Misc. GI and Liver Dz PART 1 Flashcards
What causes physiologic jaundice
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
what are things causing conjugated jaundice
obstruction, hepatitis, dubin-johnson
what are things causing unconjugated jaundice
hemolysis, hepatitis, gilbert
what is biliary atresia?
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
what is the most common cause of death from liver dz in early childhood
biliary atresia
when does jaundice present in biliary atresia?
3-6 weeks of age, as opposed to immediately with physiologic jaundice
how are ggt, alt and ast levels in biliary atresia?
ggt is disproportionately high, ast and alt mild-moderately high
is alk phos useful in biliary atresia with kids? why?
no because alk phos in kids is high because they are growing
what is alagille syndrome?
syndromic paucity of intrahepatic bile ducts,
how do you present with alagille syndrome?
neonatal jaundice, pruritis and cholestasis plus triangular face
what is another name for alagille syndrome?
arteriohepatic dysplasia
what is the mutation in alagille syndomre
jagged 1 gene for ligand for notch 1 receptor
what is dubin-johnson syndrome
hereditary conjugated hyperbilirubinemia, due to defective excretion of bili conjugates and other oragnic anions across hepatocyte canalicular membranes
what does dubin-johnson look like on histo?
brown=black pigment in liver with lysosomes containing polymers of epinephrine metabolites
what is gilbert’s syndrome?
ar defi of ugt enzyme that conjugated bilirubin
what does gilbert’s give a good prognosis for?
colon cancer, cancer mortality, atherosclerotic heart dz
what is primary biliary cirrhosis?
ai slowly progressive dz in late middle aged women presenting with pruritis, fatigue hepatomegaly elevated alk phos eventually with jaundice steatorrhea and xanthomas
what is the marker for primary biliary cirrhosis
anti-mitchondrial ab
what is primary sclerosing cholangitis?
probably ai fibrosing cholangitis of intra and extra hepatic bile ducts
what is associated with PSC
inflammatory bowel dz, typically in early middle aged men
what does PSC present with
jaundice fatigue pruritis, beading of bile ducts due to strictures and dilatations
what is budd-chiari syndrome
portal HTN due to thrombosis of hepatic veins.
what dzs are associated with budd-chiari
polycythemia vera, myeloproliferative dz, pregnancy, postpartum state, oral contraception, abdominal cancer
what lobe of the liver hypertrophies in budd-chiara
caudate
what are complications of acute cholecystitis?
gangrene or rupture
in noncalculous cholecystitis, what seriously ill pts are common
postop after major surgery
severe trauma or burn or sepsis
chronic cholecystitis is associated with what bacteria
e coli or enterococci
what is choledocholithiasis
gallstones in bile ducts
what is ascending cholangitis due to
e coli, klebsielle, clostridium bacteroides enterbacter, enteroccous, - all acute
chronic - parasites