Paucity of intrahepatic bile ducts,solitary kidney and atrophic pancreas with diabetes mellitus: Atypical Alagille syndrome? |
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| Authors: | K Devriendt L Dooms W Proesmans F de Zegher E Eggermont V Desmet K Devriendt |
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| Institution: | (1) Department of Paediatrics, University Hospital Leuven, Leuven, Belgium;(2) Department of Pathology, University Hospital Leuven, Leuyen, Belgium;(3) Centre for Human Genetics, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium |
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| Abstract: | 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 (Iyemark syndrome). |
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| Keywords: | Alagille syndrome Diabetes mellitus Ivemark syndrome Pancreas |
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