Hisano Masataka, Ashida Akira, Nakano Eiji, Suehiro Mamiko, Yoshida Yoko, Matsumoto Masanori, Miyata Toshiyuki, Fujimura Yoshihiro, Hattori Motoshi
Department of Nephrology, Chiba Children's Hospital, Chiba, Japan.
Pediatr Int. 2015 Apr;57(2):313-7. doi: 10.1111/ped.12469.
We report a case of atypical hemolytic uremic syndrome (aHUS) in a 4-year-old boy. Although the patient had the typical triad of aHUS (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury), urgent dialysis was not indicated because he had neither oliguria nor severe electrolyte abnormality. He was given eculizumab as first-line therapy, which led to significant clinical improvement, thus avoiding any risk of complications associated with plasma exchange and central venous catheterization. Retrograde functional analysis of the patient's plasma using sheep erythrocytes indicated an increase in hemolysis, suggesting impairment of host cell protection by complement factor H. The use of eculizumab as first-line therapy in place of plasma exchange might be reasonable for pediatric patients with aHUS.
我们报告了一例4岁男孩的非典型溶血性尿毒症综合征（aHUS）。尽管该患者具有aHUS的典型三联征（微血管病性溶血性贫血、血小板减少和急性肾损伤），但由于他既无少尿也无严重电解质异常，故未进行紧急透析。他接受了依库珠单抗作为一线治疗，这导致了显著的临床改善，从而避免了与血浆置换和中心静脉置管相关的任何并发症风险。使用绵羊红细胞对患者血浆进行逆向功能分析表明溶血增加，提示补体因子H对宿主细胞的保护作用受损。对于患有aHUS的儿科患者，使用依库珠单抗作为一线治疗替代血浆置换可能是合理的。
