Comparison of Spirometry, Impulse Oscillometry, and Lung Clearance Index
in Children with Primary Ciliary Dyskinesia
Abstract
Background: Primary ciliary dyskinesia (PCD) is associated with
ventilation defects and heterogeneous impairment of pulmonary function.
Spirometry may underestimate PCD severity and complexity. This study
aimed to evaluate spirometry, lung clearance index (LCI), and impulse
oscillometry (IOS) in children with PCD and healthy controls, and
compare them in terms of early detection of lung disease.
Methods: In this cross-sectional, prospective study,
participants included children aged 6-18 years with PCD and healthy
age-matched controls. Lung function tests using LCI, IOS, and spirometry
were conducted on the same day for all participants. Results:
Thirty-two children with PCD (median age 13.19 years) and 44 age-matched
healthy controls (median age 12.32 years) were studied. PCD was
associated with lower FEV1, FVC, FEV1/FVC, R5, R10, R15, R20, X5, Fres,
and LCI 2.5% mean values (p<0.05). Abnormal LCI 2.5% was
found in 46.5% of patients with predicted FEV1 > 80%.
Significant inverse correlations were observed between LCI 2.5%, FEV1,
FVC, and Fres in PCD patients (p<0.001, r:-0.635; p=0.002,
r:-0.517; p=0.006, r:-0.479; respectively. Conclusion: This is
the first study to compare LCI, IOS, and spirometry in children with
PCD. The study has shown that there are significant differences in
spirometry, LCI, and IOS values between children with PCD and healthy
controls. LCI can detect airway anomalies earlier than spirometry in PCD
patients. IOS and LCI are valuable respiratory function tests that can
be used in PCD follow-up.