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Pulmonary exacerbations (PEx), pathogens colonizing the respiratory tract, and patients' age are associated with progressive worsening of lung function among patients with cystic fibrosis (CF). However, the effect of these factors on longitudinal changes of Lung Clearance Index (LCI) remains unclear.To assess the role of age, different types of bronchial infection, and PEx on LCI deterioration.We conducted a retrospective study assessing multiple-breath washout (MBW) and spirometry changes among CF patients evaluated at quarterly outpatient clinic visits over 8 years. MBW and spirometry were performed at each visit, sputum samples and/or cough swabs were obtained for culture, whereas respiratory symptoms and clinical examination findings were recorded. Patients who had ≥5 serial MBW measurements, one of which coincided with a pulmonary exacerbation, were reviewed.Seventy-six patients were included in the study: mean age of 10.61 years (range 1.75-23.75). A total of 1152 MBW tests and 1047 spirometry tests were performed. LCI was significantly higher among CF patients aged 11-15, 16-20, and over 20 years than those under 5 years of age; ΔLCI: 1.16 (confidence interval [CI] 0.43-1.90) and 3.25 (CI 2.33-4.17), respectively. Furthermore, LCI was significantly elevated in CF patients with positive cultures for Pseudomonas aeruginosa (0.52 LCI [CI -0.12 to 0.71]) and Stenotrophomonas Maltophilia (1.41 LCI [CI 0.61-2.21]). Moreover, increased values of LCI in CF patients were significantly associated with increased risk of PEx (odds ratio [OR] 1.19, CI [1.14-1.25], p 0.001).LCI demonstrates a progression of lung disease and corresponds to changes in bacterial infections and PEx among patients with CF. LCI may be a valuable marker for tracking disease deterioration and may have a role in the routine clinical care of patients with CF.

Introduction. Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. Aim. To evaluate whether Pseudomonas aeruginosa colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if Pseudomonas colonization can predict exercise intolerance. Subjects. Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. Results. 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2 peak: 79.1%) were evaluated: 33 were chronically colonized with Pseudomonas aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2 peak% (p<0.001), LCI (p<0.001), as well as FEV1% (p<0.001) and FVC% (p<0.001). Pseudomonas colonization could predict VO2 peak% (p<0.001, r2: −0.395). Conclusion. Exercise capacity as reflected by peak oxygen uptake is reduced in Pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.

As Cystic Fibrosis (CF) treatment advances, research evidence has highlighted the value and applicability of Lung Clearance Index and Cardiopulmonary Exercise Testing as endpoints for clinical trials. In the context of these new endpoints for CF trials, we have explored the use of these two test outcomes for routine CF care. In this review we have presented the use of these methods in assessing disease severity, disease progression, and the efficacy of new interventions with considerations for future research.

Introduction: The coronavirus 2019 (COVID-19) pandemic has demanded care changes for patients with chronic disease. Patients with CF are considered at higher risk of developing severe manifestations in the case of SARS-CoV-2 infection, and a need for new ways of safer care delivery has been required to avoid transmission. Objectives: To assess the impact of the lockdown during the first wave of the COVID-19 pandemic and remote monitoring on patient’s health status and daily maintenance therapy in a middle-income resource setting. Methods: During the first wave of the pandemic period, we changed from regular clinic visits to telephone visit calls to monitor our patients’ health condition and adherence to physiotherapy and physical exercise. Results: A total of 120 patients or their caregivers have been contacted by telephone call visits over ten weeks. During this period, 38 patients (28.33%) were identified to have pulmonary exacerbation; 89.5% were prescribed oral antibiotics, 3% were hospitalized to get iv antibiotics, and 8% of the patients presented other CF complications. Most of the patients did not change the frequency of the daily physiotherapy. Moreover, 71% of the patients who performed regular physical exercise changed the frequency and the type of exercise during the quarantine period. Interestingly, mean FEV1 and body weight increased significantly and after the lockdown period. Conclusions: During the COVID-19 pandemic, the implementation of telephone contact processes aiming for CF patients’ appropriate care is of great importance. Further studies are needed to evaluate patient outcomes when transitioning from face-to-face clinics to telemedicine clinics.

