Ch 18 Robbins part II Flashcards
Which type of Crigler-Najjar syndrome is more severe?
Why?
Type 1 because there is no UGT1A1 activity compared to Type II which has decreased activity
What levels are elevated with cholestasis?
Elevated serum γ-glutamyl transpeptidase (GGT) and alkaline phosphatase
What district morphology is seen in cholestasis?
Feathery degeneration of periportal hepatocytes
How does ascending cholangitis present?
Charcot’s triad: fever, RUQ pain, jaundice
What is characterized by repeated bouts of ascending cholangitis, progressive inflammatory destruction of hepatic parenchyma, and predisposes to biliary neoplasia?
Primary hepatolithiasis
Primary hepatolithiasis causes an increased risk of?
Cholangiocarcinoma
Panlobular giant-cell transformation of hepatocytes is seen with?
Neonatal hepatitis
What is the most common cause of neonatal cholestasis and death from liver disease in early childhood?
Biliary atresia
What stool change is seen with biliary atresia?
Acholic stools (pale)
How is biliary atresia limited to common duct (type I) or right/left hepatic ducts (type II) treated?
How is obstruction above the porta hepatis (type III) treated?
1) Kasai procedure
2) It can’t and instead needs liver transplant
What population is most affected by primary biliary cirrhosis?
Middle-aged women
What Abs are seen with primary biliary cirrhosis?
Anti-mitochondrial antibodies (anti-PDC-E2)
What happens to interlobular ducts with primary biliary cirrhosis?
Florid duct lesion
What is characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts with dilation of preserved segments?
What is seen on radiographs?
1) Primary sclerosing cholangitis (PSC)
2) Beading
How does primary sclerosing cholangitis affect the smaller ducts?
Onion skin fibrosis around an atrophic duct lumen that leads to obliteration by a tombstone scar
What levels are elevated with primary sclerosing cholangitis?
Serum alkaline phosphatase
Primary sclerosing cholangitis is diagnosed with radiology of the biliary tree that shows?
Larger ducts with strictures and beading with pruning of the smaller ducts
What are congenital dilations of the common bile duct?
Choledochal cysts
What are a group of different lesions in the liver due to congenital malformations of the biliary tree?
Fibropolycystic disease (FPD)
What is characterized by small bile duct hamartomas that are normal if limited, but indicative of FPD if they are diffuse?
Von Meyenburg complexes
What pathologic finding of FPD is characterized by biliary cysts in isolation that lead to clinical symptoms?
Caroli disease
What pathologic finding of FPD is characterized by portal tracts that are enlarged by irregular broad bands of collagenous tissue that form septa to divide the liver into irregular island?
Congenital hepatic fibrosis
What is the most common cause of intrahepatic blood flow obstruction?
Cirrhosis
What hepatic issue does left sided heart failure lead to?
Centrilobular fibrosis
What is characterized by obstruction of two or more major hepatic veins that leads to hepatomegaly, pain, and ascites?
Budd-Chiari syndrome
How does Budd-Chiari syndrome present?
1) Liver is swollen and red-purple
2) Centrilobular necrosis
Toxic injury to the sinusoidal endothelium can lead to?
Sinusoidal obstruction syndrome
How is Sinusoidal obstruction syndrome clinically diagnosed?
Tender hepatomegaly, ascites, weight gain, jaundice
When is vanashing bile duct syndrome seen?
Chronic transplant rejection
What is the most common cause of jaundice in pregnancy?
Viral hepatitis
What levels are increased with preeclampsia and eclampsia?
1) Serum aminotransferases
2) Serum bilirubin
How is acute fatty liver of pregnancy diagnosed on biopsy?
Diffuse microvesicular steatosis of hepatocytes
What levels are greatly increased with intrahepatic cholestasis of pregnancy?
Bile salt
What is characterized by the entire liver transformed into nodules; looks similar to focal nodular hyperplasia, but there is no fibrosis?
What can it lead to?
1) Nodular regenerative hyperplasia
2) Portal HTN
What morphologic changes are seen with nodular regenerative hyperplasia?
Plump hepatocytes surrounded by rims of atrophic hepatocytes
What is the most common benign liver tumor?
Cavernous hemangiomas
What benign neoplasms developing from hepatocytes may rupture leading to intraabdominal bleeding that is a surgical emergency?
What form of therapy are they associated with?
1) Hepatocellular adenomas
2) Oral contraceptives
Which type of hepatocellular adenoma has no risk of malignant transformation?
Which has a very high risk of malignant transformation?
1) HNF1-α inactivated adenomas
2) Β-Catenin activated adenomas
What is the most common liver tumor of early childhood?
Hepatoblastoma
What do hepatoblastoma frequently activate?
Due to what mutation?
This mutation causes what to precipitate the hepatoblastoma?
1) WNT pathway
2) APC mutation
3) FAP
What is the most common primary malignancy of hepatocytes?
Hepatocellular carcinoma
What is a strong genetic risk factor of hepatocellular carcinoma?
1) B-catenin activation
2) p53 inactivation (aflatoxin)
What is probably the most important pathway for emergence of hepatocellular carcinoma in viral hepatitis and alcoholic liver disease?
High-grade dysplastic nodules
What rare variant of hepatocellular carcinoma presents as a single large hard scirrhous tumor with fibrous bands coursing through it?
Fibrolamellar carcinoma
What cancer of the biliary tree is the second most common primary malignant tumor after HCC?
Cholangiocarcinoma
What is a strong risk factor for Cholangiocarcinoma?
Liver fluke parasites (Opisthorchis, clonorchis)
Where are the perihilar tumors (Klatskin tumors) of Cholangiocarcinoma most commonly located?
Junction of the hepatic ducts
Which form of cholangiocarcinoma presents as obstruction to bile flow or symptomatic liver mass?
Which presents as biliary obstruction, cholangitis, and RUQ pain?
1) Intrahepatic
2) Extrahepatic
Angiosarcomas are other primary hepatic malignant tumors that has historical associations with?
Vinyl chloride
More than 95% of biliary tract disease is attributable to?
Cholelithiasis
What causes cholelithiasis?
Stones of either cholesterol or pigment (bilirubin)
Bacterial contamination of the biliary tract by E. coli, Ascaris lumbricoides, or liver fluke C. Sinensis leads to release of?
This causes?
1) Beta-glucuronidases
2) Stones of unconjugated bilirubin
Where are black pigment stones found?
Where are brown pigment stones found?
1) Sterile bile ducts
2) Infected large bile ducts
How does cholelithiasis present?
Pain in RUQ that radiates to right upper shoulder or back
Which size of stones are considered more dangerous?
Small stones (gravel)
Acute calculous cholecystitis is almost always caused by a stone obstructing what area?
Neck or cystic duct
What is there a greater risk of in patients that have acalculous cholecystitis?
Gangrene and perforation
Chronic cholecystitis can lead to what morphologic change?
What does it cause?
1) Porcelain gallbladder
2) Calcification of the wall that increases the risk of developing cholangiocarcinoma
Chronic cholecystitis causes an intolerance for?
Fatty foods
What is the most common malignancy of the extrahepatic biliary tract?
Which sex is more affected?
What is the biggest risk factor for it?
1) Adenocarcinoma of the gallbladder
2) Women
3) Gallstones
