THE SYNDROME OF DIABETES INSIPIDUS, DIABETES MELLITUS, OPTIC ATROPHY, DEAFNESS, AND ATONIA OF THE URINARY TRACT (DIDMOAD SYNDROME). TWO AFFECTED SIBS AND A SHORT REVIEW OF THE LITERATURE

نویسندگان

  • ATAOLLAH BEHROUZ AGHDAM
  • DAVOOD SHARIFI DOLOUI University of Medical Sciences, Mashhad, Islamic Republic of Iran
چکیده مقاله:

Two brothers with DIDMOAD syndrome are reported. The older brother has diabetes mellitus (type I), diabetes insipidus, optic atrophy, deafness and atonia of the urinary tract with severe symptoms such as diabetic ketoacidosis and frequent urinary tract infections. His younger brother had the same manifestations but with less severity. We report the findings of our two patients and compare them with the frequency of the symptoms in 100 patients from the literature.

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the syndrome of diabetes insipidus, diabetes mellitus, optic atrophy, deafness, and atonia of the urinary tract (didmoad syndrome). two affected sibs and a short review of the literature

two brothers with didmoad syndrome are reported. the older brother has diabetes mellitus (type i), diabetes insipidus, optic atrophy, deafness and atonia of the urinary tract with severe symptoms such as diabetic ketoacidosis and frequent urinary tract infections. his younger brother had the same manifestations but with less severity. we report the findings of our two patients and compare them ...

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Diabetes mellitus, diabetes insipidus, optic atrophy, and deafness: A case of Wolfram (DIDMOAD) syndrome

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Familial syndrome of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (didmoad) in childhood.

Marquardt and Loriaux (I974) describe a kinship of two siblings with the combination of diabetes mellitus, diabetes insipidus, and optic atrophy in which there was additional evidence of renal tract dilatation, amino-aciduria, and neurosensory hearing deficit. These authors cite 41 cases of optic atrophy and diabetes mellitus reported since an association of these conditions was first described...

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Wolfram Syndrome (Diabetes Insipidus, Diabetes, Optic Atrophy, and Deafness)

OBJECTIVE Wolfram syndrome is an autosomal recessive neurodegenerative disorder characterized by diabetes insipidus, diabetes (nonautoimmune), optic atrophy, and deafness (a set of conditions referred to as DIDMOAD). The WFS1 gene is located on the short arm of chromosome 4. Wolfram syndrome prevalence is 1 in 770,000 live births, with a 1 in 354 carrier frequency. RESEARCH DESIGN AND METHODS...

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عنوان ژورنال

دوره 2  شماره 2

صفحات  149- 154

تاریخ انتشار 1988-08

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