儿童原发性局灶节段性肾小球硬化症的长期预后分析  

Long-term prognosis of primary focal segmental glomerulosclerosis in children

作　　者：彭映潮 高春林 孙涛 张沛 夏正坤 Peng Yingchao;Gao Chunlin;Sun Tao;Zhang Pei;Xia Zhengkun(Department of Pediatrics,Jinling Hospital,the First School of Clinical Medicine,Southern Medical University,Nanjing 210002,China)

机构地区：[1]南方医科大学第一临床医学院(解放军东部战区总医院)儿科,南京210002

出　　处：《中华儿科杂志》2022年第9期894-900,共7页Chinese Journal of Pediatrics

基　　金：江苏省重点研发计划临床前沿技术(BE2017719);江苏省儿科医学创新团队(CXTDA2017022);江苏省基础研究计划(自然科学基金)青年基金项目(BK20190251)。

摘　　要：目的探讨儿童原发性局灶节段性肾小球硬化症(FSGS)的长期预后及与肾脏预后相关的危险因素。方法回顾性研究,收集2003年1月至2019年12月于东部战区总医院儿科经肾活检术确诊为原发性FSGS的124例患儿的一般情况、临床分型、肾活检结果等临床资料。通过Kaplan-Meier生存分析计算患儿的肾脏累积生存率,Cox回归风险模型、受试者工作特征(ROC)曲线等方法寻找和肾脏预后相关的危险因素。结果124例患儿中男94例(75.8%),女30例(24.2%),患儿肾活检时年龄为16(14,17)岁,13~18岁102例(82.3%);随访时间为64.8(32.1,86.0)个月。124例患儿中非特殊型49例(39.5%)、顶端型33例(26.6%)、塌陷型22例(17.7%)、细胞型14例(11.3%)、门周型6例(4.8%)。Kaplan-Meier生存分析显示,肾活检后5、10和15年进入终末期肾病(ESKD)或估算肾小球滤过率(eGFR)较基线下降≥50%的患儿肾脏累积生存率分别为66.9%、51.4%、21.0%。多因素Cox回归分析显示,高血压、肾小球节段硬化比例、中重度慢性小管间质病变均是儿童FSGS进入ESKD或eGFR较基线值下降≥50%的独立危险因素(HR=5.28、1.03、7.81,95%CI 2.77~10.05、1.01~1.04、4.08~14.98,均P<0.01)。ROC曲线分析显示,肾小球节段硬化比例(AUC=0.734,P<0.05,最佳截断值25.4%,灵敏度50.0%,特异度88.6%)、中重度慢性肾小管间质病变(AUC=0.724,P<0.05,灵敏度46.3%,特异度98.6%)具有良好的评估FSGS肾脏结局的效能。结论儿童FSGS的长期预后不佳,预后不良的危险因素为高血压、中重度慢性肾小管间质病变、肾小球节段硬化比例≥25.4%。Objective To investigate risk factors for the long-term prognosis of primary focal segmental glomerulosclerosis(FSGS)and associated with renal prognosis in children.Methods A retrospective study was conducted by collecting clinical data including general information,clinical features and renal pathological findings of 124 children with primary FSGS in Department of Pediatrics of Jinling Hospital from January 2003 to December 2019.The cumulative renal survival rate was calculated by Kaplan-Meier survival analysis.The risk factors related to renal prognosis were identified by Cox regression risk model analysis and receiver operating characteristic(ROC)curve.Results Among 124 children,94 were males(75.8%)and 30 were females(24.2%).The children were 16(14,17)years of age at the time of kidney biopsies.There were 102 cases(82.3%)aged from 13 to 18 years.The period of follow-up was 64.8(32.1,86.0)months.There were 49 cases(39.5%)with nonspecific variant,33 cases(26.6%)with tip variant,22 cases(17.7%)with collapsing variant,14 cases(11.3%)with cellular variant and 6 cases(4.8%)with periportal variant.The data of Kaplan-Meier survival analysis showed that cumulative renal survival rates of end-stage kidney disease(ESKD)or≥50%decline in estimated glomerular filtration rate(eGFR)from baseline at the year of 5,10 and 15 after renal biopsies were 66.9%,51.4%and 21.0%respectively.Multivariate Cox regression analysis showed that hypertension,glomerular segmental sclerosis ratio,moderate to severe chronic tubulointerstitial lesions were independent risk factors for progressing to ESKD or≥50%reduction in eGFR from baseline in pediatric FSGS(HR=5.28,1.03,7.81,95%CI 2.77-10.05,1.01-1.04,4.08-14.98,all P<0.01).ROC curve analysis showed glomerular segmental sclerosis ratio(AUC=0.734,P<0.05,optimal cut-off value=25.4%,sensitivity=50.0%,specificity=88.6%),moderate and severe chronic renal tubulointerstitial lesions(AUC=0.724,P<0.05,sensitivity=46.3%,specificity=98.6%)had good efficacy in evaluating renal outcomes of FSGS.Conclusio

关 键 词：肾小球硬化症 局灶节段性 儿童 预后 危险因素 

分 类 号：R726.9[医药卫生—儿科]