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Respiration. 2008;76(2):168-74. Epub 2007 Nov 7.

Long-term noninvasive ventilation in patients with cystic fibrosis.

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Pediatric Pulmonary Department, Hôpital Armand-Trousseau, Assistance Publique-Hôpitaux de Paris, INSERM UMR-S 719, Université Pierre-et-Marie-Curie-Paris 6, Paris, France.



The benefits of long-termnoninvasive positive pressure ventilation (NPPV) have not yet been evaluated in patients with cystic fibrosis (CF).


To evaluate the effect of 1 year of NPPV on lung function in patients with advanced CF.


Data were obtained from the French CF Registry. Patients who started NPPV (ventilated group, n = 41) were compared to matched controls (control group, n = 41). Each ventilated patient was matched to a control 1 year before the start of NPPV (year -1) for gender, CFTR genotype, age +/- 5 years and forced expiratory volume in 1 s (FEV(1)) +/- 10%. The ventilated group was compared to the control group at year -1, during the year of NPPV initiation (year 0) and 1 year after NPPV (year +1).


At year -1, the two groups were comparable with regard to forced vital capacity (FVC; 43.7 vs. 49.1% in the ventilated group and the control group, respectively) and FEV(1) (28.2 vs. 28.5%). At year 0, the ventilated group had significantly greater declines in FVC (-3.6 +/- 9.2 vs. +0.8 +/- 8.9%, p = 0.03) and in FEV(1) (-3.0 +/- 6.7 vs. +2.6 +/- 4.4, p < 0.0001). At year +1, the decreases in FVC (-2.1 +/- 10.0 vs. -2.2 +/- 9.9%) and in FEV(1) (-2.2 +/- 6.7 vs. -2.3 +/- 6.2%) were similar in both groups.


These data show that NPPV is associated with stabilization of the decrease in lung function in patients with advanced CF.

[Indexed for MEDLINE]

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