Background: The role of cardiopulmonary exercise testing (CPET) in the assessment of prognosis in CF (cystic fibrosis) is crucial. However, as the overall survival of the disease becomes better, the need for examinations that can predict pulmonary exacerbations (PEx) and subsequent deterioration becomes evident. Methods: Data from a 10-year follow up with CPET and spirometry of CF patients were used to evaluate whether CPET-derived parameters can be used as prognostic indexes for pulmonary exacerbations in patients with CF. Pulmonary exacerbations were recorded. We used a survival analysis through Cox Regression to assess the prognostic role of CPET parameters for PeX. CPET parameters and other variables such as sputum culture, age, and spirometry measurements were tested via multivariate cox models. Results: During a 10-year period (2009–2019), 78 CF patients underwent CPET. Cox regression analysis revealed that VO2peak% (peak Oxygen Uptake predicted %) predicted (hazard ratio (HR), 0.988 (0.975, 1.000) p = 0.042) and PetCO2 (end-tidal CO2 at peak exercise) (HR 0.948 (0.913, 0.984) p = 0.005), while VE/VO2 and (respiratory equivalent for oxygen at peak exercise) (HR 1.032 (1.003, 1.062) p&nbsp, = 0.033) were significant predictors of pulmonary exacerbations in the short term after the CPET. Additionally, patients with VO2peak% predicted &lt, 60% had 4.5-times higher relative risk of having a PEx than those with higher exercise capacity. Conclusions: CPET can provide valuable information regarding upcoming pulmonary exacerbation in CF. Patients with VO2peak &lt, 60% are at great risk of subsequent deterioration. Regular follow up of CF patients with exercise testing can highlight their clinical image and direct therapeutic interventions.

Asthma is the most common chronic respiratory disease in children and inhaled corticosteroids (ICS) constitute the first line of treatment for these patients. However, the potential growth-inhibiting effect of ICS has often been a cause of concern for both caregivers as well as physicians, and there still remains conflict regarding their safety profile.To assess whether the administration of ICS in low or medium doses is associated with height reduction in prepubertal children.We performed a retrospective study to examine the association between ICS treatment and growth deceleration in children with mild persistent asthma. The comparison of height measurements every 6 months from 3 to 8 years of age was conducted among three groups of patients: patients not receiving ICS, patients being treated with low dose of ICS and patients being treated with medium dose of ICS (GINA Guidelines 2015).This study included 284 patients (198 male, 86 female) aged 3-8 years; 75 patients were not receiving ICS, 63 patients were on low-dose ICS and 146 patients were on medium-dose ICS. The measured height every 6 months did not differ significantly (p0.05) among the three groups while the difference remained stable (p0.05), even when we evaluated males and females separately.In this "real-life" study we found that long-term treatment with ICS in low or medium doses is not associated with height reduction in prepubertal children with asthma.

Background:Νeuroendocrine tumors of the lungs are rare arising in the thymus and gastro-entero-pancreatic tract and belonging to foregut of neuroendocrine tumors. The aim of the present prospective study was to estimate the potential impact of single-photon emission computed tomography somatostatin receptor scintigraphy using 99mTc-Tektrotyd on diagnosis, treatment response, and prognosis in patients with neuroendocrine tumors of the lungs.Methods:Thirty-six patients with neuroendocrine tumors of the lungs were evaluated by using 99mTc-HYNIC-TOC scintigraphy. The scintigraphic results were compared to liver tissue uptake (Krenning score). Likewise, the functional imaging results were compared with biochemical indices including chromogranin A, neuroendocrine-specific enolase, and insulin-like growth factor 1 at the time of diagnosis (baseline) and disease progression.Results:The number of somatostatin receptors, expressed with Krenning score, did not show any correlation with the survival of patients both at baseline ( P = .08) and at disease progression ( P = .24), and scintigraphy results did not relate significantly to progression-free survival. Comparing the results of 99mTc-HYNIC-TOC scintigraphy according to the response of patients in the initial treatment, a statistically significant negative correlation was observed both in the first and in the second scintigraphy with patients’ response ( P = .001 and P < .001, respectively). The concentrations of biochemical markers were in accordance with scintigraphy results in the diagnosis.Conclusion:This study indicates that 99mTc-HYNIC-TOC scintigraphy appears to be a reliable, noninvasive technique for detection of primary neuroendocrine tumors and their locoregional or distant metastases, although it cannot be used as a neuroendocrine tumors of the lungs predictive technique.

