Department of Public Health and Infectious Diseases, Adult Cystic Fibrosis Center, Sapienza University of Rome, 00185 Rome, Italy; Cystic Fibrosis Unit, Bambino Gesù Children's Hospital, Rome, Italy.
Department of Public Health and Infectious Diseases, Adult Cystic Fibrosis Center, Sapienza University of Rome, 00185 Rome, Italy.
J Cyst Fibros. 2019 May;18(3):420-424. doi: 10.1016/j.jcf.2019.03.001. Epub 2019 Mar 14.
The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. PA of mild intensity improved by +13% in patient 1, + 84% in patients 2 and + 89% in patient 3. Oxygen uptake increased both at anaerobic threshold and at peak exercise (patient 1 + 33%, patient 2 + 42% and patient 3 + 20%). Daily physical activities and exercise tolerance improved after two years of lumacaftor-ivacaftor therapy.
校正剂 lumacaftor 与增敏剂 ivacaftor 的联合应用已被批准用于治疗纯合子 Phe508del CFTR 突变的囊性纤维化 (CF) 患者。目前尚无详细报告描述 lumacaftor-ivacaftor 对体力活动 (PA) 和运动耐量的影响。我们对 3 名 CF 成人进行了递增心肺运动测试 (CPET)，并在开始使用 lumacaftor-ivacaftor 治疗前和治疗后 2 年评估了 PA。患者 1 的低强度体力活动增加了 13%，患者 2 增加了 84%，患者 3 增加了 89%。患者 1 和 3 的无氧阈和最大运动时的摄氧量均增加 (+33% 和 +20%)，患者 2 的摄氧量增加了 42%。在接受 lumacaftor-ivacaftor 治疗 2 年后，日常体力活动和运动耐量均有所改善。
