Delatycki Martin B, Burke Jo, Christie Louise, Collins Felicity, Gabbett Michael, George Peter, Haan Eric, Ioannou Liane, Martin Nicole, McKenzie Fiona, O'Leary Peter, Scoble-Williams Nicole, Turner Gillian, Massie John
Clinical Genetics,Austin Health,Melbourne,Victoria,Australia.
Tasmanian Clinical Genetics Services,Hobart,Tasmania,Australia.
Twin Res Hum Genet. 2014 Dec;17(6):578-83. doi: 10.1017/thg.2014.65.
Since the discovery in 1989 that mutations in cystic fibrosis transmembrane conductance regulator (CFTR) underlie cystic fibrosis (CF), the most common life shortening genetic disorder in Caucasians, it has been possible to identify heterozygous mutation carriers at risk of having affected children. The Human Genetics Society of Australasia has produced a position statement with recommendations in relation to population-based screening for CF. These include: (1) that screening should be offered to all relatives of people with or carriers of CF (cascade testing) as well as to all couples planning to have children or who are pregnant; (2) the minimum CFTR mutation panel to be tested consists of 17 mutations which are those mutations that are associated with typical CF and occur with a frequency of 0.1% or higher among individuals diagnosed with CF in Australasia; (3) that genetic counselling is offered to all couples where both members are known to have one or two CFTR mutations and that such couples are given the opportunity to meet with a physician with expertise in the management of CF as well as a family/individual affected by the condition.
自1989年发现囊性纤维化跨膜传导调节因子(CFTR)突变是囊性纤维化(CF)的病因以来,CF是高加索人中最常见的缩短寿命的遗传性疾病,就有可能识别出有生育患病子女风险的杂合突变携带者。澳大利亚人类遗传学协会发表了一份立场声明,其中包含有关基于人群的CF筛查的建议。这些建议包括:(1)应向CF患者或携带者的所有亲属(级联检测)以及所有计划生育或已怀孕的夫妇提供筛查;(2)要检测的最小CFTR突变组包括17种突变,这些突变是与典型CF相关的突变,在澳大利亚被诊断为CF的个体中出现频率为0.1%或更高;(3)对于已知双方都有一个或两个CFTR突变的所有夫妇,应提供遗传咨询,并且应让这些夫妇有机会与CF管理方面的专家医生以及受该疾病影响的家庭/个人会面。