A Rare Case of Wolfram Syndrome

A 15-year-old boy was referred because of bilateral hydroureteronephrosis. He had poorly controlled diabetes mellitus since he was 4 years old. He had polyuria and polydipsia. On water deprivation test, he developed hypernatremia along with increased levels of BUN and creatinine. He also had hypertension that was effectively managed with losartan. Bilateral optic atrophy was detected on ophthalmoscopic examination. It seems that this boy is a rare case of Wolfram syndrome.Keywords: Diabetes Insipidus; Diabetes Mellitus; Wolfram Syndrome; DIDMOAD; Deafness; Optic Atrophy