Hijazi Rana, Abitbol Carolyn L, Chandar Jayanthi, Seeherunvong Wacharee, Freundlich Michael, Zilleruelo Gastón
Division of Pediatric Nephrology, University of Miami/Holtz Children's Hospital, Miami, FL, USA.
J Pediatr. 2009 Jul;155(1):111-7. doi: 10.1016/j.jpeds.2009.01.007. Epub 2009 Mar 25.
To perform a retrospective analysis of the long-term outcome of infants with end-stage kidney disease (ESKD) treated at our center during the past 25 years.
The total cohort (n = 52) was divided into era 1 (1983-1995; n = 23) and era 2 (1996-2008; n = 29). Dialysis morbidity, transplantation, and long-term survival rates were assessed and compared between the 2 eras.
Average age at initiation of dialysis was 4.4 +/- 5.3 months (range, 0.5-18 months), with 96% begun on peritoneal dialysis. The predominant diagnoses were dysplasia/obstructive uropathy and autosomal recessive polycystic kidney disease. The overall survival rate is 46%, with current age of survivors ranging from 1.5 to 25 years. Mortality rates in the 2 eras were not significantly different. The predominant mortality occurred within the first year. Twenty-four patients received an initial renal transplant at 2.6 +/- 1.7 years of age. Six patients (25%) required a second renal allograft. Increased risk for mortality included African-American ethnicity, oligoanuria, autosomal recessive polycystic kidney disease, and co-morbid diagnoses.
Long-term survival is possible in infants with ESKD, although mortality and morbidity remain high. Technical innovations are needed to accommodate smaller infants undergoing dialysis. Early initiation of dialysis treatment is preferable because prognostic indicators remain poorly defined.
对过去25年在我们中心接受治疗的终末期肾病（ESKD）婴儿的长期预后进行回顾性分析。
将整个队列（n = 52）分为第1阶段（1983 - 1995年；n = 23）和第2阶段（1996 - 2008年；n = 29）。评估并比较两个阶段的透析并发症、移植情况和长期生存率。
开始透析的平均年龄为4.4±5.3个月（范围0.5 - 18个月），96%开始接受腹膜透析。主要诊断为发育异常/梗阻性尿路病和常染色体隐性多囊肾病。总体生存率为46%，存活者目前年龄在1.5至25岁之间。两个阶段的死亡率无显著差异。主要死亡发生在第一年。24例患者在2.6±1.7岁时接受了首次肾移植。6例患者（25%）需要第二次肾移植。死亡风险增加包括非裔美国人种族、少尿、常染色体隐性多囊肾病和合并诊断。
ESKD婴儿有可能实现长期存活，尽管死亡率和发病率仍然很高。需要技术创新来适应更小的接受透析的婴儿。由于预后指标仍不明确，早期开始透析治疗较为可取。
