Harold Simmons Center for Kidney Disease Research and Epidemiology, Division of Nephrology and Hypertension, University of California Irvine Medical Center, 101 The City Drive South, City Tower, Suite 400, Orange, CA, 92868, USA.
Department of Pediatrics, Kitasato University School of Medicine, 1 Chome-15-1 Kitazato, Minami Ward, Sagamihara, Kanagawa, 252-0374, Japan.
Pediatr Nephrol. 2020 May;35(5):851-860. doi: 10.1007/s00467-019-04457-7. Epub 2020 Feb 4.
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.
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).
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/1.73m, CAKUT was associated with higher mortality in patients with eGFR < 5 mL/min/1.73 m.
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.
先天性肾和尿路异常（CAKUT）与透析前患者终末期肾病（ESRD）进展较慢有关。然而，在转为透析后，与死亡率相关的风险知之甚少。
本回顾性队列研究纳入了美国肾脏数据系统中从 1995 年到 2016 年期间开始透析的 0-21 岁的新透析患者。我们使用 Cox 回归调整病例组合变量，研究 CAKUT 与非 CAKUT 与全因死亡率之间的关系。我们还检查了 14 种非 CAKUT 与 CAKUT ESRD 病因之间的死亡率风险，并按估计肾小球滤过率（eGFR）进行分层。
在 25761 名患者中，中位（四分位距）年龄为 17 岁（11-19 岁），4780 名（19%）有 CAKUT。CAKUT 与较低的死亡率相关，调整后的危险比（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 aHR（95%CI）分别为 0.66（0.54-0.80）、0.56（0.39-0.80）、0.66（0.50-0.86）和 0.97（0.80-1.18），分别为<6 岁、6-<13 岁、13-<18 岁和≥18 岁的患者。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症（aHR，0.89；95%CI，0.75-1.04）相关的死亡率风险相当或略低，而大多数其他原发性病因与更高的死亡率风险相关。与非 CAKUT 组相比，CAKUT 组的 eGFR≥5 mL/min/1.73m 患者的死亡率较低，但 eGFR<5 mL/min/1.73 m 的 CAKUT 患者的死亡率较高。
CAKUT 与<18 岁儿童的死亡率较低有关，但≥18 岁患者的死亡率与非 CAKUT 相似。在评估开始透析的儿童的风险时，应考虑 ESRD 的病因。
