Deficiency of Complement Factor H Related (CFHR) plasma proteins and Autoantibody Positive Hemolytic Uremic Syndrome (DEAP-HUS) is a subtype of atypical hemolytic uremic syndrome, known to be associated with significant morbidity. Its pathogenesis is linked to the production of IgG autoantibodies against complement factor H, a regulator of the alternative complement pathway. The binding of the autoantibodies to the C terminal of complement factor H interferes with its regulatory function, leading to increased activation of the alternative complement pathway and consequent endothelial cellular damage. Early diagnosis and initiation of appropriate therapy is reported to lead to favorable outcomes. Institution of plasma exchange therapy within 24 h of diagnosis has been shown to rapidly lower antibody levels, leading to clinical improvement. Adjunctive immunosuppression therapy suppresses antibody production and helps in maintaining long-term clinical remission of the disease. Available data advocates a treatment regimen that combines plasma therapy (preferably plasma exchange) and immunosuppression to halt disease process and sustain long-term disease remission.

中文翻译：

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CFHR 血浆蛋白缺乏和自身抗体阳性溶血性尿毒症综合征：治疗原理、结果和监测。

补体因子 H 相关 (CFHR) 血浆蛋白缺乏和自身抗体阳性溶血性尿毒症综合征 (DEAP-HUS) 是非典型溶血性尿毒症综合征的一种亚型，已知与显着发病率相关。其发病机制与产生针对补体因子 H 的 IgG 自身抗体有关，补体因子 H 是替代补体途径的调节剂。自身抗体与补体因子 H 的 C 末端的结合会干扰其调节功能，导致替代补体途径的激活增加和随后的内皮细胞损伤。据报道，早期诊断和开始适当的治疗会带来良好的结果。已证明在诊断后 24 小时内进行血浆交换治疗可迅速降低抗体水平，从而改善临床症状。辅助免疫抑制疗法可抑制抗体产生并有助于维持疾病的长期临床缓解。现有数据提倡将血浆疗法（最好是血浆置换）和免疫抑制相结合的治疗方案，以阻止疾病进程并维持长期疾病缓解。