Abstract

Alagille syndrome (AGS) is an autosomal-dominant multi-organ disorder involving the liver, heart, eyes, face and skeleton. In addition, various renal abnormalities have also been reported in several cases. We describe a patient with a novel frameshift mutation in exon 12 of the JAG1 gene who presented with chronic renal failure. In this family, five members of three generations had clinical features implicated in AGS. Three members had adult-onset renal dysfunction with proteinuria, and two of them required haemodialysis therapy. AGS should be considered in the differential diagnosis of proteinuric renal disease, even in adult patients.

Details

Title
Adult-onset renal failure in a family with Alagille syndrome with proteinuria and a novel JAG1 mutation
Author
Hayashi, Norifumi 1 ; Okuyama, Hiroshi 1 ; Matsui, Yuki 1 ; Yamaya, Hideki 1 ; Kinoshita, Eriko 2 ; Minato, Hiroshi 2 ; Niida, Yo 3 ; Yokoyama, Hitoshi 1 

 Division of Nephrology, Kanazawa Medical University, Uchinada, Ishikawa, Japan 
 Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Ishikawa, Japan 
 Center for Medical Genetics, Kanazawa Medical University, Uchinada, Ishikawa, Japan 
Pages
295-299
Publication year
2013
Publication date
Jun 2013
Publisher
Oxford University Press
ISSN
20488505
e-ISSN
20488513
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3170678391
Copyright
© The Author 2013. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For permissions, please email: [email protected]. This work is published under http://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.