DIDMOAD syndrome DIDMOAD-SYNDROM
Wiener Medizinische Wochenschrift, cilt.144, sa.4, ss.78-81, 1994 (Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 144 Sayı: 4
- Basım Tarihi: 1994
- Dergi Adı: Wiener Medizinische Wochenschrift
- Derginin Tarandığı İndeksler: Scopus
- Sayfa Sayıları: ss.78-81
- Anahtar Kelimeler: acoustic evoked potentials, bilateral hydronephrose, cerebral MRI, diabetes insipidus, diabetes mellitus, DIDMOAD syndrome, HLA-DR-typing, N. opticus atrophia, Wolfram syndrome
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
Özet
The DIDMOAD or so called Wolfram syndrome is a hereditary disease with autosomal-recessive transmission showing 4 main features: diabetes mellitus, diabetes insipidus, nervus opticus atrophia and deafness. Beside this it shows multiple organ involvement. Our 38-year old male patient, showing all above mentioned features except deafness had urinary tract involvement and neurological symptoms. EEG, cerebral MRI, tests with evoked potentials and HLA-typing were performed to discuss the aetiopathogenetic background in our patient. Almost all symptoms of the Wolfram syndrome can be mixed up with complications of diabetes mellitus, which is usually the first symptom of the Wolfram syndrome. Because of this, wrong diagnosis is not rare. Hence in differential diagnosis in any diabetes mellitus type I patient, the possibility of the Wolfram syndrom should be discussed.