Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria.
Improvement in the symptoms of smooth muscle dystonia during eculizumab therapy in paroxysmal nocturnal hemoglobinuria.
复制标题
依库丽单抗治疗阵发性睡眠性血红蛋白尿期间平滑肌肌张力障碍症状的改善。
作者：
A. Hill;R. Rother;P. Hillmen
阵发性睡眠性血红蛋白尿症（PNH）溶血发作期间异常平滑肌肌张力障碍与腹痛、吞咽困难和勃起功能障碍的症状有关。在这里，我们报告两个PNH患者治疗补体抑制剂，依库珠单抗。补体抑制已持续2年以上，并导致血管内溶血消退和平滑肌收缩相关症状改善。
Aberrant smooth muscle dystonia during hemolytic episodes in paroxysmal nocturnal hemoglobinuria (PNH) is implicated in the symptoms of abdominal pain, dysphagia and erectile dysfunction. Here we report two PNH patients treated with the complement inhibitor, eculizumab. Complement inhibition has been sustained for over 2 years and results in resolution of intravascular hemolysis and amelioration of symptoms associated with smooth muscle contractions.