HEPATOBILIARY Flashcards
Front
Back
What are centrilobular and periportal hepatocytes?
Centrilobular hepatocytes are near the terminal hepatic vein, while periportal hepatocytes are near the portal tract.
What is the significance of dividing the liver lobule into zones?
Each zone has different metabolic activities and susceptibilities to hepatic injury.
What is biliary atresia?
A developmental anomaly or inflammatory destruction of bile ducts, leading to cholestatic jaundice and ESLD in children.
What are the clinical features of biliary atresia?
Cholestatic jaundice at 1 week of life, enlarged dark green liver, and absence of bile.
What are the features of choledochal cyst?
Cystic dilatation of the biliary tree; may involve fibrosis and risk of cholangiocarcinoma.
What is neonatal jaundice?
Transient unconjugated hyperbilirubinemia in newborns due to immature conjugation machinery.
What are the types of jaundice based on bilirubin?
Unconjugated (e.g., hemolysis, low UGT) and conjugated (e.g., biliary obstruction, Dubin-Johnson syndrome).
What is Crigler-Najjar syndrome?
A hereditary condition with severe (type 1) or mild (type 2) UGT deficiency, leading to unconjugated hyperbilirubinemia.
What is Dubin-Johnson syndrome?
An AR disorder with defective canalicular bilirubin transport, causing conjugated hyperbilirubinemia and dark liver.
What causes viral hepatitis?
Hepatitis viruses A, B, C, D, and E; other viruses like EBV, CMV, and yellow fever.
What are the stages of HBV serology?
Acute (HBsAg, HBeAg, anti-HBc IgM), Window (anti-HBc IgM), Resolved (anti-HBc IgG, anti-HBs), Chronic (HBsAg > 6 months).
What is fulminant hepatic failure?
Acute liver failure with encephalopathy, seen in severe cases of viral hepatitis (e.g., HAV, HBV, HEV).
What is the risk of chronicity in hepatitis B and C?
HBV: 20% chronic; HCV: 75-85% chronic, with cirrhosis in 10-15% within 20 years.
What is the pathogenesis of HBV?
HBV targets hepatocytes, with immune-mediated destruction by cytotoxic lymphocytes reacting to HBsAg and HBeAg.
What is Wilson disease?
An AR defect in ATP7B gene causing copper accumulation in hepatocytes, leading to cirrhosis, neurological symptoms, and Kayser-Fleischer rings.
What is hemochromatosis?
Excessive iron deposition due to HFE gene mutations, causing cirrhosis, diabetes, bronze skin, and increased HCC risk.
What are the clinical features of cirrhosis?
Portal hypertension, hepatic encephalopathy, jaundice, hypoalbuminemia, coagulopathy, and ascites.
What is alcoholic liver disease?
Hepatic damage from alcohol, ranging from fatty liver (reversible) to alcoholic hepatitis (AST > ALT) to cirrhosis.
What is non-alcoholic fatty liver disease (NAFLD)?
Fatty liver, hepatitis, or cirrhosis without alcohol exposure, commonly associated with obesity.
What is the treatment for Wilson disease?
D-penicillamine, which chelates copper.
What is the treatment for hemochromatosis?
Phlebotomy to reduce iron overload.
What are Kayser-Fleischer rings?
Copper deposits in the cornea seen in Wilson disease.
What are the complications of biliary obstruction?
Pruritus, pale stool, dark urine, fat malabsorption, and vitamin deficiencies.
What is the role of stellate cells in cirrhosis?
They produce TGF-β, leading to fibrosis in liver damage.
What is the classic triad of hemochromatosis?
Cirrhosis, diabetes mellitus, and bronze skin.
Front
Back
What are centrilobular and periportal hepatocytes?
Centrilobular hepatocytes are near the terminal hepatic vein, while periportal hepatocytes are near the portal tract.
What is biliary atresia?
A developmental anomaly causing cholestatic jaundice and ESLD in children due to inflammatory destruction of bile ducts.
What are the clinical features of biliary atresia?
Cholestatic jaundice within 1 week of life, enlarged dark green liver, and bile duct fibrosis.
What are the microscopic features of intrahepatic biliary atresia?
Few bile ducts, cholestasis, periportal inflammation, and late-stage cirrhosis.
What is a choledochal cyst?
Cystic dilatation of the biliary tree, which may lead to fibrosis and cholangiocarcinoma.
What is Caroli disease?
Dilation of large intrahepatic bile ducts with complications like cholangitis, sepsis, and cholelithiasis.
What is Alagille syndrome?
An autosomal dominant disorder due to JAG1 and Notch2 mutations causing reduced bile ducts.
What causes jaundice?
Retention of bilirubin due to increased production, hepatocyte dysfunction, or bile flow obstruction.
What is neonatal jaundice?
