The higher the value of the final score, the better the QoL of patients. Maximal respiratory pressures were measured through manovacuometry, using an analog manovacuometer (Comercial Médica®, São Paulo, Brazil). The test was performed with the child sitting comfortably to measure the maximal inspiratory pressure

(MIP) through an inspiratory maneuver based on the functional residual capacity, whereas maximal expiratory pressure (MEP) was evaluated based on total lung capacity for a forced expiration, after clipping the nose. For both measures, three maneuvers were performed and the best of the three was chosen using the technique based on Wilson’s study.21 Lung function assessment was performed by spirometry Doxorubicin clinical trial using a digital spirometer OneFlow® model (Clement Clarke International; Harlow, UK) and technical procedures, criteria for acceptability, and reproducibility followed the guidelines of the American Thoracic Society and European Respiratory Society (ATS/ERS).22 The forced expiratory volume in one second (FEV1), forced vital capacity (FVC), peak expiratory flow (PEF), and FEV1/FVC ratio were determined. The maneuver was performed three times and the highest value was used. Submaximal functional capacity was evaluated using the 6MWT according to the ATS standardization,22 on a flat, 30-meter

corridor. The children/adolescents were instructed to walk as far as possible in six minutes without running, and the test could be interrupted at any time. The children/adolescents were verbally encouraged, minute-by-minute, according to the test standardization, and after six minutes they were GPCR Compound high throughput screening asked to stop where they were in order to record the distance walked in meters. The parameters evaluated pre- and post-test included heart rate (HR) and pulse oxygen saturation (SpO2) by pulse oximetry model OXP-10 (EMAI Equipamentos Médicos Hospitalares, São Paulo, Brazil), systemic blood pressure through a sphygmomanometer model CE0050 (Tycos/WelchAllyn, Skaneateles Falls, USA), respiratory rate (RR) (counted by chest wall incursions

per minute), and the modified Borg scale score to measure dyspnea.23 The criteria for test interruption were: severe dyspnea or fatigue expressed by the patient, SpO2 < 85%, or refusal to continue the examination. Based on the reference values suggested by 4-Aminobutyrate aminotransferase Priesnitz et al.24 for healthy children, the predicted walked distance at the 6MWT for the children was calculated using the formula 6MWT = 145.343 + [11.78 x age (years)] + [292.22 x height (m)] + [0.611 x (HRFinal - HRInitial)] – [2,684 x weight (kg)] to evaluate test performance. This reference was used because it is from a study on healthy Brazilian children. Based on these values, the difference of means between the distance walked by the patient at the 6MWT (WD) and the predicted distance (PD) was obtained. Values were expressed as means and their standard deviations for quantitative variables.