儿童溶血尿毒综合征4例报道并文献回顾

    Hemolytic uremic syndrome in children: a report of four cases with a literature review

      摘要
    • 摘要: 溶血尿毒综合征（hemolytic uremic syndrome，HUS）是小儿急性肾衰竭常见原因之一，死亡率高。本文通过分析总结4例HUS患儿临床特点及诊治经过，为提高诊疗水平提供参考。方法 将湖北省妇幼保健院PICU收治的4例HUS患儿临床表现、辅助检查、治疗及转归进行回顾性分析。结果 4例患儿均进行血液净化治疗，2例患儿恢复良好；1例患儿出院后10个月复发，再次住院好转出院，随访5个月身体健康。1例患儿住院治疗68 d，间断透析治疗，因预后差放弃治疗后死亡。结论 HUS为儿童致命性疾病，血浆置换能够缓解病情、改善预后，为HUS一线治疗。婴儿期发生非典型HUS可出现血浆置换治疗不敏感，建议尽早采用其他治疗如依库珠单抗。

       

      Abstract: Objective With a high fatality rate,hemolytic uremic syndrome(HUS) is one of the common causes of acute renal failure in children.The clinical characteristics and managements of pediatric HUS were summarized.Methods Clinical manifestations,auxiliary examinations,treatments and outcomes of 4 HUS children were retrospectively reviewed.Results After blood purification treatment, two children recovered well.One case recurred at Month 10 after discharge and regained health at Month 5 after readmission.One hospitalized child died after 68-day intermittent blood purification.Conclusions As a first-line option for HUS,plasma exchange may relieve the disease and improve its prognosis.It is the first-line treatment to HUS.Eculizumab is recommended for infants non-responding to plasma exchange.

       

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