title: Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia.
abstract: 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 (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.
  
  
  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.
authors: Department of Paediatrics, University of Pisa, Pisa, Italy. m.pifferi@med.unipi.it
authors: Massimo
authors: M
authors: Pifferi
authors: Andrew
authors: A
authors: Bush
authors: Giovanni
authors: G
authors: Pioggia
authors: Davide
authors: D
authors: Caramella
authors: Gennaro
authors: G
authors: Tartarisco
authors: Maria
authors: M
authors: Di Cicco
authors: Marta
authors: M
authors: Zangani
authors: Iolanda
authors: I
authors: Chinellato
authors: Fabrizio
authors: F
authors: Maggi
authors: Giovanna
authors: G
authors: Tezza
authors: Pierantonio
authors: P
authors: Macchia
authors: Attilio
authors: A
authors: Boner
mesh_terms: D000293:Adolescent; D000328:Adult; D000465:Algorithms; D001987:Bronchiectasis / Q000503:physiopathology; D002648:Child; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy / Q000453:epidemiology; D007619:Kartagener Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D008171:Lung Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000382:microbiology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010993:Plethysmography, Whole Body; D011552:Pseudomonas Infections / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D012115:Residual Volume; D012720:Severity of Illness Index; D013147:Spirometry; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity