Abstract | OBJECTIVE: METHODS: Systematic review and meta-analysis were conducted, following PRISMA guidelines. The following databases were searched extensively: PubMed/Medline, Clinical trials.gov, Google Scholar, Scopus, Embase, and Europe PMC using the keywords: " Ivacaftor," " Elexacaftor," " Tezacaftor," VX_661", VX_770", " VX_445", " cystic fibrosis". A total of ten randomized clinical trials were included in our analysis. Primary outcomes included: Absolute change in predicted FEV1 from baseline, Absolute change in sweat chloride test from baseline, Absolute change in BMI from baseline, Absolute change in CF-QR from baseline, and Adverse Events. RESULTS: Among primary findings, significant absolute change in predictive FEV1 from baseline through 4 weeks favoured the triple CFTR protein modulators. [MD=11.80,95%CI=8.47_15.12, p value=<0.00001]; as well as CF_QR score [MD=0.00,95%CI=-2.50_2.50, p value=1.00], and BMI kg/m² change [MD=16.90,95%CI=12.73_21.06, p value=<0.00001]. No significant change was noted for CFTR channels activity in the treatment group when compared to placebo or VX_770/ VX_661 [MD= -12.57,95%CI=-94.46_69.32, p value=0.76]. CONCLUSION: In children aged ≥ 6 y old and adolescents with F508del_CFTR mutation, Elexacaftor- Tezacaftor-Ivacaftor tend to be more effective than first-generation therapy, demonstrating promising results by exhibiting significant improvement in lung function, body weight, and respiratory-related quality of life.
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Authors | Alaa Hassan Yousif Hamdan, Faiza Zakaria, Maria Kezia Lourdes Pormento, Odunayo Susan Lawal, Adaugo Opiegbe, Samina Zahid, Prathima Guntipalli, Ujala Nasr, Syed Asad Hasan Rizvi |
Journal | Current reviews in clinical and experimental pharmacology
(Curr Rev Clin Exp Pharmacol)
(Feb 01 2023)
ISSN: 2772-4336 [Electronic] Netherlands |
PMID | 36722487
(Publication Type: Meta-Analysis)
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