Introduction Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. Aim To evaluate whether Pseudomonas aeruginosa colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if Pseudomonas colonization can predict exercise intolerance. Subjects Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. Results 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2 peak: 79.1%) were evaluated: 33 were chronically colonized with Pseudomonas aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2 peak% (p < 0.001), LCI (p < 0.001), as well as FEV1% (p < 0.001) and FVC% (p < 0.001). Pseudomonas colonization could predict VO2 peak% (p < 0.001, r2: −0.395). Conclusion Exercise capacity as reflected by peak oxygen uptake is reduced in Pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.

INTRODUCTION Exercise ventilation efficiency index in cardiopulmonary exercise testing (CPET) is elevated in patients with heart failure providing useful information on disease progression and prognosis. Few data, however, exist for ventilation efficiency index among cystic fibrosis (CF) patients. AIMS To assess ventilation efficiency index (ΔVE/ΔVCO2 or V'E/V'CO2 slope) and intercept of ventilation (VE-intercept) in CF patients with mild, moderate, and severe cystic fibrosis (CF) lung disease. To assess possible correlations with ventilation inhomogeneity and structural damages as seen on high resolution computed tomography (HRCT). METHODS CF patients with mild (FEV1 > 80%, n = 47), moderate (60%

Summary Introduction Early detection of lung disease is a primary objective in monitoring patients with Cystic Fibrosis (CF); High-Resolution-Computed-Tomography (HRCT) assesses structural damage. Spirometry and cardiopulmonary exercise testing are used for functional evaluation of CF lung disease. Aim To evaluate the deterioration of exercise testing parameters over a 2-year period compared to the change of spirometry and HRCT parameters among CF patients. Methods Twenty-eight CF patients were evaluated with HRCT, spirometry, and exercise testing; 15 had two assessments with an interval of 2 years. Correlation analyses between Bhalla score parameters and functional measures were performed. Results Twenty-eight patients with CF (mean age 14.9 years, mean forced expiratory volume in 1 sec [FEV1] 83.2%) were evaluated. FEV1 was not found to change significantly in the 2-year period (P = 0.612). Both mean Bhalla score and mean peak oxygen consumption (VO2 peak %) deteriorated significantly (P = 0.014 and P = 0.026, respectively). VO2 peak and respiratory equivalents for O2 and CO2 at peak exercise were found to be significant predictors of Bhalla score (r = −0.477, P = 0.010; r = 0.461, P = 0.018; r = 0.402; P = 0.042, respectively). Anaerobic threshold was associated with changes in Bhalla score over the following 2 years. Conclusions Exercise testing is more sensitive than spirometry to detect structural changes in CF lungs. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.

Introduction: Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. Aim: To evaluate differences in CPET, LCI and High Resolution Computed Tomography (HRCT) indices among patients colonized or not with Pseudomonas Aeruginosa; to examine whether exercise testing indices correlate with lung structural damages as reflected by Bhalla score and to check if pseudomonas colonization can predict exercise intolerance. Subjects: Seventy eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. Results: 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2peak: 79.1%) were evaluated. 33 were chronically colonized with Pseudomonas Aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2peak%) (p Conclusion: Exercise capacity as reflected by peak oxygen uptake is reduced in pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.

