DDx Intrahepatic Bile Duct Paucity in Children

• Syndromic bile duct paucity (Alagille syndrome)
• Nonsyndromic bile duct paucity:
• Metabolic and genetic disorders
1. alpha-1-antitrypsin deficiency
2. cystic fibrosis (rare)
3. peroxisomal disorders (rare)
4. familial cholestatic disorders (some)
5. trisomy 21 (rare)
6. prune-belly syndrome (rare)
• Infection
1. Congenital cytomegalovirus infection (rare)
2. congenital syphilis (rare)
3. congenital rubella (rare)
• Inflammaotry and Immune Disorders
1. Graft vs host disease
2. chronic hepatic allograft rejection
3. sclerosing cholangitis
4. sarcoidosis (rare)
• other
1. drug or antibiotic-associated vanishing bile duct syndrome
2. familial idiopathic adulthood ductopenia
3. biliary atresia (late)
4. panhypopituitarism (rare)
5. idiopathic

References:

• Russo P. et al. (eds). Pathology of Pediatric Gastrointestinal and Liver Disease (2004)