| Syndromic bile duct paucity—Alagille syndrome |
| Non-syndromic bile duct paucity |
| Metabolic and genetic disorders |
| Alpha-1 antitrypsin deficiency |
| BSEP deficiency (ABCB11) |
| MDR3 deficiency (ABCB4) |
| HNF3β deficiency |
| Cystic fibrosis |
| Peroxisomal disorders |
| Niemann pick type C |
| ARC syndrome (arthrogryposis-renal-cholestasis) |
| Kabuki syndrome (rare) |
| Chromosomal abnormalities (trisomy 17, 18 or 21) (rare) |
| Infection |
| Congenital cytomegalovirus infection |
| Congenital syphilis (rare) |
| Congenital rubella (rare) |
| Inflammatory and immune disorders |
| Graft versus host disease |
| Chronic hepatic allograft rejection |
| Sclerosing cholangitis (primary or secondary) |
| Biliary atresia (late) |
| Sarcoidosis (rare) |
| Other |
| Drug- or antibiotic-associated vanishing bile duct syndrome |
| Panhypopituitarism (rare) |
| Idiopathic |
