VARIOUS CLINICAL ASPECTS OF DIDMOAD (WOLFRAM) SYNDROME

The association of juvenile diabetes mellitus (DM), diabetes insipidus (DI), optic atrophy (OA) and sensorineural deafness (D) is known as D IDMOAD or Wolfram syndrome. Aside from these four cardinal features, a wide variety of abnormalities of the nervous system, urinary tract an d endocrine glands have been described in this syndrome. In this repor t, the clinical features of six patients with DIDMOAD syndrome are pre sented. All six patients had DAO. Five of the six patients had DI, fiv e OA and five displayed abnormal audiogram findings. In addition, two had goiter, two delayed puberty, one seizure and one mental retardatio n with depression attacks. Urinary tract dilatation was recorded in fi ve patients. Four patients developed typical complications of DM. One of them had overt nephropathy and arthropathy despite the short durati on of DM. In addition, this patient had diabetic retinopathy, which is considered to be rare in this syndrome.