| Summary: | Background: Kyphoscoliosis is a skeletal condition involving the hyperflexion of the thoracic spine. It is characterized by reduced chest wall compliance and impaired respiratory mechanisms leading to progressive hypo-ventilation. We evaluated the effectiveness and the safety of non-invasive ventilation (NIV) in patients after an episode of acute respiratory failure (ARF). Methods: Eighteen patients with severe kyphoscoliosis who had been hospitalized for an episode of ARF were followed for 4 years. NIV was applied via mouthpiece (MPV) during the daytime and via mask during the night. The primary outcomes were changes in physiological and functional parameters as well as quality of life. Secondary outcomes were considered re-hospitalization and mortality rate after discharge. A set of control subjects was used for comparison. Results: All patients showed a significant improvement in several clinical, physiological, functional and quality of life parameters. Four of them (22.2%) died during the four year follow-up period. In the uni-variate analysis patients who died had higher cardiac co-morbidity, lower MIP and SNIP, higher paCO2, and oxygen desaturation index at initial admission. Conclusions: Diurnal MPV associated with nocturnal NIV had significantly improved lung function, clinical outcomes and quality of life. It should be considered as a safe alternative to traditional administering of NIV. Keywords: Kyphoscoliosis, Respiratory failure, Non-invasive ventilation, Mouthpiece ventilation
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