Simple jQuery Dropdowns
Please use this identifier to cite or link to this item:
Title: Renal failure in two patients with Wolfram syndrome
Authors: Achra Sumboonnanonda
Arun Vongjirad
Vibul Suntornpoch
Kitti Angsusingha
Paisal Parichatikanond
Tawee Laohapand
Mahidol University
Faculty of Medicine, Siriraj Hospital, Mahidol University
Keywords: Biochemistry, Genetics and Molecular Biology;Medicine
Issue Date: 1-Jan-1997
Citation: Journal of Pediatric Endocrinology and Metabolism. Vol.10, No.6 (1997), 645-651
Abstract: We describe a Thai family with three children, two of whom presented with Wolfram syndrome, which is a rare syndrome characterized by diabetes insipidus, diabetes mellitus, optic atrophy, deafness and urinary tract dilatation. A girl and her younger brother had insulin-dependent diabetes mellitus at 11 years old with early onset of renal impairment, proteinuria and hypertension. Urinary tract dilatation was demonstrated in both patients. Kidney biopsies were compatible with diabetic nephropathy. Both children also had bilateral sensorineural hearing loss. Optic atrophy with severe loss of vision was detected in the girl and bilateral cataract in her brother. Both patients were HLA DR2positive. At 16 years old, her creatinine clearance was 16 ml/min/1.73 m2. Her brother's creatinine clearance was 25 ml/min/1.73 m2at 13 years old. We conclude that renal function should be evaluated in patients with Wolfram syndrome and the cause of renal failure in these patients may be rapid and severe diabetic nephropathy.
ISSN: 0334018X
Appears in Collections:Scopus 1991-2000

Files in This Item:
There are no files associated with this item.

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.