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Original Articles

Morbidity and Mortality in the Wolfram Syndrome

  1. Brendan T Kinsley, MB, MRCP,
  2. Michael Swift, MD,
  3. Raymonde H Dumont, MD and
  4. Ronnie G Swift, MD
  1. Joslin Diabetes Center, New England Deaconess Hospital, and Harvard Medical School Boston, Massachusetts
  2. Division of Human Molecular Genetics, Department of Psychiatry, Institute for the Genetic Analysis of Common Diseases, New York Medical College Hawthorne, New York
  3. Department of Pediatrics and Division of Psychiatric Genetics, Department of Psychiatry, Institute for the Genetic Analysis of Common Diseases, New York Medical College Hawthorne, New York
  1. Address correspondence and reprint requests to Ronnie G. Swift, MD, Division of Psychiatric Genetics, Institute for the Genetic Analysis of Common Diseases, New York Medical College, 4 Skyline Dr., Hawthorne, NY 10532. E-mail: ronnie-swift{at}NYMC.edu.
Diabetes Care 1995 Dec; 18(12): 1566-1570. https://doi.org/10.2337/diacare.18.12.1566
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Abstract

OBJECTIVE To determine the major causes of morbidity and mortality in the autosomal recessive Wolfram syndrome, which is defined by diabetes and bilateral progressive optic atrophy with onset in childhood or adolescence.

RESEARCH DESIGN AND METHODS We abstracted and reviewed the medical records of 68 confirmed cases of Wolfram syndrome identified through a nationwide survey of endocrinologists, ophthalmologists, institutes, and homes for the blind. We also reviewed all available autopsy records.

RESULTS The most common causes of morbidity and mortality were the neurological manifestations of this syndrome and the complications of urinary tract atony. There was a lower frequency of diabetic ketoacidosis, no histologically proven diabetic glomerulosclerosis, and less severe, more slowly progressive, diabetic retinopathy than in classic type I diabetic patients. Mortality in Wolfram syndrome is much higher than in type I diabetes; 60% of Wolfram syndrome patients die by age 35. Recognition of these clinical differences from classic type I diabetes is important for the proper management of Wolfram syndrome patients.

CONCLUSIONS Identification of Wolfram syndrome patients among all diabetic patients presenting in childhood or adolescence is important because the management of patients with this syndrome is different from that of patients with classic type I diabetes.

  • Received March 31, 1995.
  • Revision received July 27, 1995.
  • Accepted July 27, 1995.
  • Copyright © 1995 by the American Diabetes Association

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December 1995, 18(12)
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Morbidity and Mortality in the Wolfram Syndrome
Brendan T Kinsley, Michael Swift, Raymonde H Dumont, Ronnie G Swift
Diabetes Care Dec 1995, 18 (12) 1566-1570; DOI: 10.2337/diacare.18.12.1566

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Morbidity and Mortality in the Wolfram Syndrome
Brendan T Kinsley, Michael Swift, Raymonde H Dumont, Ronnie G Swift
Diabetes Care Dec 1995, 18 (12) 1566-1570; DOI: 10.2337/diacare.18.12.1566
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