Lung clearance index short-term variability in cystic fibrosis: a pre-post pulmonary exacerbation study.

BACKGROUND: Multiple Breath washout (MBW) represents an important tool to detect early a possible pulmonary exacerbation especially in Cystic Fibrosis (CF) disease. Lung clearance index (LCI) is the most commonly reported multiple breath washout (MBW) index and in the last years was used as management measure for evaluation. Our aim was to analyze clinical utility of LCI index variability in pulmonary exacerbation in CF after intravenous (IV) antibiotic therapy. METHODS: A single-center study was conducted at CF Unit of Bambino Gesù Children's Hospital among hospitalized > 3 years patients for pulmonary exacerbations and treated with antibiotic IV treatment for 14 days. MBW and spirometry were evaluated within 72 h of admission to hospital and at the end of hospitalization. Descriptive analysis was conducted and correlations between quantitative variables were investigated. RESULTS: Fifty-seven patients (M22/F35) with an average age 18.56 (± 8.54) years were enrolled. LCI2.5 was significantly reduced at the end of antibiotic treatment in both pediatric and adult populations with an average reduction of -6,99%; 37/57 patients denoted an improvement, 20/57 are stable or worsened in LCI2.5 values and 4/57 (7.02%) had a significant deterioration (> 15%) at end of treatment. On the contrary a significative elevation of FEV1 and FVC were found, respectively of + 7,30% and of + 5,46%. A positive good correlection among LCI 2.5 and Scond (rho = + 0,615, p = 0.000) and LCI 2.5 and Sacin (rho = + 0,649, p = 0.000) and a negative strong correlation between FEV1 and LCI 2.5 were found in post treatment period. A similar modification of LCI 2.5 and FEV1 was noticed in both adult and pediatric population. CONCLUSIONS: LCI may have a role in the routine clinical care of both adult and pediatric CF patients as a good tool to assess response to IV antibiotic end-therapy in the same way as FEV1.

"What Are the Applications for Remote Rehabilitation Management in Cystic Fibrosis?": A Scoping Review Protocol.

BACKGROUND: Telemedicine is an effective, widely used strategy in the field of cystic fibrosis management. The objective of this scoping review is to summarize and analyze the scientific literature with the special focus on the tools and the strategies used in patients with a chronic disease, such as cystic fibrosis. METHODS: This scoping review will be performed in accordance with the Joanna Briggs Institute methodology. In this context, the planned scoping review is a research synthesis that will map the literature on the applications of telemedicine and telemonitoring to the management of cystic fibrosis, with the aim to identify key concepts in the research and work to be conducted that may impact clinical practice. Studies will be included if they meet the following population, concept, and context criteria: all patients with cystic fibrosis receiving treatment with the tools of telemedicine and telemonitoring. No study design, publication type, or data restrictions will be applied. MEDLINE, Scopus, CINHAL, Pedro, Embase, Web of Science, ACM Digital Library, Health Technology Assessment Database (HTA), and Cochrane Central will be searched up to September 2022. DISCUSSION: To the best of our knowledge, this will be the first scoping review to provide a comprehensive overview of the topic. The results could add meaningful information for future research and, especially, for clinical practice, when implementing telerehabilitation in cystic fibrosis treatment. Furthermore, we expect that our work may identify possible knowledge gaps on the topic. The results of this research will be published in a peer-reviewed journal and will be presented at relevant international scientific events, such as in congress or meetings.