Ozbey H, Boneval C, Kapran Y, Aksöyek S, Salman T, Celik A
Department of Pediatric Surgery, University of Istanbul, Istanbul Faculty of Medicine, Turkey.
Eur J Pediatr Surg. 1996 Jun;6(3):186-8. doi: 10.1055/s-2008-1066505.
The combination of Wilms' tumor with aniridia, ambiguous genitalia/gonadoblastoma and mental retardation is defined as WAGR syndrome. The association with pseudohermaphroditism and nephropathy was also described as Drash syndrome. Here we report two cases with WAGR and Drash syndrome. The importance of early recognition and appropriate management of Wilms' tumor with those concurrent malformations is highlighted.
肾母细胞瘤合并无虹膜、生殖器模糊/性腺母细胞瘤及智力发育迟缓被定义为WAGR综合征。与假两性畸形和肾病的关联也被描述为Drash综合征。在此我们报告两例WAGR和Drash综合征病例。强调了早期识别以及对伴有这些并发畸形的肾母细胞瘤进行恰当管理的重要性。
