11.3 Liver Flashcards
Jaundice
Yellow discoloration of the skin due to increased serum bilirubin
What is the breakdown of heme?
Heme -> Porphyrin -> Unconjugated bilirubin -> Conjugated bilirubin
Jaundice in Extravascular Hemolysis
Increased UCB which overwhelms the conjugating ability of the liver
Jaundice in the Newborn
Newborn liver has a low UGT activity to conjugate bilirubin - increased UCB
Jaundice in Gilbert Syndrome
Lowered UGT activity due to an autosomal recessive mutation - but are usually asymptomatic with increased UCB levels
Jaundice in Crigler-Najjar Syndrome
Absence of UGT - causes increased UCB leading to kernicterus and is usually fatal
Jaundice in Dubin-Johnson Syndrome
Autosomal recessive deficiency of bilirubin canalicular transport protein - increased CB with a pitch dark black liver
Jaundice in Biliary Tract Obstrction
Blockage of bile release leading to increased CB/decreased urine urobilinogen/increased alkaline phosphatase
Jaundice in Viral Hepatitis
Inflammation disrupts hepatocytes and small bile ductules - increase in BOTH CB and UCB
What are 2 consequences of extravascular hemolysis?
- Dark urine
- Increased risk of bilirubin gallstones
What is a serious consequence of newborn jaundice?
UCB is fat soluble and can deposit in the basal ganglia, causing kernicterus - neurological deficits
What is Rotor Syndrome?
It has the same signs as Dubin-Johnson Syndrome but without the liver discoloration
What are some of the signs of biliary tract obstruction?
- Dark urine
- Pruritis
- Hypercholesterolemia
- Steatorrhea
Viral Hepatitis
Inflammation of the liver parenchyma usually from hepatitis virus
What are non-hepatitis viruses that can cause hepatitis?
EBV and CMV
What does chronic hepatitis tend to involve?
Portal Tract
Transmission of HAV
Travelers via fecal-oral transmission
Transmission of HBV
Parenteral transmission via childbirth, sex, IV drugs
Transmission of HCV
Parenteral transmission especially with blood transfusion
Transmission of HEV
Contaminated water or undercooked seafood
What does HEV in a pregnant woman indicate?
Fulminant Hepatitis
How does HDV infection occur?
It is dependent on HBV for infection
What is the first serologic marker for HBV infection with acute hepatitis?
HBsAG
What are the protective Ab against HBV?
- HBcAB
- HBsAB
What Ag of HBV indicates infectivviy?
HBeAG
What type of hepatitis most often leads to chronic hepatitis?
HCV
What are the 2 mechanisms of HDV infection?
- Superinfection (on an existing HBV infection) -> MORE SEVERE
- Coinfections
Cirrhosis
Disruption of normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes
What is the key mediator of fibrosis seen in cirrhosis?
Stellate cells. They release TGF-b to mediate this.
What is the main consequence of cirrhosis?
Portal HTN
Alcohol-Related Liver Disease
Damage to the hepatic parenchyma due to consumption of alcohol - most common cause of liver disease in the West
What are some of the clinical signs of alcohol-related liver disease?
- Fatty liver
- Alcoholic hepatitis
- Cirrhosis
Alcoholic Hepatitis
Direct damage to hepatocytes often seen with bing drinking
What is the mediator of the damage in alcoholic hepatitis?
Acetaldehyde
What are Mallory bodies?
Damaged intermediate filaments in the hepatocytes
What pattern of enzymes will be seen with alcoholic hepatitis?
AST is greater than ALT - AST is in the mitochondria
Nonalcoholic Fatty Liver Disease (NAFLD)
Same signs as in the alcoholic version, but WITHOUT exposure to alcohol
- Fatty liver
- Hepatitis
- Cirrhosis
What is NAFLD associated with?
Obesity
Hemochromatosis
Excess body iron deposition that can cause cirrhosis - damage from the generation of free radicals - often due to HFE mutation
What does the presence of lipfuscin in cells indicate?
Aged though wear and tear
Wilson Disease
Autosomal recessive mutaiton in ATP7B gene in ATP-mediated Cu transport - Cu builds up in the hepatocyes
When will hemochromatosis present? What about Wilson?
Hemochromatosis - Late young adulthood
Wilson - Childhood
What is the treatment of Wilson disease?
D-penicillamine
Primary Biliary Cirrhosis
Autoimmune granulomatous destruction of intrahepatic bile ducts
Who is the classic patient of primary biliary cirrhosis?
Middle aged women
What Ab will be seen in primary biliary cirrhosis?
Anti-mitochondrial Ab
Primary Sclerosis Cholangitis
Inflammation and fibrosis of intrahepatic and extra hepatic bile ducts
What is the appearance of primary sclerosing cholangitis on imaging?
“Beaded” in uninvolved regions which are dilated
What is primary sclerosis cholangitis associated with?
- Ulcerative coititis
- p-ANCA is often positive
Reye Syndrome
Fulminant liver failure and encephalopathy in children with viral illness who take aspirin
Hepatic Adenoma
Benign tumor of hepatocytes that is associated with oral contraceptives
What is a serum tumor marker for hepatocellular carcinoma?
Alpha-fetoprotein
