BMC Pediatr. 2025 Oct 3;25(1):753. doi: 10.1186/s12887-025-06161-y.
ABSTRACT
BACKGROUND: This study aimed to evaluate the efficacy of different treatment modalities in children with cystic fibrosis (CF) and determine the superiority of specific treatment modalities.
METHODS: A comprehensive literature search was conducted using different search strings across multiple databases, including PubMed, Cochrane Library, EMBASE, WOS, Scopus, CINAHL, PsycINFO, and Google Scholar, up to October 2024. Randomized controlled trials (RCTs), case-control studies and cohort studies were included.
RESULTS: The triple therapy indicated a significant reduction in CF-related complications, with an OR of 0.29 and an RR of 0.54, accompanied by low heterogeneity (I² = 0% for both). Physiotherapy and pulmonary exercises also yielded a beneficial effect, with an OR of 0.24 and an RR of 0.49, without heterogeneity. In contrast, nutritional interventions revealed non-significant outcomes (OR = 6.91 and RR = 2.63), suggesting the need to re-evaluate these strategies. Ivacaftor alone did not achieve statistical significance (OR = 0.34 and RR = 0.58), and the confidence intervals were broad, indicating uncertainty in the effect estimates. Azithromycin exhibited a positive effect on CF management, with an OR of 2.37 and an RR of 1.54. The overall pooled OR across all treatments was 0.71, with an RR not computed due to substantial heterogeneity (I²=93%).
CONCLUSION: The study underscores the effectiveness of certain treatments, such as triple therapy and physiotherapy exercises, for CF while highlighting the considerable variability in treatment outcomes. Notably, nutritional interventions need to be carefully reassessed. The findings emphasize integrating physiotherapy and targeted pharmacological interventions into standard CF management tailored to individual needs.
PMID:41044511 | DOI:10.1186/s12887-025-06161-y
