Harper P S, Walker D A, Tyler A, Newcombe R G, Davies K
Lancet. 1979 Aug 18;2(8138):346-9. doi: 10.1016/s0140-6736(79)90356-8.
A long-term programme to reduce the incidence of Huntington's Chorea has been established in South Wales, based on a study of all individuals known to be affected or at high risk in a defined population of 1.7 million people. Systematic but non-directive genetic counselling is being given to all adults at risk, accompanied by regular follow-up to provide both further information and practical support where required. The number of observed and projected new cases of the disorder born between 1900 and 1970 has remained almost constant, despite a progressive reduction in birth-rate in the general population during this period. Prospective monitoring of all births in the high-risk population will allow an estimate to be made of future trends and will show whether preventive measures are having any significant effect on the future incidence of the disorder in the population.
基于对170万特定人群中所有已知受影响或处于高风险的个体的研究,南威尔士已制定了一项长期计划,以降低亨廷顿舞蹈症的发病率。目前正在为所有有风险的成年人提供系统但非指导性的遗传咨询,并定期进行随访,以便在需要时提供更多信息和实际支持。尽管在此期间普通人群的出生率逐渐下降,但1900年至1970年间出生的该疾病的观察到的和预计的新病例数量几乎保持不变。对高风险人群中的所有出生情况进行前瞻性监测将有助于估计未来趋势,并将显示预防措施是否对该疾病在人群中的未来发病率产生任何重大影响。