INTRODUCTION FEV1 is often considered the gold standard to monitor lung disease in cystic fibrosis (CF). Recently, there has been increasing interest in multiple breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative or even more sensitive techniques. However, limited data exist on associations among the above methods. AIM To evaluate the correlations between outcome measures of MBW and CPET and to examine if ventilation inhomogeneity can predict exercise intolerance. SUBJECTS AND METHODS Ninety-seven children and adults with CF (47 males, mean [range] age 14.9 (6.6; 26.7) years, mean FEV1 : 90.8% predicted, mean lung clearance index [LCI]: 11.4, and mean peak oxygen uptake [VO2 peak]: 82.4% predicted) performed spirometry, MBW, and CPET on the same day during their admission or outpatient visit. RESULTS LCI, m1 /m0 , and m2 /m0 (P

Background: LCI has been proven an effective tool in the detection of lung disease in CF. Objectives: a) To assess the correlation of ventilation inhomogeneity indices with structural damages of the lung, among different groups of disease severity and b) To compare the associations among MBW parameters with spirometry and CT. Method: Forty-four children and adolescents with CF participated in the study. Spirometry and multiple breath washout tests were performed. All children had a HRCT scan. The study population was divided into two groups, according to FEV1% predicted values: Group A: ≥ 85% predicted (normal) and Group B: 40-84% predicted (mild-moderate disease). Results: The patients’ mean age was 12.9 (5.67; 23.25) years, mean FEV1: 91.22 ± 24.22% and mean LCI: 10.72 ± 3.51. Children of Group A had significantly lower LCI, compared to Group B (p

AIM: To examine whether ventilation-carbon dioxide output indices(ΔVE/ΔVCO2 slope) and ventilation–oxygen output indices (ΔVE/ΔVO2 slope) and oxygen uptake (ΔVO2/ΔWR)slopes differ among patients with cystic fibrosis (CF) lung disease. To investigate if these slopes are related to structural lung damages, spirometry findings and ventilation inhomogeneity. METHODS: CF patients with mild (FEV1%>80%, n=47), moderate (60% RESULTS: Mean ΔVE/ΔVCO2 for the mild disease group was 29.9, for the moderate 29.4 and for severe disease group 27.1 with no significant differences reported between the groups (p: 0.503). However, VE together with ΔVE/ΔVCO2 slope showed a significant correlation with Bhalla score (r2=0.869, p=0.006) and ΔVO2/ΔWR slope correlated significantly with Bhalla score (r2=0.197, p=0.038) in the total population. VE plus VO2min could predict LCI (r2=0.319, p CONCLUSION: Exercise testing slopes can reflect structural lung damages in CF youths regardless disease severity. Exercise testing can provide crucial information not only on the patients9 exercise capacity, but also on commencing lung damages.

Introduction: Spirometry has been recognized as the gold standard method in monitoring patients with CF. Recently, there has been increasing interest on the significance of Lung Clearance Index (LCI) in monitoring patients with CF. There are no data on the annual % change of LCI, among patients with CF. Aim: To determine the annual % change of LCI among patients with CF; to detect if age, disease severity and chronic pseudomonas aeruginosa colonization have an impact on LCI. Method : In a 5-year prospective study, 80 stable CF patients performed 650 Multiple Breath Washout (MBW) measurements. Sixty-one patients who performed 578 measurements fulfilled the study criteria. Mean LCI of the first year was defined as LCI start and the mean LCI of the last year was defined as LCI end . The annual change in LCI (ΔLCI) and the annual % change of LCI were calculated. Results: Sixty-one patients with CF (31 males) with a mean age 9.64 years, mean FEV1% 100.4 and mean LCI start 9.03 participated in the study. The mean (sd) annual ΔLCI was 4.96% (4.16) and the mean (sd) ΔFEV 1 was 0.01% (3.41). Mean LCI start was correlated with age (p start and mean FEV 1start differed significantly between patients with different age (p start and FEV1 start . The mean LCI start was the only predictor of the mean annual ΔFEV1% (p>0.001, r 2 :0.281) Conclusion : The annual % change of LCI is 5% and it seems to be stable and independent of age, PsA and baseline lung function.

