Pathologies Flashcards
What is cirrhosis?
What is the main cause?
Cirrhosis is the destruction of hepatocytes and replacement with scar tissue
The number one cause is alcohol abuse leading to steatohepatitis (fatty liver leading to inflammation and hepatocyte destruction)
What is primary biliary cirrhosis?
Cirrhosis leading to the destruction of bile ducts
What is portal hypertension?
What is the cause?
What are associated problems?
Portal hypertension is result of cirrhosis leading to resistance to portal flow
Associated with caput medusae, splenomegaly, esophageal varices, and hepatic encephalopathy
What is jaundice a result of?
Hyperbilirubinemia resulting in yellow discoloration of skin and sclera
What are 4 causes of jaundice due to elevated UNCONJUGATED bilirubin?
Hemolytic anemia
Heart failure
Gilbert syndrome
Crigler-najjar syndrome
What are 3 causes of jaundice due to elevated levels of CONJUGATED bilirubin?
Dubin-johnson syndrome
Rotor syndrome
Biliary tree obstruction
Why does hemolytic anemia lead to jaundice? Why are unconjugated levels elevated?
Hemolytic anemia leads to an increase in bilirubin that passes the livers capacity to conjugate it resulting in elevated levels of unconjugated bilirubin
What is Gilbert syndrome?
Mild hyperbilirubinemia due to hereditary defect in UDP-glucotransferase resulting in elevated levels of unconjugated bilirubin. Not very serious
What is Crigler-Najjar syndrome?
What is the cause?
Which type is more severe?
Crigler-najjar syndrome is a hereditary, non-hemolytic, hyperbilirubinemia caused by a mutation to the gene coding for UDP-glucotransferase
Type 1 is much more severe than type 2
What is type 1 crigler-najjar syndrome?
When does it begin?
What is it associated with?
Type 1- gene for UDP-glucotransferase is ABSENT
Begins early in life and is often fatal
Associated with kernicterus (bilirubin induced neurologic dysfunction where unconjugated bilirubin accumulates in brain)
What is type 2 crigler-najjar syndrome?
When does it begin in life?
Type 2 is due to low levels of function UDP-glucotransferase
Begins later in life
What are treatments for crigler-najjar syndrome?
Specific to type 1?
Specific to type 2?
Phototherapy and blood transfusion
Type 1- liver transplant
Type 2- phenobarbital
What is Dubin-johnson syndrome?
What mutation is associated with it?
How does it affect the liver?
Dubin-johnson is a hereditary hyperbilirubinemia associated with elevated levels of CONJUGATED bilirubin due to a defective MRP2 transporter (transport out of bile canaliculi)
Liver will have black pigmentation
What is Rotor syndrome?
What is the defect?
How does it present differently than Dubin-Johnson?
Rotor syndrome is a hereditary hyperbilirubinemia as result of mutated OATP transporter which transports bile from blood into liver
Leads to buildup of conjugated > unconjugated bilirubin
Liver does not have black pigmentation
What are causes of gallstones?
Excess in pigment or excessive bilirubin/cholesterol breakdown
Too much water absorbed from bile
Too much bile acid absorbed from bile
Too much cholesterol in bile
