gov under registration NCT 01292902. Inspiratory muscle strength was evaluated using a digital manometer (MVD-300, Globalmed, Brazil) connected to a mouthpiece with a 2 mm opening. Each patient performed three maneuvers with maximum variation of up to 10% between them to achieve MIP ( Neder et al., 1999), from residual volume (RV) to total lung capacity (TLC). The best of the three maneuvers was recorded. A Cell Cycle inhibitor portable spirometer (Micro Medical, Microloop, MK8, England) was used for pulmonary function testing. Forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) were evaluated in accordance with recommendations of the

American Thoracic Society ( American Thoracic Society, 2002). The six-minute walk test (6MWT) was used to assess functional capacity in terms of distance covered (6MWD) in accordance with protocol established by the American Thoracic Society (ATS) (2002). The following resting parameters were evaluated before testing: arterial pressure (Pa), heart rate (HR), oxygen saturation (SpO2 measured by Onyx 9500 portable pulse oximeter), respiratory rate (RR), and dyspnea scale (Borg Scale). Inspiratory loaded breathing testing was performed

with a threshold device (Threshold Inspiratory Muscle Trainer, Healthscan Products Inc., Cedar Grove, New Jersey), mostly used FK228 for inspiratory muscle training in healthy subjects (Hostettler et al., 2011) and in patients with various pathologies C1GALT1 such as CHF (Dall’Ago et al., 2006 and Chiappa et al., 2008). This device was connected the mouthpiece. During the three-minute-long test (De Andrade et al., 2005), patients breathed through the mouthpiece with their noses occluded by a noseclip, using 30% MIP. An inspiratory load

of 30% was chosen taking into consideration several studies of inspiratory muscle training for this population (Laoutaris et al., 2004, Dall’Ago et al., 2006 and Chiappa et al., 2008). During the test, the participants were encouraged to maintain respiratory frequency between 12 and 16 bpm. Testing was interrupted if HR increased more than 20% and/or SpO2 <88%. Optoelectronic plethysmography (BTS Bioengineering, Italy) measures volume changes in the thoracoabdominal system through the placement of 89 markers formed by hemispheres covered with retro-reflective paper. The location of each hemisphere is determined by anatomical references in the anterior and posterior regions of the thorax and abdomen. Markers were placed on the skin using hypoallergenic bioadhesives. Eight cameras were placed around the patient and recorded images were transmitted to a computer, where a three-dimensional model is formed based on the markers OEP capture software (BTS Bioengineering, Italy). The chest wall was divided into the following compartments (Fig.