Verlingue C, David A, Audrezet M P, Le Roux M G, Mercier B, Moisan J P, Ferec C
Centre de Biogénétique, Centre Départemental de Transfusion Sanguine, Brest.
Prenat Diagn. 1993 Dec;13(12):1143-8. doi: 10.1002/pd.1970131210.
The cystic fibrosis (CF) gene has been observed to have the highest frequency of mutations in the Caucasian population. Prenatal diagnosis can now be performed with a high degree of accuracy since the identification of most of the gene's mutations, as well as the characterization of intragenic markers. However, the observation of a distribution of clinical phenotypes increases the need to identify a mild phenotype and avoid false-negative diagnosis. By screening most of the exons of the CFTR gene, we showed that a supposed obligate carrier of CF was in fact an asymptomatic affected woman.
囊性纤维化(CF)基因在白种人群中具有最高的突变频率。由于该基因的大多数突变已被识别,以及基因内标记的特征已被确定,现在可以进行高度准确的产前诊断。然而,临床表型分布的观察增加了识别轻度表型并避免假阴性诊断的必要性。通过筛查CFTR基因的大多数外显子,我们发现一名被认为是CF必然携带者的女性实际上是一名无症状的患病女性。