@article {DiakovaPA616, author = {Svetlana Diakova and Anna Bogorad and Irina Zorina and Petr Zacharov and Nikolai Syroezhin and Yuriy Mizernitskiy}, title = {Lung clearance index helps to detect abnormal lung function in children with Primary Ciliary Dyskinesia (PCD)}, volume = {54}, number = {suppl 63}, elocation-id = {PA616}, year = {2019}, doi = {10.1183/13993003.congress-2019.PA616}, publisher = {European Respiratory Society}, 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{\textquoteright}s with PCD (11,2{\textpm}1,2 years (6{\textdiv}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{\textpm}1,8; 7,8{\textdiv}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{\textpm}1,9 to 9,9{\textpm}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.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA616.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/54/suppl_63/PA616}, eprint = {https://erj.ersjournals.com/content}, journal = {European Respiratory Journal} }