Transient unconjugated hyperbilirubinemia in newborns due to immature bilirubin metabolism.
What are the classifications of jaundice?
Predominantly unconjugated (e.g., hemolysis, low UGT) or conjugated (e.g., biliary obstruction, Dubin-Johnson syndrome).
What is Crigler-Najjar syndrome?
A hereditary condition with severe (type 1) or mild (type 2) UGT deficiency causing unconjugated hyperbilirubinemia.
What is Dubin-Johnson syndrome?
An autosomal recessive disorder with defective bilirubin transport, causing conjugated hyperbilirubinemia and dark liver.
What is viral hepatitis?
Inflammation of the liver caused by hepatitis viruses (A, B, C, D, E) or other infections (e.g., CMV, EBV).
What is fulminant hepatic failure?
Acute liver failure with encephalopathy, often fatal, seen in severe viral hepatitis cases.
What are the chronic risks of hepatitis B and C?
HBV: Chronic in 20%, HCC risk. HCV: Chronic in 75-85%, cirrhosis in 10-15% within 20 years.
What is HAV’s mode of transmission?
Fecal-oral, often from contaminated water or food, with no chronic state.
What is HBV’s mode of transmission?
Parenteral, including childbirth, unprotected sex, and IV drug use.
What is the significance of HBsAg in HBV?
Indicates active infection and blood infectivity.
What is HCV’s progression risk?
Chronic disease in most cases, with high risk of cirrhosis and need for liver transplantation.
What is unique about HDV?
It only co-infects with HBV, leading to more severe disease, including cirrhosis and liver cancer.
What is the mode of transmission of HEV?
Fecal-oral, especially in developing countries; severe in pregnant women.
What are Kayser-Fleischer rings?
Copper deposits in the cornea, seen in Wilson disease.
What is Wilson disease?
An autosomal recessive disorder of copper metabolism causing cirrhosis, neurological symptoms, and corneal rings.
What are the features of hemochromatosis?
Iron overload causing cirrhosis, diabetes, bronze skin, and increased HCC risk.
What is alcoholic hepatitis?
Liver inflammation from alcohol, characterized by Mallory bodies, AST > ALT, and hepatocyte damage.
What are the complications of cirrhosis?
Portal hypertension, ascites, hepatic encephalopathy, jaundice, and coagulopathy.
What are the treatments for Wilson disease and hemochromatosis?
Wilson disease: D-penicillamine. Hemochromatosis: Phlebotomy.
Front
Back
What is primary biliary cirrhosis (PBC)?
An autoimmune granulomatous destruction of intrahepatic bile ducts, commonly in women around age 40.
What is primary biliary cirrhosis associated with?
Other autoimmune diseases; antimitochondrial antibodies are present.
What are the clinical features of primary biliary cirrhosis?
Obstructive jaundice with cirrhosis as a late complication.
What is primary sclerosing cholangitis (PSC)?
Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts.
What is the characteristic histological feature of PSC?
Periductal fibrosis with an ‘onion-skin’ appearance.
What imaging findings are associated with PSC?
Dilated, uninvolved bile ducts causing a ‘beaded’ appearance on contrast imaging.
What conditions are associated with PSC?
Ulcerative colitis; p-ANCA is often positive.
What are the complications of PSC?
Cirrhosis and increased risk of cholangiocarcinoma.
What is Reye syndrome?
Fulminant liver failure and encephalopathy in children with viral illness who take aspirin.
What causes Reye syndrome?
Likely mitochondrial damage to hepatocytes caused by aspirin use.
What are the clinical features of Reye syndrome?
Hypoglycemia, elevated liver enzymes, vomiting, and potential progression to coma and death.
What is hepatic adenoma?
A benign tumor of hepatocytes, often associated with oral contraceptive use.
What are the complications of hepatic adenoma?
Risk of rupture and intraperitoneal bleeding, especially during pregnancy.
What is hepatocellular carcinoma (HCC)?
A malignant tumor of hepatocytes.
What are the risk factors for HCC?
Chronic hepatitis (HBV, HCV), cirrhosis (alcohol, NAFLD, hemochromatosis, Wilson disease, A1AT deficiency), and aflatoxins.
What is Budd-Chiari syndrome?
Liver infarction secondary to hepatic vein obstruction, presenting with painful hepatomegaly and ascites.
What are the clinical challenges of diagnosing HCC?
Symptoms are often masked by underlying cirrhosis, leading to late detection.
What is the serum tumor marker for HCC?
Alpha-fetoprotein (AFP).
What is more common than primary liver tumors?
Metastasis to the liver, most commonly from colon, pancreas, lung, and breast cancers.
What is a common presentation of liver metastasis?
Multiple liver nodules with hepatomegaly and a nodular free edge.
Front
Back
What is primary biliary cirrhosis (PBC)?
