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Eur J Pediatr. 1996 Feb;155(2):87-90.

Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?

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  • 1Department of Paediatrics, University Hospital Leuven, Belgium.

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.

CONCLUSION:

This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).

PMID:
8775219
[PubMed - indexed for MEDLINE]
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