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Wolfram Syndrome-A Case Report
Wolfram氏症候群－病例報告
摘要
糖尿病及視神經萎縮是Wolfram氏症候群診斷上的必要條件。然此種病患常合併有尿崩症，聽覺障礙，尿道異常及退化性神經病變。本文報告一位十九歲女孩併有幼年型胰島素依賴型糖尿病，視神經萎縮，聽覺障礙及眼震顫；但並未有尿崩症，尿道異常及其他明顯的退化性神經病變。於幼年型胰島素依賴型糖尿病病患中診斷出Wolfram氏症候群是相當重要的，因其病程並不同於一般胰島素依賴型糖尿病，且會因併有退化性神經病變及尿道異常而有較高的死亡率，故醫師應同時注意其合併症且予以適當處置。
關鍵字
無資料並列摘要
Wolfram syndrome is characterized by the coexistence of diabetes mellitus and optic atrophy, but may also associate with diabetes insipid us, deafness, urinary tract dilatation, ataxia and psychiatric disorders. We report a 19 year-old female patient presenting with juvenile-onset diabetes mellitus, bilateral optic atrophy, high frequency hearing impairment, and gaze evoked nystagmus, but without diabetes insipidus, urinary tract abnormality or other obvious signs of neurodegeneration. Identification of Wolfram syndrome among juvenile-onset diabetics is important because of the distinctly different sources of morbidity and causes of mortality from those of classic insulin- dependent diabetics.
延伸閱讀
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