• Thorax · Nov 2012

    Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia.

    • Massimo Pifferi, Andrew Bush, Giovanni Pioggia, Davide Caramella, Gennaro Tartarisco, Maria Di Cicco, Marta Zangani, Iolanda Chinellato, Fabrizio Maggi, Giovanna Tezza, Pierantonio Macchia, and Attilio Boner.
    • Department of Paediatrics, University of Pisa, Pisa, Italy. m.pifferi@med.unipi.it
    • Thorax. 2012 Nov 1; 67 (11): 993-9.

    BackgroundIn primary ciliary dyskinesia (PCD) lung damage is usually evaluated by high-resolution CT (HRCT).ObjectiveTo evaluate whether HRCT abnormalities and Pseudomonas aeruginosa infection were better predicted by spirometry or plethysmography.MethodsA 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.ResultsFifty 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 (FEV(1))<80%, or forced expiratory flow between 25% and 75% of forced vital capacity (FVC) (FEF(25-75%))<70% or FEV(1)/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.ConclusionsPlethysmography 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.

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