儿童与成人原发性IgA肾病患者临床及病理特征比较及其临床意义  

Comparisons of clinical and pathological characteristics between children and adults with primary IgA nephropathy and their clinical significances

作　　者：代思雨 宋丹 宋纯东[1] 段凤阳[1] 王宁丽 郭婷[1] 张博[1] 杨晓青[1] 徐虹[4] DAI Siyu;SONG Dan;SONG Chundong;DUAN Fengyang;WANG Ningli;GUO Ting;ZHANG Bo;YANG Xiaoqing;XU Hong(Department of Pediatrics,First Affiliated Hospital,Henan University of Traditional Chinese Medicine,Zhengzhou 450000,China;School of Pediatrics,Henan University of Traditional Chinese Medicine,Zhengzhou 450046,China;School of Acupuncture and Moxibustion and Massage,Henan University of Traditional Chinese Medicine Zhengzhou 450046,China;National Children’s Medical Center,Affiliated Pediatric Hospital,Fudan University,Shanghai 201102,China)

机构地区：[1]河南中医药大学第一附属医院儿科,河南郑州450000 [2]河南中医药大学儿科医学院,河南郑州450046 [3]河南中医药大学针灸推拿学院,河南郑州450046 [4]复旦大学附属儿科医院国家儿童医学中心,上海201102

出　　处：《吉林大学学报（医学版）》2023年第3期722-732,共11页Journal of Jilin University：Medicine Edition

基　　金：国家自然科学基金项目(82074493);河南省科技厅科技攻关项目(202102310494);河南省卫健委中医药学科领军人才项目[豫卫中医函(2021) 8号];河南省中医管理局国家中医临床研究基地科研专项重点项目(2018JDZX119);河南省中医管理局国家中医临床研究基地科研专项普通项目(2018JDZX017);朗润医药科技有限公司横向项目(KY-A0332)。

摘　　要：目的:探讨儿童免疫球蛋白A (IgA)肾病(IgAN)和成人IgAN患者临床及病理特征,并阐明其临床意义。方法:收集400例原发性IgAN肾病患者的临床和病理资料,根据患者年龄分为儿童组(n=160)和成人组(n=240),比较2组患者年龄、性别、高血压发生率、发病至肾活检时间、估计肾小球滤过率(eGFR)、初次发病表现、临床分型、Lee氏分级、牛津分类标准(MEST-C)评分、免疫荧光分型和肾小球超微结构。结果:儿童组患者男女比例为1.5:1,成人组患者男女比例为1.1:1;与成人组比较,儿童组患者高血压发生率明显降低(P<0.01),发病至肾活检时间明显缩短(P<0.01),eGFR明显升高(P<0.01)。与成人组比较,儿童组患者肉眼血尿和浮肿所占百分率明显升高(P<0.01),尿检异常、泡沫尿和其他表现所占百分率明显降低(P<0.01)。与成人组比较,儿童组患者孤立性血尿型及肾病综合征型所占百分率明显升高(P<0.01),孤立性蛋白尿型及慢性肾炎型所占百分率明显降低(P<0.01)。与成人组比较,儿童组患者Lee氏分级Ⅱ级所占百分率明显升高(P<0.01),Lee氏分级V级所占百分率明显降低(P<0.01)。光镜下观察,儿童组患者Ⅱ级肾组织中仅有局灶性的系膜细胞和系膜基质增生,极少伴有肾小球硬化,肾小管与肾间质病变不明显;成人组患者V级肾组织中系膜细胞和系膜基质增生明显,有较多肾小球硬化,肾小管和肾间质病变相对较重。与成人组比较,儿童组患者MEST-C评分M1和E1患者百分率明显升高(P<0.05或P<0.01),S1和T1/T2患者百分率明显降低(P<0.01)。儿童组患者尿蛋白分级与M(r_(s)=0.462)、E (r_(s)=0.342)和C (r_(s)=0.250)评分呈明显正相关关系(P<0.01),成人组尿蛋白分级与M (r_(s)=0.217)、E (r_(s)=0.145)、S (r_(s)=0.187)、T (r_(s)=0.269)和C (r_(s)=0.256)评分呈明显正相关关系(P<0.05或P<0.01)。与成人组比较,儿童组患者IgA+IgG+IgM沉积增加(P<0.05),C3沉积率明显降低(P<0.01Objective:To discuss the clinical and pathological characteristics of IgA nephropathy(IgAN)in the children and adults,and to clarify their clinical significances.Methods:The clinical and pathological data of the patients diagnosed with primary IgAN were collected.According to their ages,the patients were divided into children group(n=160)and adult group(n=240).The ages,genders,incidences of hypertension,time from onset to renal biopsy,estimated glomerular filtration rates,initial onset manifestations,clinical types,Lee’s grades,MEST-C scores,immunofluorescence types and ultrastructures of glomeruli of the patients in two groups were compared.Results:The ratio of male to female in children group was 1.5∶1 and it was 1.1∶1 in adults group;compared with adult group,the incidence of hypertension and the time from onset to renal biopsy of the patients in child group were decreased(P<0.05),while the eGFR was increased(P<0.05).Compared with adult group,the percentages of gross hematuria and edema of the patients in child group were increased(P<0.01),and the percentages of abnormal urine test,foam urine,and other manifestations were decreased(P<0.01).Compared with adult group,the percentages of the patients with isolated hematuria and nephrotic syndrome in child group were increased(P<0.01),while the percentages of the patients with isolated proteinuria and chronic nephritis were decreased(P<0.01);compared with adult group,the percentage of the patients with gradeⅡin the Lee’s grades in child group was increased(P<0.01)and the percentage of the patients with gradeⅤin the Lee’s grades was decreased(P<0.01).The light microscope obervation results showed that there were only focal mesangial cells and mesangial matrix hyperplasia in gradeⅡrenal tissue of the patients in child group,rarely accompanied by glomerulosclerosis.The renal tubular and interstitial lesions were not significant;there were significant proliferations of the mesangial cells and matrix in gradeⅤrenal tissue of the patients in adult group

关 键 词：原发性IGA肾病 儿童 成人 临床分型 Lee氏分级 

分 类 号：R692.3[医药卫生—泌尿科学]