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association hepatic ductopenia, renal agenesis and pancreatic atrophy

Monday 22 January 2007


- hepatic biliary ductopenia (paucity of intrahepatic bile ducts)

- renal anomalies

  • unilateral renal agenesis
  • subcortical renal cysts

- pancreatic anomalies

  • insulin-dependent diabetes mellitus
  • pancreatic atrophy

Differential diagnosis

- Alagille syndrome
- renal-hepatic-pancreatic dysplasia (former Ivemark syndrome type 2)


- Devriendt K, Dooms L, Proesmans W, de Zegher F, Desmet V, Eggermont E. Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome? Eur J Pediatr. 1996 Feb;155(2):87-90. PMID: 8775219