Jaegermasterscope

Spirometry: FVC, forced expiratory volume in the first second (FEV₁), FEV₁/FVC (JaegerMasterScope, CareFusion, Hoechberg, Germany). Plethysmography: total lung capacity (TLC), residual volume (RV), functional residual capacity (FRC), RV/TLC (JaegerMasterScope, CareFusion, Hoechberg, Germany). Maximum pressures: maximal inspiratory pressure (MIP) (MicroRPM, CareFusion, Hoechberg, Germany). Physical condition: 6-minute walking test (6MWT) (Pulsox 300i, Konica Minolta, Tokyo, Japan). Arterial blood gases: pH, paCO₂, paO₂, and HCO₃ (Gem Premier 4000, Instrumentation Laboratory, Bedford, MA, USA),

Respiratory evaluations were performed before starting treatment and at different time points during follow-up (4, 7, 12, 18, 26 and 30 months after treatment). The following measurements of respiratory function were recorded in each visit: standard spirometry for FVC (Jaeger MasterScope, Carefusion, Hoechberg, Germany), MIP, MEP and SNIP (MicroRPM, Carefusion, Hoechberg, Germany), CPF (MicroPeak, Carefusion, Hoechberg, Germany), blood arterial gas for pH, paCO₂, paO₂ and HCO₃ (Gem Premier 4000, Instrumentation Laboratory, Bedford, Massachusetts, USA), nocturnal pulse oximeter for SpO₂ and CT90 (Pulsox 300i, Konica Minolta, Tokyo, Japan), a BiPAP (Bilevel Positive Airway Pressure) built in software assessment for IPAP (inspiratory positive airway pressure), EPAP (expiratory positive airway pressure), daily use of the device in hours and average number of spontaneous breaths (Philips DirectView, Murrysville, Pennsylvania, USA, and Resmed ResScan, Australia) and the need for mechanical cough assistance techniques (CoughAssist, Philips Respironics, Murrysville, Pennsylvania, USA). All respiratory function tests were performed according to international ATS/ERS (American Thorax Society / European Respiratory Society) guidelines.13 (link)