Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure
Predictors of Incident ESRD among Patients with Primary Hyperoxaluria Presenting Prior to Kidney Failure
复制标题
DOI：
10.2215/cjn.02810315
复制
发表时间：
2016-01-01
影响因子：
9.8
通讯作者：
Milliner, Dawn S.
中科院分区：
文献类型：
--
作者：
Zhao, Fang;Bergstralh, Eric.;Milliner, Dawn S.
背景和目的原发性高尿酸血症（PH）患者体内草酸盐的过度生成导致草酸钙在肾脏的沉积，并在相当数量的病例中导致终末期肾病（ESRD）。然而，肾脏结局的关键决定因素仍不清楚。因此，我们进行了一项回顾性分析，以确定参与罕见肾结石联盟（RKSC）PH Registry.Design，设置，参与者和测量的PH患者的肾脏预后的预测因素。我们采用斯皮尔曼等级相关法评估诊断时尿指标与eGFR之间的相关性，并采用Kaplan-Meier法估计肾存活率。我们通过考克斯比例风险模型确定了与肾存活率相关的因素。结果截至2014年3月，在RKSC登记处登记的409例患者中，我们从分析中排除了112例在PH诊断时患有ESRD的患者。在其余297例患者中，65%患有PH 1型，12%患有PH 2型，13%患有PH 3型，11%患有未分类的PH。PH诊断时的年龄（第25百分位数，第75百分位数）为8.1（4.0，18.2）岁，eGFR为73.0（56.4，97.5）ml/min/1.73 m2，尿草酸排泄率为1.64（1.11，2.44）mmol/1.73 m2/24小时。在中位随访3.9（1.0，12.8）年期间，59例（20%）患者发生ESRD。按四分位数分层的诊断时尿草酸排泄与ESRD事件密切相关（风险比[HR]，3.4; 95%置信区间[95%CI]，1.4 - 7.9）。随访期间，尿草酸四分位数（Q）与ESRD事件之间存在显著相关性（Q4 vs Q1：HR，3.3; 95% CI，1.2 - 9.3）。即使调整了性别、年龄和基线eGFR，这种相关性仍然存在（HR，4.2; 95%CI，1.6 - 10.8）。结论在PH患者中，较高的尿草酸排泄量预测肾脏预后不良。
Background and objectives Overproduction of oxalate in patients with primary hyperoxaluria (PH) leads to calcium oxalate deposition in the kidney and ESRD in a substantial number of cases. However, the key determinants for renal outcome remain unclear. Thus, we performed a retrospective analysis to identify predictors for renal outcome among patients with PH participating in the Rare Kidney Stone Consortium (RKSC) PH Registry.Design, setting, participants, & measurements We characterized clinical and laboratory features of patients enrolled in the RKSC PH Registry. We assessed correlation between urinary measures and eGFR at diagnosis by Spearman rank correlation and estimated renal survival using the Kaplan-Meier method. We determined factors associated with renal survival by Cox proportional hazard models.Results Of 409 patients enrolled in the RKSC Registry as of March 2014, we excluded 112 patients who had ESRD at PH diagnosis from analysis. Among the remaining 297 patients, 65% had PH type 1, 12% had type 2, 13% had type 3, and 11% had unclassified PH. Median (25th, 75th percentile) age at PH diagnosis was 8.1 (4.0, 18.2) years with an eGFR of 73.0 (56.4, 97.5) ml/min per 1.73 m(2) and urinary oxalate excretion rate of 1.64 (1.11, 2.44) mmol/1.73 m(2) per 24 hours. During a median follow-up of 3.9 (1.0, 12.8) years, 59 (20%) patients developed ESRD. Urinary oxalate excretion at diagnosis stratified by quartile was strongly associated with incident ESRD (hazard ratio [HR], 3.4; 95% confidence interval [95% CI], 1.4 to 7.9). During follow-up there was a significant association between urinary oxalate quartile (Q) and incident ESRD (Q4 versus Q1: HR, 3.3; 95% CI, 1.2 to 9.3). This association remained even when adjusted for sex, age, and baseline eGFR (HR, 4.2; 95% CI, 1.6 to 10.8).Conclusions Among patients with PH, higher urinary oxalate excretion is predictive of poor renal outcome.