Department of Clinical Genetics, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
Sci Rep. 2017 Nov 6;7(1):14595. doi: 10.1038/s41598-017-15062-9.
Vesicoureteric reflux (VUR) is the commonest urological anomaly in children. Despite treatment improvements, associated renal lesions - congenital dysplasia, acquired scarring or both - are a common cause of childhood hypertension and renal failure. Primary VUR is familial, with transmission rate and sibling risk both approaching 50%, and appears highly genetically heterogeneous. It is often associated with other developmental anomalies of the urinary tract, emphasising its etiology as a disorder of urogenital tract development. We conducted a genome-wide linkage and association study in three European populations to search for loci predisposing to VUR. Family-based association analysis of 1098 parent-affected-child trios and case/control association analysis of 1147 cases and 3789 controls did not reveal any compelling associations, but parametric linkage analysis of 460 families (1062 affected individuals) under a dominant model identified a single region, on 10q26, that showed strong linkage (HLOD = 4.90; ZLRLOD = 4.39) to VUR. The ~9Mb region contains 69 genes, including some good biological candidates. Resequencing this region in selected individuals did not clearly implicate any gene but FOXI2, FANK1 and GLRX3 remain candidates for further investigation. This, the largest genetic study of VUR to date, highlights the 10q26 region as a major genetic contributor to VUR in European populations.
膀胱输尿管反流(VUR)是儿童中最常见的泌尿科异常。尽管治疗有所改善,但相关的肾脏病变——先天性发育不良、后天性瘢痕形成或两者兼有——仍是儿童高血压和肾衰竭的常见原因。原发性 VUR 具有家族遗传性,遗传率和同胞风险均接近 50%,且表现出高度的遗传异质性。它常与泌尿道其他发育异常有关,强调其病因是泌尿道生殖系统发育障碍。我们在三个欧洲人群中进行了全基因组连锁和关联研究,以寻找易患 VUR 的基因座。对 1098 个父母-受影响子女三体型家系进行基于家系的关联分析,对 1147 例病例和 3789 例对照进行病例对照关联分析,均未发现任何引人注目的关联,但在显性模型下对 460 个家系(1062 个受影响个体)进行参数连锁分析,确定了一个位于 10q26 上的单一区域,与 VUR 有很强的连锁(HLOD = 4.90;ZLRLOD = 4.39)。该 9Mb 区域包含 69 个基因,包括一些很好的生物学候选基因。在选定个体中对该区域进行重测序并没有明确暗示任何基因,但 FOXI2、FANK1 和 GLRX3 仍然是进一步研究的候选基因。这是迄今为止对 VUR 进行的最大规模的遗传研究,突出了 10q26 区域是欧洲人群 VUR 的主要遗传贡献者。