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This Article Full Text (PDF) All Versions of this Article: chest.07-2812v1 134/2/351    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Santamaria, F. Articles by de Jong, P. A Search for Related Content PubMed PubMed Citation Articles by Santamaria, F. Articles by de Jong, P. A

STRUCTURAL AND FUNCTIONAL LUNG DISEASE IN PRIMARY CILIARY DYSKINESIA

¹Department of Pediatrics, Federico II University, Naples, Italy ²Department of Pediatric Pulmonology and Allergology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands ³Department of Radiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands ⁴Department of Diagnostic Imaging, Radiology Service, A.O.R.N. Antonio Cardarelli, Naples, Italy ⁵Department of Radiology, University Medical Center Utrecht, Utrecht, the Netherlands

pimdejong{at}gmail.com

Abstract

BackgroundHigh resolution computed tomography (HRCT) data on primary ciliary dyskinesia (PCD) related lung disease are scarce.

Study objectivesWe evaluated the lung disease in PCD children and adults by a modified Brody composite HRCT score in order to assess the prevalence of the structural abnormalities, to evaluate the correlation among HRCT score, spirometry and clinical data, and to compare the PCD scores with those of age- and sex-matched cystic fibrosis (CF) patients.

Patients and methodsTwenty PCD patients (4.6-27.5 years) underwent HRCT, spirometry and deep throat or sputum culture. A modified Brody score was used to assess bronchiectasis, mucous plugging, peribronchial thickening, parenchyma abnormalities, and mosaic perfusion.

ResultsTotal HRCT score was 6% of maximal score (range, 0.5-25.5). Subscores were: bronchiectasis, 5.6%; mucous plugging, 5.6%; peribronchial thickening, 8.3%; parenchyma, 3%; and mosaic perfusion, 0%. Prevalence of lung changes were: bronchiectasis, 80%; peribronchial thickening, 80%; mucous plugging, 75%; parenchyma, 65%; and mosaic perfusion, 45%. Sixteen of 19 PCD patients had positive cultures, and the most common pathogen was Haemophilus influenzae (84%). Total HRCT score was significantly related to age (p= 0.006), FEV₁ (p= 0.02) and FVC (p= 0.02). Bronchiectasis subscore was significantly related to FEV₁ (p= 0.04) and FVC (p= 0.03). In CF total HRCT score was significantly higher than in PCD (p= 0.02).

ConclusionsPCD shows significantly lower pulmonary HRCT scores than CF. PCD total and bronchiectasis scores correlate with spirometry. The PCD HRCT score might be used for longitudinal assessment and/or represent an outcome surrogate in future studies.

Key Words: primary ciliary dyskinesia • high resolution computed tomography • cystic fibrosis • children • adults