Pathology of the GI Tract- Liver and Gallbladder (6)

Question 1

What are choledochal cyst and what do the predispose to?

Answer 1

congenital dilations of the common bile duct; predispose to stone formation, stenosis and stricture, pancreatitis, and obstructive biliary complications within the liver

Question 2

what is fibropolycystic disease of the liver?

Answer 2

a heterogenous group of lesions in which the primary abnormalities are congenital malformations of the biliary tree

Question 3

the lesions seen in fibropolycystic disease of the liver are a part of what spectrum?

Answer 3

part of the spectrum of developmental ductal plate malformations, which result from persistence of the fetal periportal ductal plates

Question 4

what are Von meyenburg complexes and why are they significant?

Answer 4

they are small bile duct hamartomas. When they are diffuse, they signal the presence of underlying, clinically important fibropolycystic disease

Question 5

fibropolycystic disease of the liver lesions often occur in association with what?

Answer 5

autosomal recessive polycystic renal disease

Question 6

what is fibripolycystic disease of the liver associated with an increased risk of?

Answer 6

cholangiocarcinoma

Question 7

what is Caroli disease?

Answer 7

multifocal cystic dilation of the large intrahepatic bile ducts

Question 8

what is Caroli syndrome?

Answer 8

when you have caroli disease and congenital hepatic fibrosis

Question 9

what are the 3 forms of hepatic circulatory disorders?

Answer 9

impaired blood inflow; impaired intrahepatic blood flow; hepatic vein outflow obstruction

Question 10

What could cause impaired blood inflow into the liver?

Answer 10

portal vein obstruction or intra- or extrahepatic thrombosis

Question 11

what could cause impaired intrahepatic blood flow?

Answer 11

cirrhosis or sinusoid occlusion

Question 12

what could cause hepatic vein outflow obstruction?

Answer 12

hepatic vein thrombosis (Budd-Chiari syndrome); sinusoidal obstructive syndrome

Question 13

what are the manifestations of impaired blood inflow into the liver? (3)

Answer 13

esophageal varices, splenomegaly, and intestinal congestion

Question 14

what are the manifestations of impaired intrahepatic blood flow? (4)

Answer 14

ascites, esophageal varices, hepatomegaly, elevated aminotransferases

Question 15

what are the manifestations of hepatic vein outflow obstruction? (5)

Answer 15

ascites, hepatomegaly, abdominal pain, elevated aminotransferases, jaundice

Question 16

obstruction of an intrahepatic branch of the hepatic artery by emboli, thrombi, or compression may result in what?

Answer 16

a localized infarct that is either pale and anemic, or hemorrhagic if there is suffusion with portal blood

Question 17

how does occlusive disease of the portal vein or its major branches present?

Answer 17

abdominal pain, portal hypertension, esophageal varices that are prone to rupture

Question 18

when might obstruction of the extrahepatic portal vein arise?

Answer 18

neonatal umbilical sepsis or umbilical vein catheterization

Question 19

the most common cause of small portal vein branch obstruction is what?

Answer 19

schistosomiasis- the eggs of the parasites and the associated granulomatous inflammatory response obstruct the smallest portal vein branches

Question 20

where is noncirrhotic portal fibrosis and hypertension particularly common?

Answer 20

india

Question 21

occlusion of sinusoidal blood flow occurs in the following diseases?

Answer 21

sickle cell disease, DIC, eclampsia, and diffuse intrasinusoidal tumor

Question 22

what is Peliosis hepatis?

Answer 22

sinusoidal dilation that occurs in any condition in which efflux of hepatic blood is impeded; the liver contains blood-filled cystic spaces

Question 23

what is Budd-Chiari syndrome?

Answer 23

the obstruction of major hepatic veins

Question 24

what is the classic presentation of Budd-chiari syndrome?

Answer 24

triad: hepatomegaly, abdominal pain, and ascites

Question 25

hepatic vein thrombosis is associated with what?

Answer 25

myeloproliferative neoplasms such as polycythemia vera, inherited disorders of coagulation, PNH, and intraabdominal cancers

Question 26

what is occurring in sinusoidal obstruction syndrome?

Answer 26

there is toxic injury to the sinusoidal endothelium; injured sloughed endothelium obstructs sinusoidal blood flow, and associated debris accumulates in the terminal hepatic vein

Question 27

when might sinusoidal obstruction syndrome occur?

Answer 27

following allogenic hematopoietic stem cell transplantation or in cancer patients receiving certain forms of chemotherapy

Question 28

what is the gold standard for diagnosing sinusoidal obstruction syndrome?

Answer 28

histology- but biopsy is risky, so it is often made on clinical grounds

Question 29

what are the common themes of liver damage following transplantation?

Answer 29

toxic or immunologically mediated liver damage, opportunistic infections, post transplant lymphoproliferative processes

Question 30

What liver pathologies are associated with pregnancy?

Answer 30

viral hepatitis- especially HEV; other infections like HSV, listeria monocytogenes; preeclampsia and eclampsia, and acute fatty liver of pregnancy

Question 31

how is preeclampsia and eclampsia characterized?

Answer 31

maternal hypertension, proteinuria, peripheral edema, and coagulation abnormalities

Question 32

subclinical hepatic disease may be the primary manifestation of preeclampsia- what are these manifestations?

Answer 32

hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome)

Question 33

how does acute fatty liver of pregnancy present?

Answer 33

in the latter half of pregnancy, with bleeding, n/v, jaundice, and coma

Question 34

how does intrahepatic cholestasis of pregnancy present?

Answer 34

the onset of pruritus in the second or third trimester, followed in some by darkening of the urine, light stools, and jaundice

Question 35

what does the biopsy show in a patient with intrahepatic cholestasis of pregnancy?

Answer 35

canalicular cholestasis