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[原发性膜性肾病患儿肾小球中的C1q或IgA沉积]

[C1q or IgA deposition in glomeruli of children with primary membranous nephropathy].

作者信息

Xu K, Wang F, Wang Z H, Sun L Y, Yao Y, Xiao H J, Liu X Y, Su B G, Zhong X H, Guan N, Zhang H W, Ding J

机构信息

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

出版信息

Zhonghua Er Ke Za Zhi. 2022 Sep 2;60(9):901-907. doi: 10.3760/cma.j.cn112140-20220505-00411.

Abstract

To assess the correlation of glomerular C1q or IgA deposition with clinical and pathological features of primary membranous nephropathy (PMN) in children. The clinical and pathological manifestations including (phospholipase A2 receptor, PLA2R) and IgG subclasses staining in renal biopsies, serum anti-PLA2R antibody and therapeutic response of 33 children diagnosed with PMN in Peking University First Hospital from December 2012 to December 2020 were retrospectively summarized and analyzed. According to results of PLA2R test and findings renal pathological, the patients were divided into PLA2R-related group and non-PLA2R-related group, typical MN group and atypical MN group, C1q deposit group and non-C1q deposit group, as well as IgA deposit group and non-IgA deposit group respectively. T-test, Mann-Whitney test and Fisher's exact probability test were used for comparison between the groups. Among the 33 children with PMN, there were 20 males and 13 females, of that the age of onset was 11 (8, 13) years, and 32 patients had nephrotic level proteinuria. Renal biopsies were performed at 4.6 (2.1, 11.6) months after onset, and 28 patients (85%) received glucocorticoid or immunosuppressive therapy prior to renal biopsy. There were 20 cases (61%) with PLA2R-related MN and 13 cases (39%) with non-PLA2R-related MN. Compared with the non-PLA2R-related group, the PLA2R-related group had an older age of onset (12 (10, 13) 7 (3, 12) years, 2.52, 0.011), a lower preceding infection rate (45% (9/20) . 11/13, 0.032) and lower spontaneous remission rate (0 . 4/13, 0.017). Renal PLA2R positivity was significantly associated with predominant or co-deposition of IgG4 (13/17 5/15, =0.031) and low albumin levels at renal biopsy ((25±6) (29±7) g/L, 2.14, 0.041). There were 12 patients with typical PMN and 21 patients with atypical PMN, and no significant difference in clinical and pathological manifestations was found between these 2 groups (all >0.05). There were 10 cases (32.3%) with glomerular C1q deposition, and their disease course before renal biopsy was significantly shorter than those without C1q deposition (1.8 (0.8, 5.9) 6.0 (2.5, 22.3) months, 2.27, 0.023). Twelve cases (36.4%) had glomerular IgA deposition, and their course of disease,clinical and pathological manifestations were not significantly different from those without IgA deposition (all >0.05). Glomerular C1q or IgA deposition may not affect the clinical manifestations, glomerular PLA2R and IgG subclasses staining pattern, or the response to treatment of PMN in children.

摘要

评估儿童原发性膜性肾病(PMN)肾小球C1q或IgA沉积与临床及病理特征的相关性。回顾性总结并分析2012年12月至2020年12月在北京大学第一医院确诊为PMN的33例儿童的临床和病理表现,包括肾活检中的(磷脂酶A2受体,PLA2R)及IgG亚类染色、血清抗PLA2R抗体和治疗反应。根据PLA2R检测结果和肾脏病理表现,将患者分别分为PLA2R相关组和非PLA2R相关组、典型MN组和非典型MN组、C1q沉积组和非C1q沉积组以及IgA沉积组和非IgA沉积组。采用t检验、Mann-Whitney检验和Fisher确切概率检验进行组间比较。33例PMN患儿中,男20例,女13例,发病年龄为11(8,13)岁,32例患者有肾病水平蛋白尿。发病后4.6(2.1,11.6)个月进行肾活检,28例(85%)患者在肾活检前接受了糖皮质激素或免疫抑制治疗。有20例(61%)为PLA2R相关MN,13例(39%)为非PLA2R相关MN。与非PLA2R相关组相比,PLA2R相关组发病年龄较大(12(10,13)岁对7(3,12)岁,t = 2.52,P = 0.011),前驱感染率较低(45%(9/20)对84.6%(11/13),P = 0.032),自发缓解率较低(0对30.8%(4/13),P = 0.017)。肾PLA2R阳性与IgG4的主要或共沉积(13/17对5/15,P = 0.031)及肾活检时低白蛋白水平显著相关((25±6)g/L对(29±7)g/L,t = 2.14,P = 0.041)。有12例典型PMN和21例非典型PMN,两组临床和病理表现无显著差异(均P>0.05)。有10例(32.3%)肾小球有C1q沉积,其肾活检前病程明显短于无C1q沉积者(1.8(0.8,5.9)个月对6.0(2.5,22.3)个月,t = 2.27,P = 0.023)。12例(36.4%)有肾小球IgA沉积,其病程、临床和病理表现与无IgA沉积者无显著差异(均P>0.05)。肾小球C1q或IgA沉积可能不影响儿童PMN的临床表现、肾小球PLA2R及IgG亚类染色模式或治疗反应。

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