Early detection of lung disease is a primary objective in monitoring patients with Cystic Fibrosis (CF); High-Resolution-Computed-Tomography (HRCT) assesses structural damage. Spirometry and cardiopulmonary exercise testing are used for functional evaluation of CF lung disease.To evaluate the deterioration of exercise testing parameters over a 2-year period compared to the change of spirometry and HRCT parameters among CF patients.Twenty-eight CF patients were evaluated with HRCT, spirometry, and exercise testing; 15 had two assessments with an interval of 2 years. Correlation analyses between Bhalla score parameters and functional measures were performed.Twenty-eight patients with CF (mean age 14.9 years, mean forced expiratory volume in 1 sec [FEV1 ] 83.2%) were evaluated. FEV1 was not found to change significantly in the 2-year period (P = 0.612). Both mean Bhalla score and mean peak oxygen consumption (VO2 peak %) deteriorated significantly (P = 0.014 and P = 0.026, respectively). VO2 peak and respiratory equivalents for O2 and CO2 at peak exercise were found to be significant predictors of Bhalla score (r = -0.477, P = 0.010; r = 0.461, P = 0.018; r = 0.402; P = 0.042, respectively). Anaerobic threshold was associated with changes in Bhalla score over the following 2 years.Exercise testing is more sensitive than spirometry to detect structural changes in CF lungs. Pediatr Pulmonol. 2016; 51:914-920. © 2016 Wiley Periodicals, Inc.

Aim: To compare disease severity assessed by exercise testing, spirometry, MBW and HRCT among 32 patients with CF; to evaluate the deterioration of exercise testing parameters over a two-year period compared to the change of spirometry, LCI and HRCT. Methods: Thirty-two CF patients were evaluated with exercise testing, MBW spirometry, and HRCT over a seven-year period. Correlations among spirometry, exercise testing, MBW and HRCT assessments were performed. Twenty-six patients were evaluated with HRCT, spirometry and exercise testing twice, over a two-year period. The change of each parameter and their correlation, over the two-year period were evaluated. Results: 58 measurements were performed in 32 CF patients (mean age 16.6 years, mean FEV1 80.24%). FEV1 correlated significantly with Bhalla score (p0.001), VOpeak% (p0.014), Breathing Reserve (p

Aim: To compare progression of lung disease assessed with LCI and exercise capacity among children with bronchiectasis. Method: Fourteen stable CF patients and 13 stable children with non-CF bronchiectasis and comparable FEV1 were assessed with HRCT, maximal incremental cardiopulmonary exercise testing and Multiple Breath Washout (MBW) over a two-year period. Results: Fourteen patients with CF (mean age 13.5 years, mean FEV1 =80.1%) and 13 patients with non-CF bronchiectasis (mean age =13.05 years, mean FEV1=76.6%), participated in the study. In both groups, there was evidence of exercise limitation, with mean Peak Aerobic Capacity (VOpeak) 69.2 % predicted (CF group) and 76.4 % (non CF group), (p=0.321). VE/VO2 and VE/VCO2 were significantly impaired in the CF group (p Conclusions: Despite comparable FEV1 values, patients with CF seem to have more impaired exercise capacity and ventilation inhomogeneity, compared to patients with non-CF bronchiectasis. Aggressive treatment may improve lung function and exercise capacity among patients with non-CF bronchiectasis, while exercise capacity and ventilation inhomogeneity deteriorate among patients with CF in a two year period.

