阿根廷儿童队列中的抗因子H自身抗体相关溶血性尿毒症综合征

Célia Dos Santos, Jesica Trinidad, Santiago Castera, Laura Alconcher, Paula Alejandra Coccia, Federico Javie Manni, María Fabiana Alberto, Analía Sánchez-Luceros
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引用次数: 0

摘要

目的:描述阿根廷第一队列患者中抗H因子(FH)自身抗体相关的非典型溶血性尿毒症综合征(aHUS)的临床特征和频率。方法:对2013 - 2022年70例疑似aHUS患儿进行fh自身抗体检测。收集fh抗体阳性和阴性患者的临床和实验室参数进行比较。结果:筛选抗fh自身抗体的70例患者临床表现为非免疫性微血管病性溶血性贫血、血小板减少和肾损伤。14名儿童发现阳性滴度[平均:1,938任意单位/mL (AU/mL),范围179-8,500]。由于缺少临床资料,2例抗fh阳性患者和20例抗fh阴性患者被排除在数据分析之外。抗fh阳性aHUS病例(n = 12)的实验室特征和临床表现与未检测自身抗体的受试者(n = 36)非常相似。在分析的12例患者中,治疗给药存在异质性。共6例患者接受透析治疗。5名儿童接受血浆置换,3名患者接受血浆置换后给予eculizumab治疗。两名患者仅接受eculizumab治疗,一名患者仅通过支持性治疗显着改善。共有8例患者接受了免疫抑制治疗。随访3例患者,治疗后及临床缓解期2/3患者抗fh自身抗体滴度明显下降。结论:本研究的70例儿科患者队列显示,阿根廷抗fh自身抗体相关aHUS的发生率为20%。在该国实施抗fh检测可能有助于改善自身免疫性aHUS患者的治疗和随访。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS.
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