Adams J, Mehls O, Wiesel M
Department of Urology, Ruprecht Karls University, Im Neuenheimer Feld 110, 69120 Heidelberg, Germany.
Transpl Int. 2004 Nov;17(10):596-602. doi: 10.1007/s00147-004-0784-6. Epub 2004 Oct 29.
We retrospectively reviewed our long-term experience with pediatric renal transplantation into a dysfunctional lower urinary tract to evaluate graft survival, function, and special urological complications. Between 1967 and March 2000, a total of 349 renal transplantations were performed in children younger than 18 years. Malformations of the lower urinary tract were the reasons for end-stage renal failure in 66 children (18.6%). The cause of urinary tract disorders included: meningomyelocele connected with neuropathic bladder (n = 4 transplantations); prune belly syndrome (n = 5 transplantations); VATER association (n = 2 transplantations); posterior urethral valves (n = 27 transplantations); and vesico-uretero-renal reflux (n = 28 transplantations). The majority of the patients underwent surgical interventions to preserve renal function or to prepare renal transplantation. The 1- and 5-year graft survival rate was evaluated with special reference to the underlying disease. The 1-year graft survival rate in all children with lower urinary tract malformations was 83.3%, compared with 88% for all children. In those children with vesico-ureteral reflux, it was 92.8% and in the children with Vater association and prune belly syndrome, it was 85.7%. One graft was lost in the children who had neurogenic bladder, so the 1-year graft survival rate was 75%. The worst 1-year graft survival rate was obtained for boys who had posterior urethral valves (1-year graft survival rate: 74%; 5-year graft survival rate: 62.9%). Concerning the 5-year graft survival rate, it was 70% for all children with malformations of the urinary tract. The best rate was obtained for children with reflux in the native kidneys (78.5%), followed by those with VATER association and prune belly syndrome. As an additional child with neurogenic bladder lost his graft, the 5-year graft survival rate was 50%. Pediatric renal transplantation into a dysfunctional bladder can be connected with high urological complication rates which may contribute to worse graft survival. The 1- and 5-year graft survival rate in children with malformations of the lower urinary tract is worse than in children without bladder dysfunction. We regarded a striking difference between graft survival and the urological disorders which led to renal insufficiency. We obtained the worst graft survival rates in children with posterior urethral valves which are usually connected with bladder emptying problems and dysfunctional voiding. Potential pediatric transplant recipients must be classified according to pathophysiological as well as anatomical abnormalities of the urinary tract and all urological problems have to be solved prior to transplantation. At our center, living donors are favored to plan transplantation of these children properly.
我们回顾性分析了小儿肾移植至功能不全下尿路的长期经验，以评估移植肾的存活、功能及特殊泌尿系统并发症。1967年至2000年3月期间，共对18岁以下儿童进行了349例肾移植。下尿路畸形是66例儿童（18.6%）终末期肾衰竭的原因。尿路疾病的病因包括：与神经源性膀胱相关的脊髓脊膜膨出（4例移植）；梅干腹综合征（5例移植）；VATER综合征（2例移植）；后尿道瓣膜（27例移植）；以及膀胱输尿管反流（28例移植）。大多数患者接受了手术干预以保护肾功能或为肾移植做准备。特别参照基础疾病评估了1年和5年移植肾存活率。所有下尿路畸形儿童的1年移植肾存活率为83.3%，而所有儿童的这一存活率为88%。膀胱输尿管反流患儿的1年移植肾存活率为92.8%，VATER综合征和梅干腹综合征患儿的这一存活率为85.7%。1例神经源性膀胱患儿的移植肾失功，因此其1年移植肾存活率为75%。后尿道瓣膜患儿的1年移植肾存活率最差（1年移植肾存活率：74%；5年移植肾存活率：62.9%）。关于5年移植肾存活率，所有尿路畸形儿童的这一存活率为70%。原位肾有反流的患儿这一存活率最佳（78.5%），其次是VATER综合征和梅干腹综合征患儿。由于又有1例神经源性膀胱患儿的移植肾失功，其5年移植肾存活率为50%。小儿肾移植至功能不全膀胱可能伴有较高的泌尿系统并发症发生率，这可能导致移植肾存活率降低。下尿路畸形儿童的1年和5年移植肾存活率低于无膀胱功能障碍的儿童。我们注意到移植肾存活与导致肾功能不全的泌尿系统疾病之间存在显著差异。后尿道瓣膜患儿的移植肾存活率最差，后尿道瓣膜通常与膀胱排空问题及排尿功能障碍有关。必须根据尿路的病理生理及解剖异常对潜在的小儿肾移植受者进行分类，并且在移植前必须解决所有泌尿系统问题。在我们中心，倾向于选择活体供者以便妥善规划这些儿童的移植手术。
