Centre de ressources et de compétences mucoviscidose, Hospices Civils de Lyon, Lyon, France.
Centre de ressources et de compétences mucoviscidose, Hospices Civils de Lyon, Lyon, France; UMR5558, Lyon, France.
J Cyst Fibros. 2017 Nov;16(6):709-712. doi: 10.1016/j.jcf.2017.05.002. Epub 2017 May 18.
The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV were more at risk of FEV decrease.
在健康受试者中，单次给予卢美他赛和依伐卡托（LUM/IVA）组合药物后，据报道可导致平均急性绝对用力呼气量（FEV）预测值下降 4.1%。本研究旨在评估 CF 患者首剂 LUM/IVA 后的急性 FEV 变化。共纳入 32 例儿科患者。仅 3 例（9.4%）出现呼吸道表现，但 FEV 持续下降（-10.4±4.6%，范围：-1.5；-21.8%）。沙丁胺醇吸入后 FEV 仅部分恢复。先前已知存在显著可逆转气道阻塞和低 FEV 的患者发生 FEV 下降的风险更高。