Background: Allergic bronchopulmonary aspergillosis (ABPA) is a common complication among patients with CF that produces significant respiratory morbidity. Standard therapy for ABPA includes systemic corticosteroids for a prolonged period of time with potentially severe side effects and use of antifungal agents. Omalizumab is a humanized monoclonal antibody directed against IgE that prevents its binding to receptors on effector cells. It has been shown to be effective in improving asthma control in patients with a strong allergic component. Recently, sporadic reports of omalizumab use against ABPA have been reported. Methods: We present our long term experience of omalizumab in three children with CF and ABPA who were steroid dependent. Results: Five (3.4%) out of 148 children and adolescents with CF followed in our Unit over a seven year period, developed ABPA (75% females, mean age at start of therapy 14.3 years). Omalizumab was given to 3 of them (2 males, mean age 17.1 years, mean FEV1 35%). All three were already experiencing significant side effects from chronic steroid therapy (invasive aspergillosis, diabetes mellitus, depression, relapse of ABPA and severe osteoporosis) and received regular antifugal therapy. Omalizumab treatment was given for a period of 12−18 months. Oral steroids were gradually stopped. Omalizumab resulted in significant improvement of respiratory symptoms and lung function (mean FEV1 52%, p: 0.01). Conclusion: According to our results in a small number of patients, omalizumab has a significant potential as a strategy to control ABPA in steroid dependent patients. However, more studies are needed to confirm these findings.

WS12.5 Lung clearance index and exercise capacity among children with mild CFand non-CF bronchiectasis E. Hatziagorou1, V. Avramidou1, F. Kirvassilis1, K. Kontouli1, V. Georgopoulou2, J. Tsanakas1. 1Paediatric Pulmonology and CF Unit, Aristotle University of Thessaloniki, Hippokration Hospital, 3rd Paediatric Dept, Thessaloniki, Greece; 2Hippokration Hospital, Thessaloniki, Radiology Dept, Thessaloniki, Greece

Background: The positive effect of azithromycin (AZM) on lung function in Cystic Fibrosis (CF) patients have been showed by many clinical trials, but studies were less long than 1 year. Aim: To evaluate the effect of a long course of low dose AZM on lung function in CF patients. Subjects and Methods: 43 patients (26 F) with chronic infection by Pseudomonas aeruginosa (PA), age ranged between 6 and 39 years (mean ±SD: 17.8±7.4 years) had a AZM (250mg/day) 3-years treatment. In all patients FVC, FEV1, FEF 25−75, expressed as percentage of predicted, and SpO2 (%), were measured at 3 month intervals and were compared with the corresponding values recorded at 3 month intervals during 3 years before starting AZM treatment. Linear regression analysis was used and a p value

Background: In Cystic Fibrosis (CF) lung function testing is a useful tool for monitoring progression of lung disease. Studies in CF children have shown that lung clearance index (LCI) is significantly greater than healthy controls. Aim: The objectives of this study were: (a) To compare LCI among CF preschoolers (aged 2 to 5 years) and healthy controls and (b) to assess the effect of two-week iv antibiotic treatment on LCI among CF preschoolers with CF. Method: Twenty-eight children with CF (20 were infected with Pseudomonas aeruginosa) and 27 healthy controls performed Multiple-Breath Washout (MBW) measurements and LCI was assessed. The ones with Ps. aeruginosa infection were measured again one month following a two-week course of iv antibiotics. Results: Children with CF had significantly higher LCI compared to controls (mean difference for CF vs control [95%CI] 2.6 [1.8, 3.7], p< 0.001). Children with CF who were infected with Ps. aeruginosa had significantly higher LCI, when compared with Ps. Negative CF children (mean difference [95%CI]: 1.75 [0.38, 3.5], p< 0.05). LCI was reduced significantly after two-weeks course of iv antibiotics (mean difference [95%CI]: −1.97 [1.2, 3.6], p< 0.001). Conclusions: LCI is a sensitive marker to detect lung function abnormalities among preschoolers with CF. It can be used to assess severity of the disease and the effect of treatment.