Samli H, Samli M M, Yilmaz E, Imirzalioglu N
Department of Medical Biology, Faculty of Medicine, Afyon Kocatepe University, Afyon, Turkey.
Arch Androl. 2006 Nov-Dec;52(6):471-7. doi: 10.1080/01485010600691993.
Congenital bilateral absence of the vas deferens (CBAVD) is a form of infertility with an autosomal recessive genetic background in otherwise healthy males. In this study, we examined the clinical and cystic fibrosis transmembrane-conductance regulator (CFTR) gene mutations in sixty patients with bilateral absence of vas deferens that applied to andrology clinic due to male factor infertility. Urogenital anomalies of vas deferens, seminal vesicle and epididymis were detected in our patient group. CFTR gene mutations, which are known to be frequent among cystic fibrosis patients, could not be detected in our patient group with that high frequency. Delta F508 mutations were detected in only 6% of patients. IVS8 polyT alleles were positive in 68% of patients. No 1677delTA mutations and M470V variants were detected in our patient group. However, sperm retrieval is almost always possible from CBAVD patients; secondary pathologies may also result defective spermatogenesis.
先天性双侧输精管缺如(CBAVD)是一种在其他方面健康的男性中具有常染色体隐性遗传背景的不育形式。在本研究中,我们检查了60例因男性因素不育而到男科门诊就诊的双侧输精管缺如患者的临床情况及囊性纤维化跨膜传导调节因子(CFTR)基因突变情况。在我们的患者组中检测到了输精管、精囊和附睾的泌尿生殖系统异常。在囊性纤维化患者中常见的CFTR基因突变,在我们的患者组中未检测到如此高的频率。仅6%的患者检测到ΔF508突变。68%的患者IVS8多聚T等位基因呈阳性。在我们的患者组中未检测到1677delTA突变和M470V变异。然而,从CBAVD患者中几乎总能获取精子;继发性病变也可能导致生精缺陷。