An autoimmune granulomatous destruction of intrahepatic bile ducts, commonly in women around age 40.
What is primary biliary cirrhosis associated with?
Other autoimmune diseases; antimitochondrial antibodies are present.
What are the clinical features of primary biliary cirrhosis?
Obstructive jaundice with cirrhosis as a late complication.
What is primary sclerosing cholangitis (PSC)?
Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts.
What is the characteristic histological feature of PSC?
Periductal fibrosis with an ‘onion-skin’ appearance.
What imaging findings are associated with PSC?
Dilated, uninvolved bile ducts causing a ‘beaded’ appearance on contrast imaging.
What conditions are associated with PSC?
Ulcerative colitis; p-ANCA is often positive.
What are the complications of PSC?
Cirrhosis and increased risk of cholangiocarcinoma.
What is Reye syndrome?
Fulminant liver failure and encephalopathy in children with viral illness who take aspirin.
What causes Reye syndrome?
Likely mitochondrial damage to hepatocytes caused by aspirin use.
What are the clinical features of Reye syndrome?
Hypoglycemia, elevated liver enzymes, vomiting, and potential progression to coma and death.
What is hepatic adenoma?
A benign tumor of hepatocytes, often associated with oral contraceptive use.
What are the complications of hepatic adenoma?
Risk of rupture and intraperitoneal bleeding, especially during pregnancy.
What is hepatocellular carcinoma (HCC)?
A malignant tumor of hepatocytes.
What are the risk factors for HCC?
Chronic hepatitis (HBV, HCV), cirrhosis (alcohol, NAFLD, hemochromatosis, Wilson disease, A1AT deficiency), and aflatoxins.
What is Budd-Chiari syndrome?
Liver infarction secondary to hepatic vein obstruction, presenting with painful hepatomegaly and ascites.
What are the clinical challenges of diagnosing HCC?
Symptoms are often masked by underlying cirrhosis, leading to late detection.
What is the serum tumor marker for HCC?
Alpha-fetoprotein (AFP).
What is more common than primary liver tumors?
Metastasis to the liver, most commonly from colon, pancreas, lung, and breast cancers.
What is a common presentation of liver metastasis?
Multiple liver nodules with hepatomegaly and a nodular free edge.
Front
Back
What is cirrhosis?
Diffuse liver injury with transformation into regenerative nodules surrounded by fibrous bands, associated with chronic liver disease.
What are the main causes of cirrhosis?
Chronic hepatitis B, chronic hepatitis C, non-alcoholic fatty liver disease (NAFLD), and alcoholic liver disease.
What is the pathogenesis of cirrhosis?
Hepatocyte injury leads to necrosis/apoptosis, activation of stellate cells into fibrogenic myofibroblasts, and formation of fibrous septae.
What are ductular reactions in cirrhosis?
Stem cell-mediated duct-like structures seen in chronic liver disease and cirrhosis.
What is the morphology of cirrhosis?
Diffuse nodular transformation of the liver with regenerative nodules surrounded by fibrous bands.
What are the types of primary liver tumors?
Hepatocellular carcinoma (HCC), cholangiocarcinomas, hepatoblastoma, and angiosarcoma.
What is the most common primary liver tumor?
Hepatocellular carcinoma (HCC).
What are the risk factors for hepatocellular carcinoma?
Chronic hepatitis B and C, cirrhosis, alcohol, aflatoxins, and metabolic disorders like hemochromatosis.
What are the most common genetic mutations in HCC?
Gain-of-function mutations in beta-catenin and loss-of-function mutations in p53.
What is the morphology of HCC?
Can appear as unifocal masses, multifocal nodules, or diffusely infiltrative cancer.
What is cholelithiasis?
The presence of gallstones, often associated with the ‘4Fs’: fat, female, fertile, and forty.
What are the types of gallstones?
Pure (cholesterol, pigment, calcium carbonate), mixed, and combined gallstones.
What is the pathogenesis of cholesterol gallstones?
Supersaturation of bile with cholesterol, gallbladder stasis, and mucus hypersecretion leading to crystal aggregation.
What causes pigment stones?
High unconjugated bilirubin in bile due to hemolysis or biliary infections, forming calcium bilirubinate salts.
What are the characteristics of cholesterol stones?
Yellow, radiolucent stones found in the gallbladder, sometimes with calcium carbonate making them radiopaque.
What is the difference between black and brown pigment stones?
Black stones form in sterile bile, while brown stones form in infected intrahepatic or extrahepatic bile ducts.
What are common benign gallbladder tumors?
Adenomyoma (most common), adenoma, lipoma, fibroma, and hemangioma.
What is the most common malignant gallbladder tumor?
Adenocarcinoma, accounting for 90% of malignant cases.
What is the pathogenesis of pigment gallstones?
