少年型糖尿病是一种具遗传倾向的影响机体物质代谢的内分泌疾患,多属胰岛素依赖型,大多为原发性,病因不明。随着糖尿病病因学研究的深入,目前认为少年型原发性糖尿病中有些患者与病毒感染及自身免疫有关,大部患者可能为病毒感染后触发个体对胰岛产生自身免疫性胰岛炎所致,遗传素质使机体对上述致病因素具有易感性。本文概要综述少年型糖尿病中与遗传密切有关的一种特殊类型—Wolfram综合征。 Wolfram综合征的临床特点 1938年Wolfram首次报导一个家庭,在8个同胞兄妹中有4例发生糖尿病与视神经萎缩,后Ducan认为这种患者尚可伴耳聋,1970年Ikkos认为还可伴尿崩症,1977 Juvenile diabetes is a genetic predisposition of endocrine disorders affecting the body’s metabolism, mostly insulin-dependent, mostly primary, the cause is unknown. With the deepening of the research on the etiology of diabetes, some patients in juvenile primary diabetes are currently considered to be associated with viral infection and autoimmunity. Most patients may be triggered by the virus after the individual islets produce autoimmune insulitis, genetic Quality makes the body susceptible to the above pathogenic factors. This article provides an overview of a special type of Wolfram’s syndrome associated with heredity in juvenile diabetes. Clinical Features of Wolfram’s Syndrome Wolfram first reported a family in 1938, with diabetes and optic atrophy in 4 of 8 siblings. Ducan said this patient was still deaf. In 1970, Ikkos thought it could be associated with diabetes insipidus , 1977