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

التفاصيل البيبلوغرافية
العنوان: Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: Atypical Alagille syndrome?
المؤلفون: K. Devriendt, L. Dooms, W. Proesmans, F. de Zegher, E. Eggermont, V. Desmet
المصدر: European Journal of Pediatrics. 155:87-90
بيانات النشر: Springer Science and Business Media LLC, 1996.
سنة النشر: 1996
مصطلحات موضوعية: Male, Autoimmune disease, Kidney, Pathology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Biopsy, Intrahepatic bile ducts, medicine.disease, Alagille Syndrome, Diabetes Mellitus, Type 1, medicine.anatomical_structure, Liver, Dysplasia, Child, Preschool, Diabetes mellitus, Pediatrics, Perinatology and Child Health, Alagille syndrome, medicine, Humans, business, Pancreas
الوصف: 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. This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Iyemark syndrome).
تدمد: 1432-1076
0340-6199
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b9ce67ca1b6f1b3070f5c5ee39ad760f
https://doi.org/10.1007/bf02075756
حقوق: CLOSED
رقم الأكسشن: edsair.doi.dedup.....b9ce67ca1b6f1b3070f5c5ee39ad760f
قاعدة البيانات: OpenAIRE