Comparison Of Lung Clearence Index, Impulse Oscillometry And Spirometry in Children With Primary Ciliary Dyskinesia

Abstract

Background: In primary ciliary dyskinesia (PCD) ventilation defects and heterogeneous impairments to pulmonary function is common. But, spirometry may underestimate severity and complexity of PCD. Lung clearance index (LCI), is more sensitive in detecting early lung disease than FEV1 and has been less evaluated in children PCD.

Aim: The aim of this study is to evaluate LCI, impulse oscillometry (IOS) and spirometry in childen with PCD and healthy controls and compare them in terms of early detection of lung disease.

Methods: 32 children with PCD (median; 13.19 yr, range 7-18 yr)and 44 age-matched healthy controls ( median; 12.32 yr; range; 6-17 yr ) were included. Lung function tests were assesed by LCI, IOS and spirometry on the same day to the study participants. Correlation was done between LCI, IOS and spirometry measurements.

Results: Statistical significances were found in FEV1, FVC, FEV1/FVC, R5Hz, R10Hz, R15Hz, R20Hz, X5Hz, Fres and LCI 2.5% values in children with PCD compared with controls (p<0.05). While mean LCI2,5% value in patientswas 10.99±3.34, it was 6.99±0.77 in healthy controls (p<0.001). LCI 2.5% was abnormal in 46.5% of patients with FEV1>80% predicted. There was a strong inverse correlation between LCI %2.5 and FEV1, FVC and positive corelation with Fres in PCD patients (p<0.001 r:-0.635, p=0.002 r: -0.517,p=0.35 r:0.394 respectively).

Conclusion: This is the first study comparing LCI, IOS and spirometry in PCD patients. PCD is characterised by marked peripheral airway dysfunction. LCI and IOS are a can be used in the diagnosis and follow-up of PCD.