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Title: Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome ?
Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?
Authors: Devriendt, Koenraad ×
Dooms, Luc
Proesmans, Willem
de Zegher, Francis
Desmet, Valeer
Eggermont, Ephrem #
Issue Date: 1996
Dec-1996
Publisher: Springer-Verlag
Series Title: European Journal of Pediatrics vol:155 pages:87-90
European Journal of Pediatrics. vol:155 issue:2 pages:87-90
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).
ISSN: 0340-6199
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Clinical Genetics Section (-)
Section Newborn (-)
Department of Human Genetics - miscellaneous
Translational Cell & Tissue Research
Faculty of Medicine - miscellaneous
× corresponding author
# (joint) last author

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