Published ahead of print on November 5, 2004, doi:10.1164/rccm.200407-948OC
Am. J. Respir. Crit. Care Med., Volume 171, Number 4, February 2005, 371-378
A more recent version of this article appeared on February 15, 2005
Submitted on July 21, 2004
Accepted on November 4, 2004
Ventilation Inhomogeneities in Relation to Standard Lung Function in Patients with Cystic Fibrosis
Richard Kraemer1*, Andrea Blum1, Andreas Schibler1, Roland A Ammann1, and Sabina Gallati1
1 Department of Paediatrics, Division of Human Genetics, University of Berne, Berne, Switzerland
* To whom correspondence should be addressed. E-mail: richard.kraemer{at}insel.ch.
Based on serial lung function measurements performed in 142 children (68 males; 74 females) with cystic fibrosis, prospectively evaluated over an age range 6 to 20 years, we attempted to determine whether the lung clearance index as a measure of ventilation inhomogeneities could be a discriminating factor of disease progression. Annual follow-up lung function measurements featuring functional residual capacity determined by whole-body plethysmography and multibreath nitrogen washouts, effective specific airway resistance, flow volume curves, lung clearance index, 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 lung clearance index, followed by MEF50 and FRCpleth (p<0.0001). Associations between onset of chronic P. aeruginosa infection, and CFTR genotype with FEV1 (p=0.027) and FVC (p=0.007) were identified. The study shows, that the lung clearance index 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, which should be evaluated concomitantly as determinants of lung progression in CF.
Key words: Cystic fibrosis, ventilation inhomogeneities, onset of progression, genotype, Pseudomonas aeruginosa
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