Feuillet-Fieux M N, Ferrec M, Gigarel N, Thuillier L, Sermet I, Steffann J, Lenoir G, Bonnefont J P
Department of Genetic Biochemistry, Hopital Necker-Enfants Malades, Paris Cedex, France.
Clin Genet. 2004 Apr;65(4):284-7. doi: 10.1111/j.1399-0004.2004.00230.x.
Cystic fibrosis (CF) is considered as a rare disease in black Africans. In fact, this disease is likely to be underestimated since clinical features consistent with CF diagnosis are often ascribed to environmental factors such as malnutrition. Very little is known about CFTR mutations in affected patients from Central Africa. We report here four novel mutations, i.e., IVS2 + 28 (intron 2), 459T > A (exon 4), EX17a_EX18del (exons 17-18), and IVS22 + IG > A (intron 22), in such patients. An update of CFTR mutations reported in black patients from various ethnies is included. These data might be helpful for genetic counselling regarding CF in black patients.
囊性纤维化(CF)在非洲黑人中被视为罕见疾病。事实上,这种疾病可能被低估了,因为与CF诊断相符的临床特征常常被归因于营养不良等环境因素。对于来自中非的患病患者中CFTR突变情况知之甚少。我们在此报告了此类患者中的四个新突变,即IVS2 + 28(内含子2)、459T > A(外显子4)、EX17a_EX18del(外显子17 - 18)和IVS22 + IG > A(内含子22)。文中还纳入了不同种族黑人患者中所报告的CFTR突变的最新情况。这些数据可能有助于对黑人患者进行CF的遗传咨询。