Abstract: Wolfram syndrome is a rare autosomal recessive disorder caused by mutations in WFS1, a gene implicated in endoplasmic reticulum and mitochondrial function. It is characterized by insulin-requiring diabetes mellitus and optic atrophy. An older name for the syndrome is DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). A 17 year old male patient, a know case of diabetes mellitus type I diagnosed 11 years back and significant for optic atrophy, diagnosed at age 11 which progressed to severe visual impairment over the following 6 years.
This study point up the need for careful evaluation of such cases having insulin dependent diabetes mellitus and optic atrophy. Wolfram syndrome affects different organs and systems in the body. Thus, multidisciplinary care by physicians and healthcare professionals from a range of disciplines is required. Careful clinical monitoring and supportive care can help relieve the suffering of patients and improve their quality of life.
Keywords: Wolfram syndrome; Optic atrophy; Diabetes mellitus; Deafness.
Title: A CASE OF WOLFRAM SYNDROME - CASE REPORT
Author: Glady Francis Mathew, Roshin Susan Gigi, Khalid PK
International Journal of Healthcare Sciences
ISSN 2348-5728 (Online)
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