Document Detail

Noninvasive ventilation in cystic fibrosis.
MedLine Citation:
PMID:  20387292     Owner:  NLM     Status:  MEDLINE    
A physiological rationale has been demonstrated for the use of noninvasive positive pressure ventilation (NPPV) in patients with cystic fibrosis. Indeed, as lung disease progresses, respiratory muscle load increases. As a result, patients develop a compensatory mechanism of a rapid shallow breathing pattern in an attempt to reduce this increase in load. Although this breathing strategy may maintain the level of minute ventilation, alveolar hypoventilation, characterized by hypercapnia and hypoxemia, may develop. Physiological studies during wakefulness and sleep have demonstrated that NPPV is associated with a reduction in respiratory muscle-load and work of breathing, an increase in minute ventilation and an improvement in gas exchange. NPPV has been shown to reduce oxygen desaturation during exercise and chest physiotherapy and may facilitate the recovery of a severe respiratory exacerbation. However, long-term data are lacking and are warranted to determine the optimal criteria to propose NPPV in these patients, and to assess the benefits in terms of improved survival and quality of life.
Brigitte Fauroux
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Expert review of respiratory medicine     Volume:  4     ISSN:  1747-6348     ISO Abbreviation:  Expert Rev Respir Med     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-04-13     Completed Date:  2010-06-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101278196     Medline TA:  Expert Rev Respir Med     Country:  England    
Other Details:
Languages:  eng     Pagination:  39-46     Citation Subset:  IM    
AP-HP, Hopital Armand Trousseau, Pediatric Pulmonary Department, Research unit INSERM UMR 5-938, National Reference Centre for Rare Lung Diseases, Pierre et Marie Curie-Paris University, 28 Avenue du Docteur Arnold Netter, Paris F-75012, France.
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MeSH Terms
Cystic Fibrosis / physiopathology*,  therapy*
Exercise Tolerance
Positive-Pressure Respiration / methods*
Quality of Life
Respiratory Muscles / physiopathology
Work of Breathing

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