Liver part two...cirrhosis Flashcards
What is cirrhosis? What is the typical cause of cirrhosis?
Cirrhosis is widely diffuse interconnecting fibrosis scars with nodular parenchymal regeneration
Cirrhosis is usually caused by alcohol
How does cirrhosis cause death?
Progressive liver failure
Complications of portal hypertension
Hepatocellular carcinoma
What are three causes of hepatic failure?
Chronic liver disease (like cirrhosis)
Acute liver failure w/massive liver necrosis (viral, drug, toxin)
Hepatic dysfunction w/o overt necrosis (Reye’s syndrome, acute fatty liver of pregnancy)
What is the most common cause of portal hypertension?
Cirrhosis
What are the 4 main complications of portal hypertension?
Ascites
Portosystemic shunts
Splenomegaly
Hepatic encephalopathy
What is seen on an alcoholic steatosis biopsy?
Accumulation of lipid
Macrovesicular steatosis (microscopically)
What is seen on an alcoholic hepatitis biopsy?
Steatosis
Hepatocyte injury and/or inflammation
What are mallory bodies? What are they seen with?
Mallory bodies are cytokeratin
Mallory bodies are seen with alcoholic liver disease, NAFLD, and PBC
What are the gross findings of alcoholic cirrhosis?
Bridging fibrosis
Intervening hepatocytes regenerate –> nodules
What are the 5 major causes of death with alcoholic cirrhosis?
Hepatic encephalopathy --> coma Massive GI tract hemorrhage Infection Hepatorenal syndrome Hepatocellular carcinoma
Who is at risk for Non-Alcoholic Fatty Liver Disease (NAFLD)?
Patients with obesity, dyslipidemia, hyperinsulinemia, and/or insulin resistance (DMII)
What is Primary Biliary Cirrhosis?
PBC is progressive inflammatory destruction of intrahepatic bile ducts
Who usually develops PBC?
40-50yo females
May have an autoimmune disorder
What are the primary symptoms of PBC?
Insidious onset of fatigue
Anicteric pruritis
What are some key diagnostic labs for PBC?
Elevated ALP and cholesterol
AMA+
What is seen on liver biopsy of PBC?
Periportal hepatitis and periportal fibrosis –> bridging necrosis with bridging fibrosis –> cirrhosis
What is Secondary Biliary Cirrhosis?
Cirrhosis secondary to any disorder causing extra hepatic bile duct obstruction
What are 5 causes of Secondary Biliary Cirrhosis?
Stones Tumors Biliary atresia CF Choledochal cysts
What is Primary Sclerosing Cholangitis?
Progressive, random, uneven fibroinflammatory obliteration of extra hepatic and intrahepatic bile ducts
What other GI disorder is usually seen with PSC?
80% of PSC cases are associated with IBD…usually ulcerative colitis (pANCA)
How can PSC be diagnosed?
pANCA (which is why it is with ulcerative colitis)
Elevated ALP
Biopsy
What is seen on a biopsy of PSC?
Fibrosing cholangitis –> beads on a string
Obliterated bile duct
What is Hemochromatosis?
Iron overload
What are the manifestations of Hemochromatosis?
Iron accumulates in the liver
Hemosiderin deposits in pancreas, heart, skin, endo, joints
What is seen on liver biopsy of Hemochromatosis?
Periportal iron deposits
Micronodular cirrhosis
How is Hemochromatosis diagnosed?
Liver biopsy…perform quantitative iron in paraffin block (I don’t actually know what this means…)
How are people screened for Hemochromatosis?
Fasting transferrin saturation -(if +)-> repeat transferrin saturate with serum ferritin -(if +)-> HFE gene test
How is Hemochromatosis treated?
Phlebotomy
Iron chelating agents
What is Wilson’s disease?
Autosomal recessive disorder of copper metabolism (ATP7B in liver)
Impaired secretion of copper into bile
How is Wilson’s disease diagnosed?
Serum ceruloplasmin (low…unless also liver disease, then normal)
24hr urine copper is elevated
Serum copper levels are low
When should Wilson’s disease be considered?
Liver disease < 30yo
What is seen on liver biopsy of Wilson’s disease?
Increased hepatic copper
If negative, do a quantitative liver copper
What can be seen in the eyes of a patient with Wilson’s disease?
Kayser-Fleischer rings
What is Alpha-1-Antitrypsin (A1AT) deficiency?
Co-dominant autosomal disorder characterized by abnormally low levels of A1AT
A1AT is produced by the liver
What is the effect of A1AT deficiency in the lungs? Liver?
Lungs: empysema (neutrophil elastase»_space; A1AT)
Liver: Accumulation of A1AT in hepatocytes
How is A1AT deficiency screened for?
A1AT level and genotype
What is seen on a liver biopsy of A1AT deficiency?
Cytoplasmic globular inclusions
There are two groups of DRUG induced liver injury: what are the two groups?
Direct (all who are exposed are effected…tylenol)
Indirect (not all who are exposed are affected)
What is Reye’s syndrome?
Liver injury (microvesicular steatosis) and encephalopathy post-viral illness…usually after aspirin treatment
How can Reye’s syndrome be avoided?
Don’t give aspirin to febrile kids
What is seen on liver biopsy with Reye’s syndrome?
Microvesicular steatosis with small lipid vacuoles within the hepatocytes
What is neonatal cholestasis? What are two examples?
Group of disorders characterized by prolonged conjugated hyperbilirubinemia in neonates
Biliary atresia and neonatal hepatitis
What is biliary atresia? (besides a cause of neonatal cholestasis)
Exactly what it sounds like… complete/partial obstruction of lumen of the extra hepatic biliary tree
Noticed within first 3mos. of life
What is neonatal hepatitis? (besides a cause of neonatal cholestasis)
Hepatitis occurring in early infancy (1-2mos.)
What 4 things can possibly cause neonatal hepatitis?
Biliary atresia
Inherited metabolic disorders (A1AT d, tyrosinemia, CF)
Infectious agents
Drugs
What are the 4 main causes of granulomatous hepatitis?
Idiopathic
Sarcoidosis
Drugs
TB
Why are AST and ALT measured?
Elevations reflect hepatocellular damage
NOT specific to liver
What do ALP and GGT assess?
Reflect injury to the bile duct epithelium/canalicular membrane
Marker for cholestasis
What do albumin, PT, and certain clotting factors indicate about the liver?
General hepatic synthesis (function)
How are quantitative liver function tests done?
Measure hepatic metabolism of a drug to determine liver function
What are the key findings of chronic liver diseases?
Elevation of AST and ALT is greater than the bilirubin elevation
What are the key findings of biliary tract diseases?
Elevation of bilirubin is greater than elevation of AST and ALT
