Pathology of the GI Tract- Liver and Gallbladder (6) Flashcards
What are choledochal cyst and what do the predispose to?
congenital dilations of the common bile duct; predispose to stone formation, stenosis and stricture, pancreatitis, and obstructive biliary complications within the liver
what is fibropolycystic disease of the liver?
a heterogenous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree
the lesions seen in fibropolycystic disease of the liver are a part of what spectrum?
part of the spectrum of developmental ductal plate malformations, which result from persistence of the fetal periportal ductal plates
what are Von meyenburg complexes and why are they significant?
they are small bile duct hamartomas. When they are diffuse, they signal the presence of underlying, clinically important fibropolycystic disease
fibropolycystic disease of the liver lesions often occur in association with what?
autosomal recessive polycystic renal disease
what is fibripolycystic disease of the liver associated with an increased risk of?
cholangiocarcinoma
what is Caroli disease?
multifocal cystic dilation of the large intrahepatic bile ducts
what is Caroli syndrome?
when you have caroli disease and congenital hepatic fibrosis
what are the 3 forms of hepatic circulatory disorders?
impaired blood inflow; impaired intrahepatic blood flow; hepatic vein outflow obstruction
What could cause impaired blood inflow into the liver?
portal vein obstruction or intra- or extrahepatic thrombosis
what could cause impaired intrahepatic blood flow?
cirrhosis or sinusoid occlusion
what could cause hepatic vein outflow obstruction?
hepatic vein thrombosis (Budd-Chiari syndrome); sinusoidal obstructive syndrome
what are the manifestations of impaired blood inflow into the liver? (3)
esophageal varices, splenomegaly, and intestinal congestion
what are the manifestations of impaired intrahepatic blood flow? (4)
ascites, esophageal varices, hepatomegaly, elevated aminotransferases
what are the manifestations of hepatic vein outflow obstruction? (5)
ascites, hepatomegaly, abdominal pain, elevated aminotransferases, jaundice
obstruction of an intrahepatic branch of the hepatic artery by emboli, thrombi, or compression may result in what?
a localized infarct that is either pale and anemic, or hemorrhagic if there is suffusion with portal blood
how does occlusive disease of the portal vein or its major branches present?
abdominal pain, portal hypertension, esophageal varices that are prone to rupture
when might obstruction of the extrahepatic portal vein arise?
neonatal umbilical sepsis or umbilical vein catheterization
the most common cause of small portal vein branch obstruction is what?
schistosomiasis- the eggs of the parasites and the associated granulomatous inflammatory response obstruct the smallest portal vein branches
where is noncirrhotic portal fibrosis and hypertension particularly common?
india
occlusion of sinusoidal blood flow occurs in the following diseases?
sickle cell disease, DIC, eclampsia, and diffuse intrasinusoidal tumor
what is Peliosis hepatis?
sinusoidal dilation that occurs in any condition in which efflux of hepatic blood is impeded; the liver contains blood-filled cystic spaces
what is Budd-Chiari syndrome?
the obstruction of major hepatic veins
what is the classic presentation of Budd-chiari syndrome?
triad: hepatomegaly, abdominal pain, and ascites
hepatic vein thrombosis is associated with what?
myeloproliferative neoplasms such as polycythemia vera, inherited disorders of coagulation, PNH, and intraabdominal cancers
what is occurring in sinusoidal obstruction syndrome?
there is toxic injury to the sinusoidal endothelium; injured sloughed endothelium obstructs sinusoidal blood flow, and associated debris accumulates in the terminal hepatic vein
when might sinusoidal obstruction syndrome occur?
following allogenic hematopoietic stem cell transplantation or in cancer patients receiving certain forms of chemotherapy
what is the gold standard for diagnosing sinusoidal obstruction syndrome?
histology- but biopsy is risky, so it is often made on clinical grounds
what are the common themes of liver damage following transplantation?
toxic or immunologically mediated liver damage, opportunistic infections, post transplant lymphoproliferative processes
What liver pathologies are associated with pregnancy?
viral hepatitis- especially HEV; other infections like HSV, listeria monocytogenes; preeclampsia and eclampsia, and acute fatty liver of pregnancy
how is preeclampsia and eclampsia characterized?
maternal hypertension, proteinuria, peripheral edema, and coagulation abnormalities
subclinical hepatic disease may be the primary manifestation of preeclampsia- what are these manifestations?
hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome)
how does acute fatty liver of pregnancy present?
in the latter half of pregnancy, with bleeding, n/v, jaundice, and coma
how does intrahepatic cholestasis of pregnancy present?
the onset of pruritus in the second or third trimester, followed in some by darkening of the urine, light stools, and jaundice
what does the biopsy show in a patient with intrahepatic cholestasis of pregnancy?
canalicular cholestasis
