PT  - JOURNAL ARTICLE
AU  - S S Najjar
AU  - M G Saikaly
AU  - G M Zaytoun
AU  - A Abdelnoor
TI  - Association of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. The Wolfram or DIDMOAD syndrome.
AID  - 10.1136/adc.60.9.823
DP  - 1985 Sep 01
TA  - Archives of Disease in Childhood
PG  - 823--828
VI  - 60
IP  - 9
4099  - http://adc.bmj.com/content/60/9/823.short
4100  - http://adc.bmj.com/content/60/9/823.full
SO  - Arch Dis Child1985 Sep 01; 60
AB  - Seven patients with a rare syndrome of diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract, and other abnormalities (Wolfram or DIDMOAD syndrome) are reported. Of the seven patients, three siblings were followed up for 10-17 years. All seven patients had diabetes mellitus and optic atrophy; six had diabetes insipidus; and in the four patients investigated there was dilatation of the urinary tract. The severity of diabetes varied, and all required insulin for control of the hyperglycaemia. In one patient the course of the disease simulated maturity onset diabetes of the young; another presented with ketoacidosis; but none had haplotypes usually associated with insulin dependent diabetes mellitus. The diabetes insipidus responded to chlorpropamide, suggesting partial antidiuretic hormone deficiency. Onset of optic atrophy and loss of vision occurred relatively late and progressed slowly, although in one patient there was a rapid deterioration in visual acuity. Deafness was mild, of late onset, and of sensorineural origin. A degenerative process affecting the central and peripheral nervous system can explain all the manifestations of the syndrome except diabetes mellitus. The pathogenesis of the diabetes mellitus remains obscure.