ESPN/ERA-EDTA Registry, Academic Medical Center, Amsterdam, The Netherlands.
1] Department for Pediatric Nephrology and Hypertension, Medical University of Gdansk, Gdansk Poland [2] Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Heidelberg, Germany.
Kidney Int. 2014 Jul;86(1):168-74. doi: 10.1038/ki.2013.561. Epub 2014 Feb 5.
End-stage renal disease requiring renal replacement therapy (RRT) during the neonatal period is a very rare condition, and little information is available regarding long-term RRT and outcomes. To gain more information, we performed a collaborative study on patient characteristics and treatment outcomes in children who started RRT as neonates during their first month of life between 2000 and 2011 who were prospectively registered in the ESPN/ERA-EDTA, the IPPN (since 2007), the Japanese registry, or the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. During the first month of life, 264 patients from 32 countries started RRT and were followed for a median of 29 months (interquartile range 11-60 months). Most neonates (242) started on peritoneal dialysis, 21 started on hemodialysis, and 1 patient with a transplant. The most important causes of renal failure were congenital anomalies of the kidney and urinary tract in 141, cystic kidneys in 35, and cortical necrosis in 30. Within 2 years after the start of RRT, 69 children changed dialysis modality and 53 received a renal transplant. After a median of 7 months, 45 children had died, mainly because of infection, resulting in an estimated 2-year survival of 81%, and 5-year survival of 76%. Growth retardation (63%), anemia (55%), and hypertension (57%) were still major problems after 2 years. Thus, relatively good medium-term patient survival may be achieved with RRT started during the neonatal period, but specific therapeutic challenges continue to exist in this age group.
需要在新生儿期进行肾脏替代治疗（RRT）的终末期肾病是一种非常罕见的情况，关于长期 RRT 和结局的信息很少。为了获得更多信息，我们对 2000 年至 2011 年间在 ESPN/ERA-EDTA、IPPN（自 2007 年起）、日本登记处或澳大利亚和新西兰透析和移植（ANZDATA）登记处前瞻性登记的在生命的第一个月开始接受 RRT 的新生儿患者的特征和治疗结局进行了合作研究。在生命的第一个月，来自 32 个国家的 264 名患者开始接受 RRT，中位随访时间为 29 个月（四分位距 11-60 个月）。大多数新生儿（242 名）开始腹膜透析，21 名开始血液透析，1 名接受移植。肾衰竭的最重要原因是肾脏和尿路先天性畸形 141 例，囊性肾病 35 例，皮质坏死 30 例。在开始 RRT 后的 2 年内，69 名儿童改变了透析方式，53 名接受了肾移植。中位数为 7 个月后，45 名儿童死亡，主要死于感染，估计 2 年生存率为 81%，5 年生存率为 76%。生长迟缓（63%）、贫血（55%）和高血压（57%）在 2 年后仍然是主要问题。因此，在新生儿期开始 RRT 可能会获得相对较好的中期患者生存率，但该年龄组仍存在特定的治疗挑战。
