Open Access Journal
QUALIS
B1
2021-2024
quadriênio
Open Access Journal
B1
2021-2024
quadriênio
Cystic Fibrosis (CF) is a multisystemic genetic diseasecharacterized by the production of thick and viscous mucus thataffects the respiratory system. The accumulation of secretions in the airways promotes recurrent pulmonary infections, chronicinflammation, and progressive bronchial obstruction, leading todeterioration of pulmonary function. These alterations contributeto the development of complications such as hypoxemia, hypercapnia, and acute respiratory failure (ARF). In thiscondition, non-invasive ventilation (NIV) stands out as a therapeutic resource capable of improving gas exchange, reducingrespiratory workload, and preventing the need for endotrachealintubation. The aim of this study was to analyze the management of NIV in adult patients with CF during episodes of ARF and its effects on oxygenation, respiratory muscle fatigue, pulmonaryfunction, dyspnea, respiratory comfort, and physical performance. An integrative review was conducted with searches performedbetween August and September 2025 in the Cochrane Library, PEDro, MEDLINE via PubMed, and LILACS via BVS databases, including studies published over the past teen years. Afterscreening and full-text analysis, six studies were included, comprising randomized clinical trials and crossover designs. The results demonstrated that NIV improves pulmonary function, increases oxygenation, reduces dyspnea and fatigue, and, whencombined with respiratory physiotherapy, enhances airwayclearance and exercise performance. It is concluded that NIV represents an effective, safe, and clinically relevant intervention in the management of ARF in adults with CF, contributing torespiratory stability and providing less invasive ventilatorysupport.
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Copyright (c) 2026 Tarcylla Maria Ferreira Da Silva, Jéssica Vitória Da Silva, Maria Eduarda Alves Salvino, Bruna Rafaelly Alves De Oliveira