患有最小蛋白尿症的儿童的IgA脏病:进行活检还是不进行活检?
Alexandra Cambier1,2, Jean-Philippe Roy3, Claire Dossier4
1Division of Nephrology, Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Montreal, QC, Canada. alexandra.cambier.med@ssss.gouv.qc.ca.
Pediatric nephrology (Berlin, Germany)
|September 12, 2023
概括
患有IgA脏病 (cIgAN) 和最小蛋白尿症的儿童可能仍然存在严重的损伤. 脏活检揭示了急性和慢性淋巴细胞病变,即使尿液中没有大量的蛋白质.
科学领域:
- 儿科脏病学 儿科脏病学
- 免疫学 免疫学 免疫学
- 组织病理学 组织病理学
背景情况:
- 蛋白尿症的严重程度与儿科IgA脏病 (cIgAN) 中的管间隙病变和球炎症相关.
- 有限的数据存在于cIgAN患者的组织病理学发现中,蛋白尿是最小的或不存在的.
- 对于少量蛋白尿的cIgAN,脏活检的适用性并没有很好地定义.
研究的目的:
- 为了调查IgA脏病 (cIgAN) 呈现最小蛋白尿症的儿童的组织病理学发现.
- 评估小儿IgA瘤病例中最小蛋白尿和脏活检结果之间的相关性.
主要方法:
- 从4个中心 (巴黎和蒙特利尔) 的28名cIgAN患者脏活检的回顾性分析.
- 纳入标准:尿蛋白/肌素比 (UPCr) ≤0.03g/mmol和正常估计的淋巴细胞过率 (eGFR) >90ml/min/1.73m2在活检时.
- 显微镜和慢性组织学损伤评估,包括介质,内毛细管和外毛细管细胞增多,血球样硬化和管状缩/间歇性纤维化.
主要成果:
- 中位数年龄11.82岁,中位数随访时间4年;中位数eGFR为116毫升/分钟/1.73米2,中位数UPCr在活检时为0.02g/mmol.
- 在35.7% (微观) 和64.3% (宏观) 的血液中存在血.
- 组织学揭示了中脉 (53.5%),内毛细管 (32.1%),或外毛细管 (7.1%) 的细胞增多;球球硬化 (42.8%);管状缩/间隙纤维化 (7.1%); podocytopathic 特性 (21.4%).
- 治疗包括ACE抑制剂 (42.8%),免疫抑制疗法 (21.4%),或没有治疗 (35.7%).
- 在随访时,EGFR中位数为111.9ml/min/1.73m2,UPCr中位数为0.028g/mmol.
结论:
- 患有最小蛋白尿症的儿科IgA脏病 (cIgAN) 可能与严重的急性和慢性淋巴细胞病变有关.
- 这些发现凸显了低蛋白尿症的CigAN患者中亚临床损伤的潜力.
- 需要进一步的研究来完善cIgAN中的活检指示,以最小的蛋白尿.
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