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Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia
  1. Massimo Pifferi1,
  2. Andrew Bush2,
  3. Giovanni Pioggia3,
  4. Davide Caramella4,
  5. Gennaro Tartarisco3,
  6. Maria Di Cicco1,
  7. Marta Zangani4,
  8. Iolanda Chinellato5,
  9. Fabrizio Maggi6,
  10. Giovanna Tezza5,
  11. Pierantonio Macchia1,
  12. Attilio Boner5
  1. 1Department of Paediatrics, University of Pisa, Pisa, Italy
  2. 2Imperial School of Medicine at the National Heart and Lung Institute, London, UK
  3. 3Institute of Clinical Physiology, CNR, Pisa, Italy
  4. 4Department of Diagnostic and Interventional Radiology, University of Pisa, Pisa, Italy
  5. 5Department of Paediatrics, University of Verona, Verona, Italy
  6. 6Department of Experimental Pathology, University of Pisa, Pisa, Italy
  1. Correspondence to Dr Massimo Pifferi, University of Pisa, Department of Pediatrics, Via Roma 67, Pisa 56126, Italy; m.pifferi{at}


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.

  • Primary ciliary dyskinesia
  • spirometry
  • plethysmography
  • high-resolution CT
  • sputum culture
  • paediatric asthma
  • paediatric lung disease
  • rare lung diseases
  • viral infection
  • asthma guidelines
  • cystic fibrosis
  • exhaled airway markers
  • lung physiology
  • pulmonary oedema
  • systemic disease and lungs
  • asthma
  • asthma in primary care
  • bronchiectasis
  • pneumonia
  • airway epithelium
  • asthma epidemiology
  • immunodeficiency
  • infection control

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  • Funding This research was supported by the Fondazione Carlo Laviosa, Italy.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by Hospital Ethical Committee of Pisa.

  • Provenance and peer review Not commissioned; externally peer reviewed.