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Pathoma > Liver Diseases > Flashcards

Liver Diseases Flashcards

1
Q

Jaundice

A

Yellow discoloration of the skin –> earliest sign is scleral icterus (yellow discoloration of the sclera)

  • due to increased serum bilirubin, usually >2.5 mg/dL
  • arises with disturbances in bilirubin metabolism

Normal bilirubin metabolism

  • RBCs are consumed by macrophages of the reticuloendothelial system
  • protoporphorin from heme is converted to unconjugated bilirubin –> carried by albumin to the liver
  • Uridine glucuronyl transferase in hepatotcytes conjugates bilirubin
  • conjugated bilirubin is transferred to bile canaliculi to form bile –> stored in gall bladder
  • bile is released into the small bowel to aid in digestion
  • intestinal flora convert conjugated bilirubin to urobilinogen
  • urobilinogen is oxidized to stercobilin (makes stool brown) and urobilin (partially reabsorbed into blood and filtered by kidney, making urine yellow)
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2
Q

Causes of jaundice

A
  1. Extravascular hemolysis
  2. Ineffective erythropoiesis
  3. Physiologic jaundice of the newborn
  4. Gilbert Syndrome
  5. Crigler-Najjar syndrome
  6. Dubin Johnson syndrome
  7. Biliary tract obstruction
  8. Viral hepatitis
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3
Q

Extravascular hemolysis or ineffective erythropoiesis

A

High levels of UCB overwhelm the conjugating ability of the liver
- increased UCB

Clinical features

  • Dark urine due to increased unrine urobilinogen –> UCB is not water soluble and thus is absent from urine
  • increased risk for pigmented bilirubin gallstones
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4
Q

Physiologic jaundice of the newborn

A

Newborn liver has transiently low UGT activity
- increased UCB

Clinical features
- UCB is fat soluble and can deposit in the basal ganglia = kernicterus –> leads to neurological deficits and death

Treatment –> phototherapy = makes UCB water soluble

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5
Q

Gilbert syndrome

A

Mildly low UGT activity

  • autosomal recessive
  • increased UCB

Clinical features
- jaundice during stress (e.g. severe infection), otherwise not clinically significant

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6
Q

Crigler-Najjar syndrome

A

Absence of UGT
- increased UCB

Clinical features

  • Kernicterus
  • usually fatal
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7
Q

Dubin-Johnson Syndrome

A

Deficiency of bilirubin canalicular transport protein

  • autosomal recessive
  • increased CB

Clinical features
- liver is dark, otherwise not clinically significant

Rotor syndrome –> similar but lacks liver discoloration

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8
Q

Biliary tract obstruction

A

Associated with gallstones, pancreatic carcinoma, cholangiocarcinoma, parasites and liver fluke

  • increased CB
  • decreased urobilinogen
  • increased alk phosphatase

Clinical features

  • dark urine due to bilirubinuria and pale stool
  • pruritis due to increased plasma bile acids
  • hypercholesterolemia with xanthomas
  • steatorrhea with malabsorption of fat soluble vitamins
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9
Q

Viral hepatitis

A

Inflammation disrupts hepatocytes and small bile ductules
- increased UCB and CB

Clinical features

  • dark urine due to increased urine bilirubin
  • urine urobilinogen is normal or decreased
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10
Q

Basic principles of viral hepatitis

A

Inflammation of liver parenchyma, usually due to hepatitis virus –> other causes include EBV + CMV
- hepatitis virus causes acute hepatitis, which may progress to chronic hepatitis

Acute hepatitis presents as jaundice (mixed CB + UCB) with dark urine (due to CB), fever, malaise, nausea, and elevated liver enzymes (ALT>AST)

  • inflammation involves lobules of the liver and portal tracts and is characterized by apoptosis of hepatocytes
  • some cases may be asymptomatic with elevated liver enzymes
  • symptoms last 6 months
  • inflammation predominantly involves portal tract
  • risk of progression to cirrhosis
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11
Q

HepA + HepE

A

Fecal oral transmission

  • HAV is commonly acquired by travelers
  • HEV is commonly acquired from contaminated water or undercooked seafood
  • acute hepatitis –> no chronic state
  • Anti-virus IgM marks active infection
  • Anti virus IgG is protective, and its presence indicates prior infection or immunization (available for HepA)
  • HEV infection in pregnant women is associated with fulminant hepatitis = liver failure with massive liver necrosis
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12
Q

HepB

A

Parenteral transmission

Results in acute hepatitis –> chronic disease occurs in 20% of cases

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13
Q

HepC

A

Parenteral tranmission

Results in acute hepatitis, chronic disease occurs in most cases

  • HCV-RNA test confirms infection
  • decreased RNA levels indicate recovery
  • persistence indicates chronic disease
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14
Q

HepD

A

Dependent on HBV for infection

- superinfection upon existing HBV is more severe than coinfection = infection with HBV and HDV at same time

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15
Q

Cirrhosis

A

End stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes
- fibrosis is mediated by TGF B from stellate cells –> lie beneath the endothelial cells that line the sinusoids

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16
Q

Clinical features of cirrhosis

A

Portal hypertension leads to

  • ascites –> fluid in the peritoneal cavity
  • congestive splenomegaly/hypersplenism
  • portosystemic shunts –> esophageal varices, hemorrhoids, and caput medusae
  • hepatorenal syndrome = rapidly developing renal failure secondary to cirrhosis

