表现为大量蛋白尿的儿童原发性IgA肾病临床病理及预后分析
Analysis on clinicopathology and prognosis of primary IgA nephropathy in children with massive proteinuria
夏华 1文煜冰 2陈朝英 1涂娟 1李华荣 1耿海云 1王楠楠 1黄永莉1
作者信息
- 1. 首都儿科研究所附属儿童医院肾脏内科,北京 100020
- 2. 中国医学科学院 北京协和医学院 北京协和医院肾内科,北京 100730
- 折叠
摘要
目的 探讨表现为大量蛋白尿的儿童原发性IgA肾病(IgA nephropathy,IgAN)的临床病理特点及预后.方法 该研究为回顾性队列研究,回顾性分析2008年1月至2021年12月首都儿科研究所附属儿童医院肾脏内科收治的表现为大量蛋白尿的原发性IgAN患儿的临床资料,根据初始治疗6个月后尿蛋白是否转阴分为有效组和无效组.随访终点事件定义为蛋白尿减少小于50％或达到终末期肾病(end-stage renal disease,ESRD).采用 MedCalc 软件进行 Kaplan-Meier生存分析,并用Log-rank检验比较两组肾脏累积生存率的差异.结果 研究期间在该院肾脏内科住院的经肾活检确诊为原发性IgAN的患儿共127例,其中57例表现为大量蛋白尿,被纳入本研究,占总IgAN患儿的44.9％.57例患儿中,病理分级Lee氏Ⅲ级33例(57.9％),Lee氏Ⅲ级以下11例(19.3％),Lee氏Ⅲ级以上13例(22.8％).随访时间为4.0(3.0,5.8)年.57例患儿中46例(80.7％)初始治疗有效(有效组),11例(19.3％)初始治疗无效(无效组).与有效组比较,无效组起病时合并急性肾损伤(acute kidney injury,AKI)比例更高(7/11比13/46,x2=4.878,P=0.027),肾功能恢复时间更长[1.0(0.4,4.7)个月比0.5(0.2,0.8)个月,Z=-2.031,P=0.042],差异均有统计学意义.与有效组比较,无效组患儿Lee氏Ⅲ级以上比例更高,差异有统计学意义(5/11比8/46,x2=3.971,P=0.046).Lee氏Ⅲ级以上患儿牛津病理分型中毛细血管内细胞增殖(E1)(11/13比20/44,x2=6.204,P=0.013)、节段性肾小球硬化和/或球囊粘连(S1)(12/13比17/44,x2=11.566,P=0.001)、细胞和/或细胞纤维性新月体形成且≥25％(C2)(9/13比7/44,x2=14.131,P=0.001)与Lee氏Ⅲ级及以下患儿相比差异均有统计学意义.57例患儿中,2例发生终点事件,均为尿蛋白未缓解,没有患儿进展到ESRD.无效组加用钙调神经磷酸酶抑制剂(calcineurin inhibitors,CNIs)后,至随访终点,Kaplan-Meier生存分析和Log-rank检验结果显示,两组肾脏累积生存率差异无统计学意义(Log-rank检验,x2=0.537,P=0.460).结论 大量蛋白尿是儿童原发性IgAN的突出表现,病理类型以Lee氏Ⅲ级为主.伴有大量蛋白尿的儿童IgAN经积极治疗,中短期预后良好.对于初始治疗反应欠佳的大量蛋白尿的IgAN,起病时更常合并AKI,肾功能恢复时间更长.CNIs的应用可能对于改善大量蛋白尿的IgAN肾脏结局有一定的作用.
Abstract
Objective To investigate the clinicopathological features and the prognosis of IgA nephropathy(IgAN)in children with massive proteinuria.Methods It was a retrospective cohort study.Clinical data of IgAN children with massive proteinuria admitted to the Department of Nephrology,Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2008 to December 2021 were retrospectively analyzed.Patients were divided into effective group and ineffective group according to whether urine protein turned negative after 6 months of initial treatment.The follow-up endpoint event was defined as a reduction in proteinuria of less than 50％or end-stage renal disease(ESRD)achievement.MedCalc software was used to perform Kaplan-Meier survival analysis,and Log-rank test was used to compare the difference of renal survival between the two groups.Results A total of 127 patients were diagnosed as primary IgAN by renal biopsy,of whom 57 patients with IgAN showed massive proteinuria.These 57 IgAN patients with macroproteinuria accounted for 44.9％of the total IgAN patients and were enrolled in the study.Among the 57 cases,33 cases(57.9％)were Lee's grade Ⅲ,11 cases(19.3％)were below Lee's grade Ⅲ,and 13 cases(22.8％)were above Lee's grade Ⅲ.The follow-up time was 4.0(3.0,5.8)years.In the initial treatment,among 57 patients,46(80.7％)were effective(effective group)and 11(19.3％)were ineffective(ineffective group).Compared with the effective group,the ineffective group had a higher proportion of concurrent AKI at the onset of disease and longer recovery time of renal function,with significant difference(7/11 vs.13/46,x2=4.878,P=0.027).Compared with the effective group,the proportion of Lee grade Ⅲ or above was higher in the ineffective group,and the difference was statistically significant(5/11 vs.8/46,x2=3.971,P=0.046).There were significant differences in endocapillary hypercellularity(E1),segmental glomerulosclerosis or adhesion(S1)and cellular/fibrocellular crescents(C2)of Oxford classification between IgAN children with Lee grade Ⅲ or below and those over Lee grade Ⅲ(11/13 vs.20/44,x2=6.204,P=0.013;12/13 vs.17/44,x2=11.566,P=0.001;9/13 vs.7/44,x2=14.131,P=0.001).Among 57 patients,endpoint events occurred in 2 patients who both were urinary protein unmitigated,and none of the children progressed to ESRD.There was no significant difference in cumulative renal survival between the two groups by Kaplan-Meier survival analysis and Log-rank test(x2=0.537,P=0.460)after addition of calcineurin inhibitors(CNls)to the initial treatment ineffective group.Conclusions Macroproteinuria isthe prominent manifestation of IgAN in children.The pathological type is mainly Lee grade Ⅲ.Children with macroproteinuria have a good prognosis in the short and medium term after active treatment.For IgAN with macroproteinuria that does not respond well to initial treatment,AKI is more common at onset,and renal function recovery time is longer.The application of CNIs may have a certain effect on improving the renal outcome of IgAN with massive proteinuria.
关键词
儿童/肾小球肾炎,IgA/病理学,临床/预后/蛋白尿/钙调神经磷酸酶抑制剂Key words
Child/Glomerulonephritis,IgA/Pathology,clinical/Prognosis/Proteinuria/Calcineurin inhibitors引用本文复制引用
出版年
2024
NETL
NSTL
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