Microvascular Diabetes Complications in Wolfram Syndrome (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness
[DIDMOAD])
An age- and duration-matched comparison with common type 1 diabetes
Aline Cano , MD 1 ,
Laurent Molines , MD 1 ,
René Valéro , PHD 1 ,
Gilbert Simonin , MD 1 ,
Véronique Paquis-Flucklinger , MD, PHD 2 ,
Bernard Vialettes , MD 1 and
the French Group of Wolfram Syndrome *
1 Department of Nutrition, Metabolic Diseases and Endocrinology, University of Méditerranée, “La Timone” Hospital, Marseille,
France
2 Department of Medical Genetics, University of Nice, “Archet-2” Hospital, Nice, France
Address correspondence and reprint requests to Bernard Vialettes, MD, Service de Nutrition, Maladies Métaboliques, Endocrinologie,
Centre Hospitalier Universitaire La Timone, 264 rue Saint Pierre, 13005 Marseille, France. E-mail: bernard.vialettes{at}ap-hm.fr
Abstract
OBJECTIVE —Some previous studies suggested that patients suffering from Wolfram syndrome or DIDMOAD (diabetes insipidus, diabetes mellitus,
optic atrophy, and deafness) might be relatively preserved from diabetic retinopathy and nephropathy. However, these data
were not conclusive because either observations were only anecdotic or did not match with control type 1 diabetic populations.
RESEARCH DESIGN AND METHODS —A group of 26 French diabetic patients with DIDMOAD was compared with a population of 52 patients with common type 1 diabetes
matched for age at diabetes diagnosis (8.62 ± 1.84 vs. 8.27 ± 1.30 years; P = NS) and diabetes duration (12.88 ± 1.58 vs. 12.87 ± 1.13 years; P = NS) to study the quality of glycemic control and the incidence of microvascular complications.
RESULTS —Glycemic control was significantly better in the DIDMOAD group than in the type 1 diabetic group (A1C: 7.72 ± 0.21 vs. 8.99
± 0.25%, respectively; P = 0.002), with significant lower daily insulin requirements (0.71 ± 0.07 vs. 0.88 ± 0.04 UI · kg −1 · day −1 , respectively; P = 0.0325). The prevalence of microvascular complications in the DIDMOAD group was half that observed in the type 1 diabetic
group, but the difference was not significant.
CONCLUSIONS —Diabetes in DIDMOAD patients is more easily controlled despite the presence of other handicaps. This better glycemic control
could explain the trend to decreased microvascular diabetes complications observed in previous studies.
DIDMOAD, diabetes insipidus, diabetes mellitus, optic atrophy, and deafness
Footnotes
Published ahead of print at http://care.diabetesjournals.org on 29 May 2007. DOI: 10.2337/dc07-0380.
A table elsewhere in this issue shows conventional and Système International (SI) units and conversion factors for many substances.
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Microvascular Diabetes Complications in Wolfram Syndrome (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness [DIDMOAD])

10.2337/dc07-0380