Decreased detoxification results in

  • mental status changes, asterixis, and eventual come due to increased serum ammonia –> metabolic, so reversible
  • gynecomastia, spider angiomata, and palmar erythema due to hyperestrinism
  • jaundice

Decreased protein synthesis leads to

  • hypoalbuminemia with edema
  • coagulopathy due to decreased synthesis of clotting factors –> degree of deficiency is followed by PT
17
Q

Alcohol related liver disease

A

Damage to hepatic parenchyma due to consumption of alcohol
- most common cause of liver disease in the west

Fatty liver = accumulation of fat in hepatocytes –> results in a heavy, greasy liver; resolves with abstinence

Alcoholic hepatitis results from chemical injury to hepatocytes –> generally seen with binge drinking

  • acetaldehyde mediates damage = metabolite of alcohol
  • characterized by swelling of hepatocytes with formation of mallory bodies (damaged cytokeratin filaments), necrosis and acute inflammation
  • presents with painful hepatomegaly and elevated liver enzymes (AST>ALT) –> may result in death

Cirrhosis is a complication of long term, chronic alcohol induced liver damage –> occurs in 10-20% of alcoholics

18
Q

Non-alcoholic fatty liver disease

A

Fatty change, hepatitis, and/or cirrhosis that develop without exposure to alcohol or other known insult

  • associated with obesity
  • diagnosis of exclusion
  • ALT > AST
19
Q

Hemochromatosis

A

Excess body iron leading to deposition in tissues (hemosiderosis) and organ damage (hemochromatosis)
- tissue damage is mediated by generation of free radicals

Due to autosomal recessive defect in iron absorption (primary) or chronic transfusions (secondary)
- primary hemochromatosis is due to mutations in the HFE gene, usually C282Y –> cysteine is replaced by tyrosine at amino acid 282

20
Q

Clinical features and tx of hemochromatosis

A

Presents in late adulthood

  • classic triad = cirrhosis, secondary diabetes mellitus and bronze skin
  • other findings = dilated cardiomyopathy, cardiac arrhythmias and gonadal dysfunction due to testicular atrophy

Increased risk of hepatocellular carcinoma

Tx –> phlebotomy

21
Q

Diagnosis of hemochromatosis

A

Labs show increased ferritin, decreased TIBC, increased serum iron and increased % sat (iron overloaded state)

Liver biopsy reveals accumulation of brown pigment in hepatocytes
- prussian blue stain distinguishes iron (blue) from lipofuscin –> brown pigment that is a by product from the turnover (“wear and tear”) of peroxidized lipids –> commonly present in hepatocytes

22
Q

Wilson disease

A

Autosomal recessive defect in ATP-mediated hepatocyte copper transport in ATP7B gene

  • results in lack of copper transport into bile and lack of copper incorporation into ceruloplasmin
  • copper builds up in hepatocytes, leaks into serum, and deposits in tissues
  • copper mediated production of hydroxyl free radicals leads to tissue damage
23
Q

Clinical features, labs and tx of Wilsons disease

A

Presents in childhood with…

  • cirrhosis
  • neurologic manifestations –> behavioral changes, dementia, chorea, and parkinsonian symptoms due to deposition of copper in basal ganglia
  • kayser-fleisher rings in the cornea
  • increased risk of hepatocellular carcinoma

Labs show increased urinary copper, decreased serum ceruloplasmin and increased copper on liver biopsy

Tx –> D-penicillamine (chelates copper)

24
Q

Primary biliary cirrhosis

A

Autoimmune granulomatous destruction of intrahepatic bile ducts

  • classically arises in women (avg age 40 years)
  • associated with other autoimmune diseases

Etiology is unknown
- antimitochondrial antibody is present

Presents with features of obstructive jaundice
- cirrhosis is a late complication

25
Q

Primary sclerosing cholangitis

A

Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts

  • periductal fibrosis with an onion skin appearance
  • uninvolved regions are dilated –> results in a “beaded” appearance on contrast imaging

Etiology is unknown, but associated with ulcerative collitis
- p-ANCA often positive

Presents with obstructive jaundice
- cirrhosis is a late complication

Increased risk for cholangiocarcinoma

26
Q

Reye syndrome

A

Fulminant liver failure and encephalopathy in children with viral illness who take aspirin
- likely related to mitochondrial damage of hepatocytes

Presents with

  • hypoglycemia
  • elevated liver enzymes
  • nausea with vomiting
  • may progress to coma and death
27
Q

Hepatic adenoma

A

Benign tumor of hepatocytes

Associated with oral contraceptive use –> regresses upon cessation of drug

Risk of rupture and intraperitoneal bleeding, especially during pregnancy
- tumors are subcapsular and grow with exposure to estrogen

28
Q

Hepatocellular carcinoma

A

Malignant tumor of hepatocytes

Risk factors

  • chronic hepatitis
  • cirrhosis –> alcohol, nonalcoholic fatty liver disease, hemochromatosis, wilson disease, and A1AT deficiency
  • aflatoxins –> derived from aspergillus (induce p53 mutations)

Increased risk for budd-chiari syndrome

  • liver infarction secondary to hepatic vein obstruction
  • presents with painful hepatomegaly and ascites

Tumors are often detected late because symptoms are masked by cirrhosis –> poor prognosis
Serum tumor marker is alpha fetoprotein

29
Q

Metastasis to liver

A

More common than primary liver tumors –> most common sources include colon, pancreas, lung and breast carcinomas

Results in multiple nodules in the liver

clinically may be detected as hepatomegaly with a nodular free edge of the liver

Pathoma (16 decks)

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