'DIDMOAD' syndrome.

Syndrome of the month Wolfram (DIDMOAD) syndrome

Wolfram syndrome (MIM 222300) is the association ofjuvenile onset diabetes mellitus and optic atrophy, also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). Patients present with diabetes mellitus followed by optic atrophy in the first decade, cranial diabetes insipidus and sensorineural deafness in the second decade, dilated renal outflow tracts early in t...

The Wolfram or DIDMOAD syndrome

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...

DIDMOAD syndrome with megacystis and megaureter.

A case of DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy and nerve deafness) is described. There was unusually severe urinary tract dilatation which led to an ileal conduit diversion. Immunohistological study of the bladder wall and ureter revealed a marked diminution in nerve fibres, which may have been primary or secondary to the muscle hypertrophy. The possible pathog...

DIDMOAD syndrome: a family with three affected siblings.

In 1938 Wolfram reported the occurrence of diabetes mellitus and optic atrophy in four of eight sibs.' Over 100 similar cases have since been reported and several other clinical features have been described.2 3,4 The genetic disorder is commonly known as DIDMOAD syndrome, the mnemonic for Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness. This report describes three siblings wit...

DIDMOAD syndrome in a Chinese male with HLA DR7 DRw12.