Renal outcome with eculizumab in two diarrhea-associated hemolytic–uremic syndrome cases with severe neurologic involvement

SummaryThe kidney and brain are the two target organs in patients with Shiga toxin-producing Escherichia coli-associated hemolytic–uremic syndrome (STEC-HUS). Activation of the alternative complement pathway occurs in patients with STEC-HUS. A monoclonal antibody (eculizumab) directed against C5 has been reported to be effective against severe neurologic involvement in patients with STEC-HUS. We report on two STEC-HUS children with severe neurologic involvement treated with eculizumab. Despite prompt resolution of initial neurologic findings upon treatment with eculizumab, proteinuria and hypertension persisted in these patients. The persistence of these two risk factors is particularly emphasized to discuss the long-term effects of eculizumab, especially on renal involvement.產志賀毒素大腸桿菌相關溶血性尿毒症候群 (STEC-HUS) 的目標器官包括腎臟及腦部，在患者體內會出現補體替代途徑的活化。有報告指出，對於 STEC-HUS 所表現的重度神經學併發症，C5 單株抗體 (eculizumab) 具有若干效用。以下兩宗兒童個案，均為兼具重度神經學併發症的 STEC-HUS，且接受了 eculizumab 治療。治療期間神經學方面出現迅速改善，然而仍持續有蛋白尿及高血壓情形，因此必須注意 eculizumab 的長期影響特別是腎臟效應。