Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis

Kraemer, Richard, Blum, Andrea, Schibler, Andreas, Ammann, Roland A. and Gallati, Sabina (2005) Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 171 4: 371-378. doi:10.1164/rccm.200407-948OC

Author Kraemer, Richard
Blum, Andrea
Schibler, Andreas
Ammann, Roland A.
Gallati, Sabina
Title Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis
Journal name American Journal of Respiratory and Critical Care Medicine   Check publisher's open access policy
ISSN 1073-449X
Publication date 2005-02
Sub-type Article (original research)
DOI 10.1164/rccm.200407-948OC
Volume 171
Issue 4
Start page 371
End page 378
Total pages 8
Place of publication New York, NY, United States
Publisher American Thoracic Society
Language eng
Formatted abstract
Based on serial lung function measurements performed in 142 children (68 males; 74 females) with cystic fibrosis (CF), prospectively evaluated over an age range of 6 to 20 years, we attempted to determine whether the lung clearance index (LCI) as a measure of ventilation inhomogeneities could be a discriminating factor of disease progression. Annual follow-up lung function measurements featuring FRC determined by whole-body plethysmography and multibreath nitrogen washouts, effective specific airway resistance, flow-volume curves, LCI, and gas exchange characteristics were analyzed by linear mixed-model analysis and Kaplan-Meier statistics. The earliest occurring and strongest factor of progression was the LCI, followed by maximal expiratory flow (MEF50) and FRC determined by plethysmography (p < 0.0001). Associations between onset of chronic Pseudomonas aeruginosa infection and CF transmembrane conductance regulator (CFTR) genotype with FEV1 (p = 0.027) and FVC (p = 0.007) were identified. The study shows that the LCI predicts earlier in life and represented much better functional progression than FEV1. Moreover, there is no single functional predictor of progression in CF, but aside from risk factors, such as onset of chronic P. aeruginosa infection and genotype, pulmonary hyperinflation, airway obstruction, and ventilation inhomogeneities are important pathophysiologic processes that should be evaluated concomitantly as determinants of lung progression in CF.
Keyword Cystic fibrosis
Onset of progression
Pseudomonas aeruginosa
Ventilation inhomogeneities
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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