Source: Annual Congress 2002 - Bridging the gap - lung function from infancy through childhood
Disease area: Paediatric lung diseases

Abstract

Different functional characteristics such as airway obstruction, pulmonary hyperinflation, lung restriction, ventilation inequalities and gas exchange disturbances may reflect progression of lung involvement in CF. The question is by which pulmonary function (PF) parameters progression of lung disease can best be predicted in relation to concomitant factors such as genetic background and chronic P. aeruginosa colonization (CPAC) . Over an age range of 4 to 16 years vital capacity (VC), thoracic gas volume, functional residual capacity, trapped gases (TG), lung clearance index (LCI), airway resistance (Raw), forced expiratory volume in one second, maximal expired flow at 50 % VC (MEF₅₀) and blood gases were assessed annually by whole-body plethysmography, multibreath nitrogen washouts and blood gas analysis in 52 children with CF. Values were expressed in standard deviation scores (SD-S) of a healthy control population. Using Kaplan Meier statistics, the event of deterioration was defined as the age at which PF (averaged over 2 consecutive measurements) exceeded ±] 2 SD for the first time. With a median age at onset of event the LCI was the earliest and most sensitive lung function parameter describing progression of lung disease (LCI: 7.3 years; TG: 14.8 years; Raw: 16.3 years; MEF₅₀: 16.9 years; p<0.001). Moreover, TG and LCI discriminated best between children with and without CPAC (p<0.02). Furthermore, the LCI discriminated between genetic groups, ΔF508/R553X (nonsense mutation) versus ΔF508/3905insT (frameshift mutation) (p<0.001), and ΔF508(2) versus ΔF508/3905insT (p<0.02).
Conclusion: In relation to CPAC as environmental factor and in association with the genotype as predisposing factor, disease progression in CF can best be reflected by indices of intrapulmonary gas distribution.

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Citations should be made in the following way:
A. Schibler, A. Lüscher, R. A. Amman, S. Gallati, R. Kraemer (Bern, Switzerland). Progression of lung involvement in patients with cystic fibrosis (CF), assessed by longitudinal respiratory function measurements. Eur Respir J 2002; 20: Suppl. 38, 256

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