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association hepatic ductopenia, renal agenesis and pancreatic atrophy
Monday 22 January 2007
Synopsis
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)
References
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