Abstract
Introduction: Structural changes in the lung visualized with CT may correlate with histologic findings in a number of chronic airway diseases and bronchiectasis.Chest CT is an important tool in detection and management of bronchiectasis, but there is little information about CT findings in primary ciliary dyskinesia(PCD).The aim of this study was to analyze the chest CT findings of patients with PCD and make clinical correlation with radiological findings.
Method: We analyzed the radiological findings of 95 patients diagnosed with PCD folllowing at Hacettepe University Pediatric Pulmonology Department. CT findings were correlated with phenotypic findings, including situs type, spirometry and microbiologic findings in the airways.
Results: The mean age of the patients were 10.8(1-20 years)years and 49(%52) of the patients were female. Situs inversus totalis(%13.7) or heterotaxy(%5.3) was identified in %19 of the patients. Chest CT revealed out %7.4 pectus excavatum, %3.2 sternal tilt, %4.2 pectus carinatum. The most common lung lobes involved were %60 right-middle lobe, %58.7 left-lower lobe, %53 left lingula, %41.6 right lower lobe, %22.8 right upper lobe, %7.5 left upper lobe and central bronchiectasis was the most common finding. It was found that bronchiectasis score increased with age and lung function tests decreased with bronchiectasis severity.
Conclusion: Chest CT shows that pulmonary disease related to PCD predominantly involves the middle and lower lobes of the lungs. This finding contrasts to the more diffuse disease of cystic fibrosis, which has an upper-lobe predilection. Bronchiectasis severity correlated with worse pulmonary function as reflected by FEV1 and older age at CT.
- Copyright ©ERS 2015