Liver and intrahepatic bile ducts-nontumor
Intrahepatic biliary atresia
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 23 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.
● Also called paucity of intrahepatic bile ducts in neonates
● Occurs in syndromic form (Alagille’s syndrome) or nonsyndromic form (Eur J Hum Genet. 2012 Mar;20(3):251-7)
● In nonsyndromic form, bile duct loss is present from birth
● May actually represent cystic fibrosis, alpha-1-antitrypsin deficiency, trihydroxycoprostanic acidemia
● Bile duct recovery depends on etiology
● Loss of interlobular bile ducts (0.5 ducts/portal tract vs. normal 0.9-1.8 ducts/portal tract
● Count only bile ducts accompanying hepatic arterioles in center of portal tracts, keratin staining may be helpful)
● Usually minimal ductular proliferation
● No/minimal fibrosis, no/minimal inflammation
Alagille syndrome with bile duct paucity
End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Intrahepatic biliary atresia
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