Hasan HATİPOĞLU, Müjgan Güngör HATİPOĞLU, Özden KANSU

WOLFRAM (DIDMOAD) SENDROMU BULUNAN BİR OLGUDAKİ ORAL BULGULAR (OLGU BİLDİRİMİ)

Wolfram Sendromu, nadir görülen konjenital, birçok sistemi etkileyebilen sendromdur. Diabetes mellitus,diabetes insipidus, optik atrofi ve sağırlık ile karakterizedir. Hastalarda nörolojik rahatsızlıklar ile üriner sistemrahatsızlıkları ve psikolojik rahatsızlıklar da izlenir. Diabetes mellitus ve optik atrofi ilk ortaya çıkan bulgulardır.Bu olgu raporunda 20 yaşındaki Wolfram sendromlu erkek hastanın klinik ve radyografik ağız bulgularısunulmaktadır.

Anahtar Kelimeler:

Wolfram sendromu, DIDMOAD, oral bulgular

A CASE WITH WOLFRAM (DIDMOAD) SYNDROME AND ORAL FINDINGS (A CASE REPORT)

Wolfram Syndrome is an unusual, congenital, multi system disorder. Diabetes mellitus, Diabetes insipidus, opticatrophy and deafness are most observable symptoms. Neurological, urinary tract and psychiatric disorders are theother common symptoms. Diabetes mellitus and optic atrophy are usually the first findings.In this case report we present 20 year old-male patient, that was have Wolfram syndrome. Oral clinical andradiological findings were presented.

Keywords:

Wolfram Syndrome, DIDMOAD, oral findings,

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[1] Internet: OMIM, In: Online Mendelian Inheritance in Man.http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?cmd=entry&id=222300 (2007)
[2] Swift, R.G., Sadler, D.B., Swift, M.,” Psychiatric findings in Wolfram syndrome homozygotes”, Lancet,336: 667-669 (1990).
[3] Barrett, T.G., Bundey, S.E., Macleod, A.F., “Neurodegeneration and diabetes: UK nationwide study of Wolfram (DIDMOAD) syndrome”, Lancet, 2;346 (8988): 1458-63 (1995).
[4] Hardy, C., Khanim, F., Torres, R., Scott-Brown M., Seller A, Poulton J., Collier D., Kirk J.,Polymeropoulos M., Latif F., Barrett T., “Clinical and molecular genetic analysis of 19 Wolfram syndrome kindreds demonstrating a wide spectrum of mutations in WFS1”, Am J Hum Genet.,65(5):1279-90 (1999).
[5] Bu, X., Rotter, J.I., ”Wolfram syndrome: a mitochondrial-mediated disorder ?”, Lancet, 4;342 (8871):598-600 (1993).
[6] Sugano, N., Kawamoto, K., Numazaki, H., Murai, S., Ito, K., “Detection of mitochondrial DNA mutations in human gingival tissues”, J. Oral Sci., 42 (4):221-223 (2000).
[7] Zen, P.R., Pinto, L.L., Schwartz, I.V., Barrett, T.G., Paskulin, G., “Report of a Brazilian patient with Wolfram Syndrome”, J. Pediatr (Rio J)., 78(6): 529-32 (2002).
[8] Rotig, A., Cormier, V., Chatelain, P., Francois, R., Saudubray, J.M., Rustin, P., Munnich, A., ”Deletion of mitochondrial DNA in a case of early-onset diabetes mellitus, optic atrophy, and deafness (Wolfram syndrome, MIM 222300)”, J. Clin. Invest., 91(3): 1095-8 (1993).
[9] Langlais, R.P., Miller, C.S., ”Color Atlas of Common Oral Diseases”, Lea & Febiger, Philadelphia/London, 46-47 (1992).
[10] Mealey, B.,” Periodontal Medicine”, Rose, F.L., Mealey B.L., Genco, R..J., Cohen, W.D., B.C. Decker Inc., Toronto, 121-150 (1999).
[11] Terezhalmy, G. T., “Internal Medicine for Dentistry, 2 nd edition”, Kaye, D., Rose, L.F., The C.V.Mosby Company, St Louis, 1153-1154 (1990).