BACKGROUND Congenital anomalies of the kidney and urinary tract (CAKUT) is associated with a slower progression to end-stage renal disease (ESRD) in pre-dialysis patients. However, little is known about the associated mortality risks after transitioning to dialysis. METHODS This retrospective cohort study included 0-21 year-old incident dialysis patients from the United States Renal Data System starting dialysis between 1995 and 2016. We examined the association of CAKUT vs. non-CAKUT with all-cause mortality, using Cox regression adjusted for case mix variables. We also examined the mortality risk associated with 14 non-CAKUT vs. CAKUT ESRD etiologies and under stratification by estimated glomerular filtration rate (eGFR). RESULTS Among 25,761 patients, the median (interquartile range) age was 17 (11-19) years, and 4780 (19%) had CAKUT. CAKUT was associated with lower mortality, with an adjusted hazard ratio (aHR) of 0.72 (95%CI, 0.64-0.81) (reference: non-CAKUT). In age-stratified analyses, CAKUT vs. non-CAKUT aHRs (95%CI) were 0.66 (0.54-0.80), 0.56 (0.39-0.80), 0.66 (0.50-0.86), and 0.97 (0.80-1.18) among patients < 6, 6-< 13, 13-< 18, and ≥ 18 years at dialysis initiation, respectively. Among non-CAKUT ESRD etiologies, the risk of mortality associated with primary glomerulonephritis (aHR, 0.93; 95%CI 0.80-1.09) and focal segmental glomerulosclerosis (aHR, 0.89; 95%CI, 0.75-1.04) were comparable or slightly lower compared to CAKUT, whereas most other primary causes were associated with higher mortality risk. While the CAKUT group had lower mortality risk compared to the non-CAKUT group patients with eGFR ≥5 mL/min/1.73m2, CAKUT was associated with higher mortality in patients with eGFR < 5 mL/min/1.73 m2. CONCLUSIONS CAKUT is associated with lower mortality among children < 18 years old, but showed comparable mortality with non-CAKUT among patients ≥ 18 years old. ESRD etiology should be considered in risk assessment for children initiating dialysis.

中文翻译：

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依赖透析的儿童肾脏疾病和死亡率的主要原因。

背景先天性肾脏和泌尿道异常 (CAKUT) 与透析前患者进展至终末期肾病 (ESRD) 的速度较慢有关。然而，人们对转为透析后的相关死亡风险知之甚少。方法 这项回顾性队列研究包括来自美国肾脏数据系统的 0-21 岁事件透析患者，他们在 1995 年至 2016 年间开始透析。我们检查了 CAKUT 与非 CAKUT 与全因死亡率的关联，使用 Cox 回归调整对于案例混合变量。我们还检查了与 14 种非 CAKUT 与 CAKUT ESRD 病因相关的死亡风险，并根据估计的肾小球滤过率 (eGFR) 进行分层。结果 在 25,761 名患者中，中位（四分位间距）年龄为 17 (11-19) 岁，4780 名 (19%) 患有 CAKUT。CAKUT 与较低的死亡率相关，调整后的风险比 (aHR) 为 0.72（95%CI，0.64-0.81）（参考：非 CAKUT）。在年龄分层分析中，CAKUT 与非 CAKUT 的 aHRs (95%CI) 在 <<透析开始时分别为 6、6-< 13、13-< 18 和 ≥ 18 岁。在非 CAKUT ESRD 病因中，与原发性肾小球肾炎（aHR，0.93；95%CI 0.80-1.09）和局灶节段性肾小球硬化（aHR，0.89；95%CI，0.75-1.04）相关的死亡风险相当或略低CAKUT，而大多数其他主要原因与较高的死亡风险相关。虽然与 eGFR ≥ 5 mL/min/1.73m2 的非 CAKUT 组患者相比，CAKUT 组的死亡风险较低，CAKUT 与 eGFR < 5 mL/min/1.73 m2 患者的较高死亡率相关。结论 CAKUT 与 18 岁以下儿童的较低死亡率相关，但在 ≥ 18 岁的患者中显示出与非 CAKUT 相当的死亡率。在对开始透析的儿童进行风险评估时，应考虑 ESRD 病因。