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南非开普有色人种社区的亨廷顿舞蹈症

Huntington's chorea in the Cape coloured community of South Africa.

作者信息

Hayden M R, Beighton P

出版信息

S Afr Med J. 1977 Nov 19;52(22):886-8.

PMID:146919
Abstract

Huntington's chorea is a grave genetic neurological disorder which appears to be a more serious problem in South Africa than has generally been recognized. The minimum prevalence of the disease in the Cape Coloured is 3,5/100 000 of the population. Sixteen per cent of patients were younger than 20 years at the time of onset of the disease. This is among the highest incidence of juvenile Huntington's chorea in the world. The gene for Huntington's chorea has been in South Africa for 300 years. It was first introduced into this country from Holland in the middle of the 17th century and then later from England in the middle and end of the 19th century. The number of affected individuals appears to be on the increase in South Africa. Unfortunately, at present, no methods are available for effective control of this disorder.

摘要

亨廷顿舞蹈症是一种严重的遗传性神经疾病,在南非似乎是一个比普遍认知更为严重的问题。开普混血人种中该疾病的最低患病率为每10万人中有3.5例。16%的患者在疾病发作时年龄小于20岁。这是世界上青少年亨廷顿舞蹈症发病率最高的情况之一。亨廷顿舞蹈症的基因在南非已存在300年。它于17世纪中叶首次从荷兰引入该国,随后在19世纪中叶和末期又从英国引入。在南非,受影响的个体数量似乎在增加。不幸的是,目前尚无有效控制这种疾病的方法。

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