Cholestatic Liver Disease

Posted by e-Medical PPT
Adult Cholestatic Liver Disease
  • Primary Biliary Cirrhosis
  • Primary Sclerosing Cholangitis
  • Intrahepatic cholestasis
  • GVHD liver
  • TPN induced cholestasis
  • Sarcoidosis
  • idipathic ductopenia
  • Liver transplant rejection
  • Cystic fibrosis
  • AIDS cholangiopathy
Pediatric cholestatic diseases
  • Cystic fibrosis
  • Alagille’s syndrome
  • Biliary atresia
  • Byler’s disease
  • Inborn errors of metabolism
Liver disease in cholestasis
Mediated by high levels of toxic bile acids
Preferential retention of hydrophobic bile acids
Direct damage to hepatocytes and bile duct cells
Lithocholic and chenodeoxycholic acids are toxic
Degree of liver damage related to bile acid level
Damage to cell membranes, necrosis
Cholestasis may lead to immune activation
Mechanism is probably multifactorial
Initial immunologic insult
Bile duct loss and failure to transport bile acids
Retention of hydrophobic bile acids
Cholate, chenodeoxycholate accumulate
further direct and immune-mediated damage

Clinical complications of cholestasis
Pruritus very common and may be disabling
Exact mechanism unknown
Possible relationship with bile acid levels
Fat malabsorption and steatorrhea
Fat-soluble vitamin deficiencies (A, D, E, K)
Marked elevation in cholesterol levels
Xanthomas and xantholesmas
 Early and severe osteoporosis may occur

Laboratory tests in cholestatic disease

Very high serum alkaline phosphatase, GGT
Less striking elevation in other liver enzymes
Serum bilirubin is elevated late in the disease
Four stages of disease:
 one: no symptoms
 two: fatigue and pruritus
 three: liver fibrosis and elevated bilirubin
 four: cirrhosis and liver failure

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