Biliary syndromes and HCC

Question 1

primary biliary cirrhosis: pathology/pathophysiology and classic patient; presentation, labs, additional information.

Answer 1

autoimmune granulomatous destruction of intrahepatic bile ducts. lymphocyte infiltrate is also present. causes destruction of the intralobar bile ducts. cirrhosis is a late complication.
classically arises in women (avg age is 40).
associated with other autoimmune conditions.
presentation: obstructive jaundice: dark urine, pruritis, light stools, hepatosplenomegaly
labs: incr. CB, incr. cholesterol, incr. ALP
notes: antimitochondrial antibody is present (important!)

Question 2

primary sclerosing cholangitis: pathology/pathophysiology and classic patient; presentation, labs, additional information.

Answer 2

pathology: inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. causes a periductal fibrosis with an onion-skinning appearance.
uninvolved regions are dilated, which causes a beaded appearance of intra- and extra-hepatic bile ducts on ERCP
presentation: obstructive jaundice (pruritis, dark urine, pale stools, jaundice, hepatosplenomegaly). may progress to cirrhosis
notes: this is associated with ulcerative colitis, and the p-ANCA may be positive! incr. risk of cholangiocarcinoma and cirrhosis.
(labs are the same: incr. ALP, incr. Conjugated bilirubine (CB), incr. cholesterol)

Question 3

secondary biliary cirrhosis: pathology/pathophysiology and classic patient; presentation, labs, additional information.

Answer 3

pathology: extrahepatic biliary obstruction like gallstones, biliary stricture, chronic pancreatitis, carcinoma of the pancreatic head causes incr. pressure in the intrahepatic ducts which cause injury, fibrosis, and bile stasis.
presents with symptoms of obstructive jaundice: pruritis, jaundice, hepatosplenomegaly, pale stools, darke urine
labs: incr. CB, incr. cholesterol, incr ALP
notes: may be complicated by ascending cholangitis

Question 4

Reye syndrome: WHAT IS IT? How does it present?

Answer 4

fulminant liver failure and encephalopathy in kids with a viral illness (esp. VZV and influenza B) who take ASA. we know there is mitochondrial damage of hepatocytes.
pts have HYPOGLYCEMIA, elevated liver enzymes, and nausea with vomiting. may progress to fatal hepatoencephalopathy
(remember, you would give a child ASA for Kawasaki disease, which is a vasculitis).

Question 5

alpha-1 antitrypsin deficiency

Answer 5

misfolded gene produce protein aggregates in hepatocellular ER and causes cirrhosis with PAS positive globules in the liver. codominant trait. in luncs, it can cause panacinar emphysema.

Question 6

hepatic adenocarcinoma: what is it, causes, and complications

Answer 6

benign tumor of hepatocytes associated with oral contraceptives and steroids. risk of rupture and intraperitoneal hemorrhage, esp. during pregnancy (high levels of estrogen). often subcapsular
regresses upon cessation of OCPs.

Question 7

carvernous hemangioma: what is it, complications

Answer 7

common, benign liver tumor that typically presents btw 30-50 yrs of age. Do NOT biopsy because of risk of hemorrhage.

Question 8

angiosarcoma: what is it, causes

Answer 8

malignant tumor of endothelial origin. associated with exposure to arsenic or vinyl chloride.

Question 9

nutmeg liver: what is it, causes, complications

Answer 9

due to backup of blood into the liver. commonly cuased by right sided heart failure or budd-chiari syndrome. liver looks like a nutmeg. if it persists, centrilobar congestion and necrosis can cause cardiac cirrhosis.

Question 10

budd-chiari syndrome

Answer 10

occlusion of IVC of hepatic veins with centrilobar congestion and necrosis, leading to congestive liver disease (hepatomegaly, ascites, abdominal pain, and eventual liver failure). may develop varices and have visible abdominal and back veins. no JVD. associated with hypercoagulable states, like polycythemia vera, pregnangy, and or with compression of the the hepatic veins, as may be seen with HCC.

Question 11

What are the risk factors for HCC?

Answer 11

cirrhosis (nonalcoholic fatty liver, alpha-1 antitrypsin, hemochromatosis, wilson disease, alcohol), chronic hepatitis, or aflatoxins derived from aspergillus

Question 12

What are common complications of HCC? presentation? tumor marker?

Answer 12

incr. risk for budd-chiari syndrome (liver infarction secondary to hepatic vein obstruction). presents with painful hepatomegaly and ascities.
alpha-fetoprotein is a marker.

Question 13

Whare are important sources of mets to the liver? How is it detected clinically?

Answer 13

colon, pancreas, lung and breast. casues multiple nodules in the liver that presents as hepatomegaly with nodular free edge of liver