title: Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?
abstract: A child with the tentative diagnosis of Alagille syndrome is reported. Additional renal abnormalities are unilateral kidney agenesis and a kidney with subcortical cysts with decreased function. At the age of 5 years, insulin-dependent diabetes mellitus developed, with the pancreas being atrophic and negative pancreatic islet cell antibodies.
  
  
  CONCLUSIONS
  This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).
authors.affiliation: Department of Paediatrics, University Hospital Leuven, Belgium.
authors.forename: K
authors.initials: K
authors.lastname: Devriendt
authors.forename: L
authors.initials: L
authors.lastname: Dooms
authors.forename: W
authors.initials: W
authors.lastname: Proesmans
authors.forename: F
authors.initials: F
authors.lastname: de Zegher
authors.forename: V
authors.initials: V
authors.lastname: Desmet
authors.forename: E
authors.initials: E
authors.lastname: Eggermont
mesh_terms: D016738:Alagille Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D001706:Biopsy; D002675:Child, Preschool; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D010179:Pancreas / Q000473:pathology