PMID: 18209300Jan 23, 2008Paper

Wolfram's (DIDMOAD) Syndrome and Chronic Renal Failure

Saudi Journal of Kidney Diseases and Transplantation : an Official Publication of the Saudi Center for Organ Transplantation, Saudi Arabia
M A HasanA Najada

Abstract

Wolfram's syndrome is usually considered as an autosomal recessive condition, with wide phenotypic variation. The syndrome is commonly called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness), although some patients have additional clinical findings including ataxia, hypogonadism, hydronephrosis and psychiatric illnesses. We report a patient with DIDMOAD syndrome with emphasis on the urological tract and its progressive complications. Unfortunately, he developed end-stage renal failure and needed hemodialysis at the age of 14 years. The presentation, investigations and management are discussed.

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