... progressively affected, influencing intrapulmonary gas mixing and gas exchange. We examined the long-term course of blood gas measurements in relation to characteristics of lung function and the influence ... Polkey MI, LofasoF: Setting of noninvasive pressure support in young patients with cystic fibrosis. Eur Respir J 2004, 24:624-630.31. Fauroux B: Nonivasive ventilation in cystic fibrosis. In Cystic Fibrosis ... course of deterioration of gas exchange in relation to changes in lung volume, ventila-tion distribution, trapped gas and airway obstruction, as well as the influence of specific genotypes in CF patients, from...