Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis
Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis
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DOI：
10.1016/j.jcf.2017.05.002
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发表时间：
2017-11-01
影响因子：
5.2
通讯作者：
Reix, Philippe
中科院分区：
文献类型：
--
作者：
Labaste, Aurelie;Ohlmann, Camille;Reix, Philippe
据报道，在健康受试者中单独应用LUM/IVA后，S用力呼气量(FEV_1)的急性平均绝对下降率为-4.1%。本研究的目的是评估CF患者首次Lum/IVA治疗后FEV1的急性变化。共纳入32例儿科患者。仅3例患者出现呼吸道症状(9.4%)，但FEV1持续下降(-10.4+/-4.6%，范围：-1.5；-21.8%)。吸入沙丁胺醇后FEV1仅部分恢复。先前已知的明显可逆性气道阻塞和低FEV1的患者更有可能出现FEV1下降。(C)2017欧洲囊性纤维化学会。爱思唯尔出版，版权所有。
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 1 s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 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 FEV1 consistently decreased (-10.4 +/- 4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV1 were more at risk of FEV1 decrease. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.