Lung function tests in patients with primary ciliary dyskinesia

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Background/objective: Recent studies suggested relatively stable lung disease without siginificant lung function decline in Primary Ciliary Dyskinesia(PCD).The aim of this study was to evaluate the alteration of lung functions in patients with PCD and to assess the risk factors affecting the decline of lung function tests. Methods: Patients with PCD following at Hacettepe University Pediatric Pulmonology Department were recruited into the study and clinical characteristics, lung function tests within last 5 years, radiological and microbiological results were evaluated. Results: 167 patients with PCD were included and the mean age of the patients was 15.3±3.4(7-23) years.The mean diagnostic age was 1.8±3.1(0-13) years. 88 patients who performed the lung function tests within last 5 years were compared.The mean FVC was found 81.1±16.9%, FEV1 was found 71.6±16.7% and FEF25-75 was found 54.2±19.4% in the first year.During their clinical follow-up the mean FVC was found 74.8±17.1%, FEV1 was found 72.3±19.1% and FEF25-75 was found 60.3±27.7% in the 5th year.FVC% decreased significantly( p ) in the last five years, however FEV1% did not change(p=0.95); also FEF25-75% increased significantly( p=0.01 ).Bronchiectasis and sputum microbiology results did not affect the lung function alteration within years significantly. Conslusion: Although lung function and further lung damage in PCD can be stabilized after established PCD diagnosis, we showed FVC decline within years predicting the decreasing lung capacity.However FEF25-75 improved significantly suggesting physiotherapy effect on mucociliary clearance of the small airways.It is believed that progression and extent of lung disease can be slowed with early diagnosis and therapy.