coli, klebsielle, clostridium bacteroides enterbacter, enteroccous, - all acute

chronic - parasites