Increased unconjugated bilirubin in bile leads to calcium bilirubinate precipitates, forming black or brown stones.
What is the role of aflatoxins in HCC?
Aflatoxins induce TP53 mutations, increasing the risk of hepatocellular carcinoma.
Front
Back
What is the cause of jaundice in paravascular hemolysis or ineffective erythropoiesis?
High levels of unconjugated bilirubin (UCB) overwhelm the liver’s conjugating ability.
What are the laboratory findings in paravascular hemolysis or ineffective erythropoiesis?
Increased UCB and dark urine due to increased urine urobilinogen (UCB is not water soluble).
What is the risk associated with paravascular hemolysis?
Increased risk for pigmented bilirubin gallstones.
What causes physiologic jaundice of the newborn?
Transiently low UGT activity in the newborn liver.
What are the clinical features of physiologic jaundice of the newborn?
Increased UCB, risk of kernicterus (UCB deposits in basal ganglia), neurological deficits, and death.
What is the treatment for physiologic jaundice of the newborn?
Phototherapy, which makes UCB water-soluble.
What is Gilbert syndrome?
An autosomal recessive condition with mildly low UGT activity, causing increased UCB.
When does jaundice occur in Gilbert syndrome?
During periods of stress (e.g., severe infection); otherwise, it is not clinically significant.
What is Crigler-Najjar syndrome?
An autosomal recessive disorder with an absence of UGT, leading to severe UCB elevation.
What are the clinical features of Crigler-Najjar syndrome?
Kernicterus, which is usually fatal.
What is Dubin-Johnson syndrome?
An autosomal recessive disorder with a deficiency in bilirubin canalicular transport protein, causing increased conjugated bilirubin (CB).
What is a characteristic feature of Dubin-Johnson syndrome?
Darkly pigmented liver, but it is otherwise not clinically significant.
What is Rotor syndrome?
Similar to Dubin-Johnson syndrome but lacks liver discoloration.
What causes obstructive jaundice?
Biliary tract obstruction due to gallstones, pancreatic carcinoma, cholangiocarcinoma, parasites, or liver flukes (e.g., Clonorchis sinensis).
What are the laboratory findings in obstructive jaundice?
Increased CB, decreased urine urobilinogen, and increased alkaline phosphatase.
What are the clinical features of obstructive jaundice?
Dark urine (bilirubinuria), pale stools, pruritus (due to increased plasma bile acids), hypercholesterolemia with xanthomas, and steatorrhea.
What causes jaundice in viral hepatitis?
Inflammation disrupting hepatocytes and small bile ductules.
What are the laboratory findings in viral hepatitis?
Increased CB and UCB.
What are the clinical features of viral hepatitis?
Dark urine due to increased urine bilirubin, with normal or decreased urine urobilinogen.
Front
Back
What is the transmission route for Hepatitis A (HAV) and Hepatitis E (HEV)?
Fecal-oral transmission.
What are common sources of HAV and HEV infection?
HAV: Travelers; HEV: Contaminated water or undercooked seafood.
Do HAV and HEV cause chronic hepatitis?
No, they only cause acute hepatitis.
What serologic markers indicate active HAV or HEV infection?
Anti-virus IgM indicates active infection.
What does the presence of Anti-HAV IgG indicate?
Prior infection or immunization (available for HAV only).
What is HEV infection in pregnancy associated with?
Fulminant hepatitis (liver failure with massive necrosis).
What is the transmission route for Hepatitis B (HBV)?
Parenteral (e.g., childbirth, unprotected intercourse, intravenous drug abuse).
What is the chronicity rate of HBV infection?
Chronic disease occurs in 20% of cases.
What is the transmission route for Hepatitis C (HCV)?
Parenteral (e.g., intravenous drug abuse, unprotected intercourse, needle stick).
What is the risk of transfusion-related HCV infection?
Almost nonexistent due to blood supply screening.
What is the chronicity rate of HCV infection?
Chronic disease occurs in most cases.
What test confirms HCV infection?
HCV-RNA test.
What indicates recovery from HCV?
Decreased HCV-RNA levels.
What is required for Hepatitis D (HDV) infection?
HBV infection is necessary for HDV replication.
Which is more severe: HDV superinfection or coinfection?
Superinfection upon existing HBV is more severe.
What is the first serologic marker to rise in acute HBV infection?
HBsAg.
What indicates active replication and infectivity in HBV?
HBeAg or HBV-DNA presence.
What serologic markers are present in the window phase of HBV?
Anti-HBc IgM.
What serologic markers are present in resolved HBV infection?
Anti-HBc IgG and Anti-HBs IgG (protective).
What defines chronic HBV infection?
HBsAg persistence for more than 6 months.
What serologic marker is seen in individuals vaccinated against HBV?
Anti-HBs IgG (protective).
