Lung clearance index helps to detect abnormal lung function in children with Primary Ciliary Dyskinesia (PCD)

Abstract

Purpose: to compare the degree of severity of structural lung disorders detected by spirometry, lung clearance index (LCI) and high-resolution computed tomography (HRCT) in children with Primary Ciliary Dyskinesia (PCD).

Methods: A complex study was performed in 24 child’s with PCD (11,2±1,2 years (6÷17y.); 14 girls, 10 boys): ultrasonic spirometry, HRCT and LCI from multiple-breath washout (MBW); 10 of this patients were carried out determination of LCI before and after the therapeutic bronchoscopy.

Results: Abnormal lung function was defined by spirometry in 72% patients: the most of them showed a significant decline VC, FVC, FEV1 and FEF75; 35% of patients had a positive bronchodilator test. According to HRCT all patients had signs of chronic bronchitis; cylindrical or mixed bronchiectasis was detected in 44% of cases. A significant excess of normal values of LCI (11,3±1,8; 7,8÷16,5) was recorded in all examined patients; the increase of LCI correlated with the number of segments with bronchiectasis. After the therapeutic bronchoscopy, 10 patients had a distinct decrease in LCI (from 12,5±1,9 to 9,9±1,7 (р<0,05))

Conclusions: LCI is a more sensitive indicator than spirometry to identify the abnormal lung function in children with PCD; changing LCI may be useful for non-invasive monitoring of structural lung disorders and treatment efficacy in children with PCD.