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

  1. Aline Cano, MD1,
  2. Laurent Molines, MD1,
  3. René Valéro, PHD1,
  4. Gilbert Simonin, MD1,
  5. Véronique Paquis-Flucklinger, MD, PHD2,
  6. Bernard Vialettes, MD1 and
  7. the French Group of Wolfram Syndrome*
  1. 1Department of Nutrition, Metabolic Diseases and Endocrinology, University of Méditerranée, “La Timone” Hospital, Marseille, France
  2. 2Department of Medical Genetics, University of Nice, “Archet-2” Hospital, Nice, France
  1. 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.

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.

    The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked “advertisement” in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

  • *

    ↵* A list of the members of the French Group of Wolfram Syndrome can be found in the appendix.

    • Accepted May 21, 2007.
    • Received February 23, 2007.
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  1. Published online before print May 29, 2007, doi: 10.2337/dc07-0380 Diabetes Care September 2007 vol. 30 no. 9 2327-2330
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