PT  - JOURNAL ARTICLE
AU  - Massimo Pifferi
AU  - Andrew Bush
AU  - Giovanni Pioggia
AU  - Davide Caramella
AU  - Gennaro Tartarisco
AU  - Maria Di Cicco
AU  - Marta Zangani
AU  - Iolanda Chinellato
AU  - Fabrizio Maggi
AU  - Giovanna Tezza
AU  - Pierantonio Macchia
AU  - Attilio Boner
TI  - Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia
AID  - 10.1136/thoraxjnl-2011-200137
DP  - 2012 Nov 01
TA  - Thorax
PG  - 993--999
VI  - 67
IP  - 11
4099  - http://thorax.bmj.com/content/67/11/993.short
4100  - http://thorax.bmj.com/content/67/11/993.full
SO  - Thorax2012 Nov 01; 67
AB  - Background In primary ciliary dyskinesia (PCD) lung damage is usually evaluated by high-resolution CT (HRCT). Objective To evaluate whether HRCT abnormalities and Pseudomonas aeruginosa infection were better predicted by spirometry or plethysmography. Methods A cross-sectional study performed in consecutive patients with PCD who underwent sputum culture, spirometry, plethysmography and HRCT within 48 h. Principal component analysis and soft computing were used for data evaluation. Results Fifty patients (26 children) were studied. P aeruginosa infection was found in 40% of the patients and bronchiectasis in 88%. There was a correlation between infection with P aeruginosa and extent of bronchiectasis (p=0.009; r =0.367) and air-trapping (p=0.03; r =0.315). Moreover, there was an association between infection with P aeruginosa and residual volume (RV) values >150% (p=0.04) and RV/total lung capacity (TLC) ratio >140% (p=0.001), but not between infection with P aeruginosa and forced expiratory volume in 1 s (FEV1)<80%, or forced expiratory flow between 25% and 75% of forced vital capacity (FVC) (FEF25–75%)<70% or FEV1/FVC<70% (<80% in children). Severity of the total lung impairment on chest HRCT directly correlated with RV when expressed as per cent predicted (p=0.003; r =0.423), and RV/TLC (p<0.001; r =0.513) or when expressed as z scores (p=0.002, r =0.451 and p<0.001, r =0.536 respectively). Principal component analysis on plethysmographic but not on spirometry data allowed recognition of different severities of focal air trapping, atelectasis and extent of bronchiectasis. Conclusions Plethysmography better predicts HRCT abnormalities than spirometry. Whether it might be a useful test to define populations of patients with PCD who should or should not have HRCT scans requires further longitudinal studies.