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familial cholestasis

Wednesday 4 June 2003

Classification

extrahepatic cholestasis

intrahepatic cholestasis

- biliary ducts

  • Alagille’s syndrome
  • Norwegian cholestasis
  • neonatal sclerosing cholangitis
  • turkish nonsyndromic paucity of interlobular bile ducts
  • north american indian cirrhosis (16q22)

- lobule

  • congenital anomalies of bile acid metabolism
  • progressive familial intrahepatic cholestases (PFIC)
    • PFIC-1 (Byler’s disease) (P-type adenosine triphosphatse - 18q21-22)
    • benign recurrent intrahepatic cholestasis (BRIC) (P-type adenosine triphosphatse - 18q21-22)
    • PFIC-2 (Canalicular bile acide transporte ABCB11 - 2q24)
    • PFIC-3 (canalicular phospholipid transporter ABCB4 - 7q21)

- Unknown origin

  • Greenland Eskimo cholestasis
  • indian childhood cirrhosis
  • childhood cirrhosis in Arab israelis
  • tyrolean infantile cirrhosis