11.3 Liver Flashcards by Kevin N

Jaundice

Yellow discoloration of the skin due to increased serum bilirubin

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What is the breakdown of heme?

Heme -> Porphyrin -> Unconjugated bilirubin -> Conjugated bilirubin

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Jaundice in Extravascular Hemolysis

Increased UCB which overwhelms the conjugating ability of the liver

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Jaundice in the Newborn

Newborn liver has a low UGT activity to conjugate bilirubin - increased UCB

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Jaundice in Gilbert Syndrome

Lowered UGT activity due to an autosomal recessive mutation - but are usually asymptomatic with increased UCB levels

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Jaundice in Crigler-Najjar Syndrome

Absence of UGT - causes increased UCB leading to kernicterus and is usually fatal

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Jaundice in Dubin-Johnson Syndrome

Autosomal recessive deficiency of bilirubin canalicular transport protein - increased CB with a pitch dark black liver

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Jaundice in Biliary Tract Obstrction

Blockage of bile release leading to increased CB/decreased urine urobilinogen/increased alkaline phosphatase

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Jaundice in Viral Hepatitis

Inflammation disrupts hepatocytes and small bile ductules - increase in BOTH CB and UCB

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What are 2 consequences of extravascular hemolysis?

Dark urine

- Increased risk of bilirubin gallstones

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What is a serious consequence of newborn jaundice?

UCB is fat soluble and can deposit in the basal ganglia, causing kernicterus - neurological deficits

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What is Rotor Syndrome?

It has the same signs as Dubin-Johnson Syndrome but without the liver discoloration

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What are some of the signs of biliary tract obstruction?

Dark urine
Pruritis
Hypercholesterolemia
Steatorrhea

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Viral Hepatitis

Inflammation of the liver parenchyma usually from hepatitis virus

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What are non-hepatitis viruses that can cause hepatitis?

EBV and CMV

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What does chronic hepatitis tend to involve?

Portal Tract

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Transmission of HAV

Travelers via fecal-oral transmission

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Transmission of HBV

Parenteral transmission via childbirth, sex, IV drugs

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Transmission of HCV

Parenteral transmission especially with blood transfusion

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Transmission of HEV

Contaminated water or undercooked seafood

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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