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 DIDMOAD or Wolfram syndrome. Aside from these four cardinal features, a wide variety of abnormalities of the nervous system, urinary tract and endocrine glands have been described in this syndrome. In this report, the clinical features of six patients with DIDMOAD syndrome are presented. All six patients had DM. Five of the six patients had DI, five OA and five displayed abnormal audiogram findings. In addition, two had goiter, two delayed puberty, one seizure and one mental retardation with depression attacks. Urinary tract dilatation was recorded in five patients. Four patients developed typical complications of DM. One of them had overt nephropathy and arthropathy despite the short duration of DM. In addition, this patient had diabetic retinopathy, which is considered to be rare in this syndrome.
Okten
A
A
Department of Pediatrics, Karadeniz Technical University Faculty of Medicine, Trabzon.
Gedik
Y
Y
Demirci
A
A
Mocan
H
H
Erduran
E
E
Aslan
Y
Y
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology / Q000473:pathology; D011859:Radiography; D014421:Turkey; D014463:Ultrasonography; D014570:Urologic Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D014929:Wolfram Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology