上海交通大学学报（医学版）

上海交通大学学报（医学版） ›› 2017, Vol. 37 ›› Issue (10): 1389-.doi: 10.3969/j.issn.1674-8115.2017.10.016

• 论著（临床研究） • 上一篇    下一篇

表现为肾病综合征的局灶节段性肾小球硬化患儿肾组织  免疫荧光沉积情况和糖皮质激素疗效的关系

单文婕，龚莹靓，朱亚菊，金晶，董瑜，李玉峰   

  1. 上海交通大学 医学院附属新华医院小儿肾脏内科，上海 200092
  • 出版日期:2017-10-28 发布日期:2017-11-01
  • 通讯作者: 李玉峰，电子信箱：mieuniversity@hotmail.com
  • 作者简介:单文婕（1988—），女，回族，住院医师，硕士；电子信箱：emily_mail@126.com

Correlation between immunofluorescence deposition and corticosteroid effect in childhood focal segmental glomerulosclerosis manifesting with nephrotic syndrome

SHAN Wen-jie, GONG Ying-liang, ZHU Ya-ju, JIN Jing, DONG Yu, LI Yu-feng   

  1. Department of Pediatric Nephrology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
  • Online:2017-10-28 Published:2017-11-01

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摘要： 目的 · 分析表现为肾病综合征的局灶节段性肾小球硬化（FSGS）患儿肾组织免疫荧光沉积情况和糖皮质激素疗效的关系。方 法 · 回顾性分析 1990 年 1 月至 2015 年 12 月在上海交通大学医学院附属新华医院小儿肾脏内科临床诊断为肾病综合征并行肾穿刺活 检明确病理类型为 FSGS 患儿的肾组织免疫荧光沉积情况及临床随访资料。结果 · 47 例表现为肾病综合征的 FSGS 患儿，免疫荧光类 型分布：IgA 为主型2 例（占 4.26%）、 IgM 为主型7 例（占 14.89%）、补体为主型4 例（占 8.51%）、 IgG+A+M 型 1 例（占 2.13%）、 IgG+A+M+ 补体型5 例（占 10.64%）、 IgA+ 补体型1 例（占 2.13%）、 IgM+ 补体型12 例（占 25.53%）、无免疫复合物型15 例（占 31.91%）。免疫荧光沉积情况：IgA+ 有 9 例，IgM+ 有 25 例，IgG+ 有 8 例，C3+ 有 23 例，C4+ 有 3 例，C1q+ 有 6 例，FN+ 有 5 例，免疫 荧光沉积全阴性的有 12 例。口服泼尼松足量治疗 4 周后完全缓解 34 例（占 72.34%），部分缓解 7 例（占 14.89%），无缓解 6 例（占 12.77%）。不同免疫荧光类型患者间的激素疗效差异无统计学意义（H=1.792，P=0.408）；不同免疫荧光沉积情况中 C1q+ 与 C1q- 患者 的激素疗效差异有统计学意义（χ2=7.22，P=0.027），余组间差异均无统计学意义。结论 · 对于表现为肾病综合征的 FSGS 患儿，若肾 组织有 C1q 沉积，则糖皮质激素对其的疗效相对较差。

关键词: 肾病综合征, 局灶节段性肾小球硬化, 免疫荧光, 糖皮质激素, 疗效, 儿童

Abstract:

Objective · To explore the correlation between immunofluorescence (IMF) deposition and corticosteroid effect in childhood focal segmental glomerulosclerosis (FSGS) manifesting with nephrotic syndrome (NS).  Methods · Renal IMF deposition and clinical data of the children clinically diagnosed with NS and pathologically diagnosed with FSGS in the Department of Pediatric Nephrology in Xinhua Hospital, Shanghai Jiao Tong University from January 1990 to December 2015 were reviewed and analyzed retrospectively.  Results · The renal pathological types classified by IMF of 47 patients diagnosed with FSGS manifesting with NS showed that 2 cases (4.26%) were IgA type, 12 cases (14.89%) were IgM type, 4 cases (8.51%) were complement (C) type, 1 case (2.13%) was IgG+A+M type, 5 cases (10.64%) were IgG+A+M+C type, 1 case (2.13%) was IgA+C type, 12 cases (25.53%) were IgM+C type, and there was no immune complexes present in 15 cases (31.91%). The IMF deposition showed 9 cases with IgA+, 25 cases with IgM+, 8 cases with IgG+, 23 cases with C3+, 3 cases with C4+, 6 cases with C1q+, 5 cases with FN+, 12 cases with all negative. After 4 weeks of treatment with oral prednisone at full dose, complete remission was presented in 34 cases (72.34%), partial remission was presented in 7 cases (14.89%), and no remission was presented in 6 cases (12.77%). There was no statistically difference in the corticosteroid effect among the different types of IMF (H=1.792, P=0.408). The corticosteroid effect had statistical differences between C1q+ and C1q- patients (χ2=7.22, P=0.027), while it had no significant differences in other conditions.  Conclusion · In childhood FSGS manifesting with NS, C1q+ patients have relatively poor reaction to the corticosteroid therapy compared to C1q- ones.

Key words:  nephrotic syndrome, focal segmental glomerulosclerosis, immunofluorescence, corticosteroid, treatment effect, child