Evaluation of the results of impulse osilometry, lung clearance index and respiratory function tests in patients with A diagnosis of primary ciliary dyskinesia and comparing with the patient's clinical state

Primary ciliary dyskinesia (PCD) is a genetic disease characterized by impairment of mucociliary movement due to defective ciliary movement. The aim of this study is to perform lung clearance index (LCI), spirometry and impulse oscillometry (IOS) tests in healthy children who have no disease with children with PCD and compare the results to show that it is a valuable respiratory function test that can be used. In the study, 32 children with PSD who were followed up in the Marmara University Pediatric Chest Diseases outpatient clinic and 44 healthy children who were admitted to the child outpatient clinic were included. LCI, spirometry and IOS tests were performed on the sick and healthy children and the results were compared. Tests of PSD and healthy children were compared; R5, R10, R15, R20 and Fres values ​​of sick children among the IOS were higher than the healthy group; The X5 value was found to be lower than the healthy group (p<0.05). When tests were compared for the patient group; FEV1 with X15, X20, Fres., LCI 2.5%, LCI 5%, FVC with X15, X20 Fres., LCI 2.5%, LCI 5%, FEV1/FVC with Fres., LCI %2.5, LCI %5 were found to be significantly correlated (p<0.05). As age increased, R5, R10, R15, R20 values ​​were found to be significantly lower (p<0.05) and X5, X10, X15, AX values ​​were found to be significantly higher (p<0.05). When evaluated according to height and weight, there was no significant difference in IOS and LCI parameters, while for LCI, a significant difference with age was found only in the FRC parameter (p=0.001). When the results are evaluated according to body mass index; X5, FRC and VTmean values ​​of lean patients were found to be significantly lower (p<0.05). LCI was significantly correlated with 2.5% when compared to FEV1 (p=0.001) and while FEV1 was normal, the LCI value was found to be higher in 46.5% of the patients, and this data showed that LCI can be used for earlier diagnosis than spirometry. Our study is the first clinical study comparing LCI, IOS and spirometry in children with children with PCD. Our study; It has shown that there are significant differences in spirometer, LCI and IOS values ​​in children with PCD compared to healthy controls, LCI can detect airway anomalies earlier than spirometry in PCD patients, IOS and LCI are a valuable respiratory function test that can be used in the diagnosis and follow-up of PCD.