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巴布亚新几内亚亨廷顿舞蹈症登记册。

The Huntington's Disease Register of Papua New Guinea.

作者信息

Scrimgeour E M

出版信息

P N G Med J. 1982 Mar;25(1):16-8.

PMID:6214908
Abstract

The discovery of Huntington's chorea in New Britain, Papua New Guinea in 1977 was followed by the establishment of a National Huntington's Disease Register. At present there are 11 choreics and 136 potential choreics registered within 5 kindreds. The disease presents early in Papua New Guinea and the average age of onset in living choreics in New Britain was 21 years. Affected persons here do not as a rule seek medical advice, and an active case detection policy was therefore adopted. The development of a genetic register in an unsophisticated and superstitious community poses special problems. Genetic counselling has so far proved to be ineffective.

摘要

1977年在巴布亚新几内亚的新不列颠发现亨廷顿舞蹈症后,该国建立了全国亨廷顿病登记册。目前,在5个家族中有11名舞蹈症患者和136名潜在舞蹈症患者登记在册。这种疾病在巴布亚新几内亚发病较早,新不列颠在世的舞蹈症患者的平均发病年龄为21岁。这里的患者通常不寻求医疗建议,因此采取了积极的病例发现政策。在一个落后且迷信的社区建立基因登记册存在特殊问题。到目前为止,遗传咨询已证明是无效的。

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