抗H因子抗体阳性溶血尿毒综合征7例分析

doi:10.6040/j.issn.1671-7554.0.2021.0589

摘要/Abstract

摘要： 目的抗H因子抗体阳性的溶血尿毒综合征是溶血尿毒综合征(HUS)中的严重亚型,通过探讨患儿的临床特点、治疗及转归情况,为临床诊治提供依据。方法回顾性收集2011年5月至2017年12月收治的7例抗H因子抗体阳性HUS患儿临床资料,其中男5例、女2例,5.83~13.5岁,平均7.93岁,分析其一般情况、前驱感染、临床特点、治疗及转归,并随访3~8年。结果前驱感染:发热、咳嗽等上呼吸道感染症状占57.14%(4/7),呕吐、腹痛等消化系统症状占71.42%(5/7);临床特点:所有患儿均抗H因子抗体阳性、ADAMTS13活性正常,H因子浓度2例正常,5例降低;7例均呈现中至重度贫血、血小板减少症、高胆红素血症、大量蛋白尿、严重的急性肾损害及低补体C3血症。3例行肾穿刺活检,3例行基因检测。治疗与转归:急性期7例患儿均早期给予丙种球蛋白、血浆置换联合大剂量甲强龙冲击治疗,后序贯口服糖皮质激素,1例死亡,6例缓解(平均治疗27.83 d)。5例因尿常规持续异常加用免疫抑制剂治疗。随访3~8年,4例完全缓解,2例部分缓解。4例复测抗H因子抗体2例转阴性,2例仍阳性。结论抗H因子抗体阳性HUS患儿临床表现重,急性期在对症支持治疗基础上,早期积极使用血浆置换、丙种球蛋白,并联合大剂量甲强龙冲击治疗,可迅速控制病情,续贯长疗程应用维持性免疫抑制治疗,可减少复发,延长患儿缓解期。抗H因子抗体和补体C3有可能作为预后监测指标。

关键词: 溶血尿毒综合征, 抗H因子抗体, 临床特点, 治疗, 随访

Abstract: Objective Anti-H factor antibody-positive hemolytic uremic syndrome(HUS)is a severe subtype of HUS. The clinical characteristics, treatment and outcome of children with such disease were discussed to provide reference for clinical diagnosis and treatment. Methods Clinical data of 7 patients with anti-H factor antibody-positive HUS during May 2011 and Dec. 2017 were retrospectively analyzed, including 5 male and 2 female, aged 5.83 to 13.5(mean 7.93)years. The general condition, pre-infection, clinical characteristics, treatment, outcome and 3-8-year follow-up results were analyzed. Results Pre-infection: upper respiratory tract infection symptoms such as fever and cough were discovered in 57.14%(4/7)patients; digestive system symptoms such as vomiting and abdominal pain manifested in 71.42%(5/7)patients. Clinical features: all children were positive for anti-H factor antibody, ADAMTS13 activity was normal, 2 cases of H factor titer were normal, and the other were decreased; All cases showed moderate to severe anemia, thrombocytopenia, hyperbilirubinemia, massive proteinuria, severe acute kidney damage, and low-complement C3. Renal biopsy was performed in 3 cases and genetic testing in 3 cases. Treatment and outcome: all patients in the acute phase were treated with Gamma globulin, plasma exchange and high-dose methylprednisolone, followed by oral glucocorticoids. The outcome was 1 death and 6 relief, with the mean treatment time of 27.83 days. Five patients were treated with immunosuppressive agents due to persistent abnormalities in urine routine. After 3-8 years of follow-up, 4 patients had complete remission and 2 had partial remission; of the 4 cases who repeated amti H factor antibody examination, 2 were negative and 2 were still positive. Conclusion Children with anti-H factor antibody-positive HUS have severe clinical manifestations. On the basis of symptomatic supportive therapy in the acute phase, early use of plasma exchange, Gamma globulin, combined with high-dose methylprednisol impact therapy, can quickly control the condition. Long-term application of immunosuppressive agents can reduce recurrence and prolong the remission period. H factor antibody and C3 may be used as indicators of prognostic monitoring.

Key words: Hemolytic uremic syndrome, Anti-H factor antibody, Clinical characteristics, Treatment, Follow-up

中图分类号:

R574

孔昕欣,孙书珍,李倩,陈元,周爱华,王莉,姚秀俊. 抗H因子抗体阳性溶血尿毒综合征7例分析[J]. 山东大学学报 (医学版), 2022, 60(4): 82-86.

KONG Xinxin, SUN Shuzhen, LI Qian, CHEN Yuan, ZHOU Aihua, WANG Li, YAO Xiujun. Analysis of 7 cases of anti-H factor antibody-positive hemolytic uremic syndrome[J]. Journal of Shandong University (Health Sciences), 2022, 60(4): 82-86.

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