Résumé :
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Successive respiratory tract infections in Cystic Fibrosis (CF) lead to progressive lung destruction with associated respiratory failure and subsequent supplemental oxygen need. Disease progression culminates with severe chronic respiratory failure and at this stage non-invasive positive pressure ventilation (NPPV) usually plays an important role as a bridge until lung transplant is performed. The authors present five cases of paediatric CF patients (three female) followed at the CF Centre of Hospital Santa Maria who underwent NPPV with Bilevel positive airway pressure ventilation with nasal mask. The mean age at NPPV beginning was 18 years (range 11,7 to 24,6 years). When NPPV was begun all patients were oxygen dependent for a mean interval of 19 months and were already on lung transplant waiting list. All patients maintained NPPV until lung transplant or death and the mean duration of NPPV was 28,4 months. Two patients were successfully lung transplanted at 13 years and 29 years of age after 16 months and 4,8 years of NPPV duration, respectively. The remaining three patients died after a mean NPPV period of 22,7 months. All patients had severe lung function compromise at the time of NPPV beginning and all revealed significant clinical benefit after NPPV initiation, namely in what concerns quality of life. One of the deceased patients was able to return to an active professional life for three more years while undergoing nocturnal NPPV. One of the main drawbacks of NPPV in these patients was the difficulty seen in the initial adaptation due to bronchorrea. Nevertheless, NPPV ended up having a significant positive impact in chest physiotherapy and airway clearance, as well as in exercise tolerance. The clinical improvement observed in this group of CF patients suggests that NPPV should not be exclusively seen as a pre-lung transplantation strategy and should in fact be considered as a useful approach in earlier CF disease stages. Previous studies have already documented significant clinical improvements in CF patients undergoing NPPV, namely concerning nocturnal hypoventilation, exercise tolerance, chest physiotherapy and during respiratory exacerbation approaches. Although NPPV initiation criteria in CF remain to be validated, polysomnographic evaluations may assume a determinant role.
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