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中国循证儿科杂志

中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (1): 52-57.DOI: 10.3969/j.issn.1673-5501.2023.01.008

• 论著 • 上一篇    下一篇

单中心341例紫癜性肾炎患儿不同病理分型一致性的病例系列报告

宋纯东 1,5，王彩云2,5，宋丹3，杨晓青1，张博1，徐虹4，张霞1，杨濛1，丁樱3，任献青3，翟文生3
  

  1. 1 河南中医药大学第一附属医院郑州，450000；2 河南省新密市中医院新密，452370；3 河南中医药大学郑州，450046；4 复旦大学附属儿科医院上海，201102; 5 共同第一作者


  • 收稿日期:2022-08-22 修回日期:2022-08-31 出版日期:2023-02-25 发布日期:2023-02-25
  • 通讯作者: 张博

341 children with purpura nephritis in a single center with consistency in various pathological types: A case series report

SONG Chundong1, 5, WANG Caiyun2, 5, SONG Dan3, YANG Xiaoqing1, ZHANG Bo1, XU Hong4, ZHANG Xia1, YANG Meng1, DING Ying3, REN Xianqing3, ZHAI Wensheng3   

  1. 1 The First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Zhengzhou 450000, China; 2 Traditional Chinese Medicine in Xinmi City, Xinmi 452370, China; 3 Henan University of Traditional Chinese Medicine, Zhengzhou 450000, China; 4 Children's Hospital of Fudan University, Shanghai 201102, China; 5 Cofirst author
  • Received:2022-08-22 Revised:2022-08-31 Online:2023-02-25 Published:2023-02-25
  • Contact: ZHANG Bo，email：dianabobo@163.com

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摘要： 背景：临床常将国际儿童肾脏病研究协作组(ISKDC) 的分级标准用于紫癜性肾炎(HSPN)，但其只能反映发病时肾脏活动性炎症情况，不能反映慢性病变的情况。 目的：探讨IgA肾病牛津分类（MEST-C）评分标准在儿童HSPN中的应用价值。 设计：病例系列报告。 方法：回顾性收集2015年1月至2017年12月于河南中医药大学第一附属医院住院、年龄≤14岁、符合HSPN诊断标准且行肾活检的患儿。采集患儿的一般情况、肾活检前尿和血标本实验室检查指标、临床表现、ISKDC分级、基于病理报告的免疫荧光分型。依据病理报告结果在病理科医生指导下参照MEST-C评分标准对肾脏病理重新评估，因本研究无管状萎缩/间质纤维化(T1/T2病变)，故将肾小管间质病变分为急性（Ta）和慢性（Tc）评分，分为系膜细胞增生（M0/M1）、内皮细胞增生（E0/E1）、节段性硬化/粘连（S0/S1）、Ta0/Ta1、Tc0/Tc1、新月体形成（C0/C1/C2）组，对MEST-C与ISKDC病理分级和免疫荧光病理行kappa一致性检验。 主要结局指标：HSPN不同病理分型的一致性。 结果：①341例HSPN患儿进入本文分析，男191例，女150例，中位发病年龄9(8,11)岁，首发症状至出现尿检异常的间隔时间为10(3,21)d。②临床分型以血尿和蛋白尿型最常见，C1组血尿和蛋白尿型比例较高，M1、E1和Ta1组肾病综合征型比例较高，S1和Tc1组慢性肾炎型比例较高，P均<0.05；M1、Ta1、C2与较严重的镜下血尿有关，M1、E1、Ta1、C2与大量蛋白尿有关，P均<0.05；M1、Ta1、C1/C2与eGFR水平下降有关，P<0.05。③ISKDC分级中Ⅱ、Ⅲ级最常见，无Ⅰ、Ⅴ、Ⅵ级病例；MEST-C评分中以E1和C1病理改变较多见；MEST-C与ISKDC分级存在相关性，M1、E1、Ta1、C1/C2组ISKDC病理分级较重，S1、Tc1组ISKDC分级较轻。 结论：MEST-C评分与儿童HSPN临床表现、实验室指标的一致性符合临床预期；MEST-C病理指标均与ISKDC分级存在一致性，Ta和E指标分别与补体C3和Fib沉积严重程度存在一致性。

关键词: 儿童, 紫癜性肾炎, IgA肾病牛津分类, 临床, 病理

Abstract: Background: The International Study of Kidney Diseases in Children (ISKDC) classification is often used clinically in purpura nephritis (HSPN), but it can only reflect active inflammation of the kidney at the onset, instead of chronic leisons. Objective: To investigate the application value of the Oxford classification of IgA nephropathy (MEST-C) scale in children with HSPN. Design: Case series report. Methods: Children aged ≤14 years who were hospitalized in the First Affiliated Hospital of Henan University of Traditional Chinese Medicine from January 2015 to December 2017, who met the diagnostic criteria for HSPN and underwent renal biopsy were retrospectively collected. General information, laboratory indicators of urine and blood samples before renal biopsy, clinical manifestations, ISKDC grading, and immunofluorescence typing based on pathology reports were collected. The renal pathology was re-evaluated based on the pathology report with reference to the MEST-C scale under the guidance of the pathologist. Since there were no T1/T2 lesions in this study, the renal tubulointerstitial lesions were classified into acute (Ta) and chronic (Tc) scores. All patients were divided into the groups of mesangial cell hyperplasia (M0/M1), endothelial cell hyperplasia (E0/E1), segmental sclerosis/adhesion (S0/S1), Ta0/Ta1, Tc0/Tc1, crescent formation (C0/C1 /C2). The kappa concordance test was performed for MEST-C and ISKDC grading and immunofluorescence pathology. Main outcome measures: The consistency of different pathological types of HSPN. Results: A total of 341 children with HSPN were analyzed incuding 191 males and 150 females, with a median age of onset of 9(8,11) years and an interval of 10 (3, 21) days between the first symptoms and abnormal urine test resutls. Clinical typing was most common for hematuria and proteinuria, with a higher proportion of hematuria and proteinuria in the C1 group, a higher proportion of nephrotic syndrome in the M1, E1, and Ta1 groups, and a higher proportion of chronic nephritis in the S1 and Tc1 groups (all with P < 0.05). M1, Ta1, and C2 groups were associated with more severe microscopic hematuria(P < 0.05). M1, E1, Ta1, and C2 groups were associated with large amounts of proteinuria(P < 0.05). M1, Ta1, C1/C2 groups were associated with decreased eGFR levels(P < 0.05). ISKDC grade II and III were the most common, and there were no cases of grade I, V, and VI. E1 and C1 pathological changes were more common in MEST-C score. MEST-C was correlated with ISKDC grading. M1, E1, Ta1, C1/C2 groups had higher ISKDC grading, and that of S1 and Tc1 groups were lower. Conclusions: The consistency of MEST-C scores with clinical manifestations and laboratory indicators of HSPN in children was in accordance with clinical expectations. All MEST-C pathological indices were consistent with ISKDC grading, and Ta and E indices were consistent with the severity of complement C3 deposition and fibrinogen, respectively.

Key words: Children, Purpura nephritis, IgA nephropathy Oxford classification, Clinical, Pathology