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

Abstract

UNLABELLED:

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).

• Authors: K Devriendt, L Dooms, W Proesmans, F de Zegher, V Desmet, E Eggermont
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 155 Issue 2 Pg. 87-90 (Feb 1996) ISSN: 0340-6199 [Print] Germany
• PMID: 8775219 (Publication Type: Case Reports, Journal Article)

Topics

• Alagille Syndrome (complications, diagnosis, pathology)
• Biopsy
• Child, Preschool
• Diabetes Mellitus, Type 1 (complications, pathology)
• Humans
• Liver (pathology)
• Male
• Pancreas (pathology)