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

作　　者：夏华 文煜冰[2] 陈朝英[1] 涂娟[1] 李华荣[1] 耿海云[1] 王楠楠 黄永莉 Xia Hua;Wen Yubing;Chen Chaoying;Tu Juan;Li Huarong;Geng Haiyun;Wang Nannan;Huang Yongli(Department of Nephrology,Children's Hospital,Capital Institute of Pediatrics,Beijing 100020,China;Department of Nephrology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Science,Beijing 100730,China)

机构地区：[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

出　　处：《中华肾脏病杂志》2024年第1期36-41,共6页Chinese Journal of Nephrology

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,χ^(2)=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,χ^(2)=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,χ^(2)=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,χ^(2)=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,χ^(2)=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�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, χ^(2)=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, χ^(2)=3.971, P=0.046). There were significant differences in endocapillary hypercellularity (E1), segmental glomerulosclerosis or adhesion (S1) and cellular/fibrocel

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 

分 类 号：R726.9[医药卫生—儿科]