ArticleRespiratory research2023
Heterogeneity of weight gain after initiation of Elexacaftor/Tezacaftor/Ivacaftor in people with cystic fibrosis.
Article in Respiratory research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 28 citations in OpenAlex.
- The evolving understanding of cystic fibrosis-related diabetes in the highly effective modulator therapy era: a scoping review.Diabetologia · 2026Article
- Prevalence of Overweight and Obesity in the Era of CFTR Modulators in Patients with Cystic Fibrosis.Nutrients · 2026Article
- The impact of elexacaftor-tezacaftor-ivacaftor on cardiometabolic risk factors: a systematic review.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Long-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor on Nocturnal Cardiorespiratory Polygraphy Parameters in Patients with Cystic Fibrosis: A Prospective Study.Life (Basel, Switzerland) · 2025Article
- Efficacy of elexacaftor/tezacaftor/ivacaftor in non-cystic fibrosis bronchiectasis patients with a single pathogenicERJ open research · 2025Article
- Height Velocity in Pediatric Cystic Fibrosis Under Triple CFTR Modulator Therapy: A Real-Life Monocentric Experience.Journal of clinical medicine · 2025Article
- Interrelationships between diet quality and health-related quality of life in Irish adults living with cystic fibrosis.European journal of nutrition · 2025Article
- 20 years of the Montreal Cystic Fibrosis Related Diabetes Screening Cohort: key insights.European respiratory review : an official journal of the European Respiratory Society · 2025Review
- Nasal Epithelium Transcriptomics Predict Clinical Response to Elexacaftor/Tezacaftor/Ivacaftor.American journal of respiratory cell and molecular biology · 2024Article
- Is Obesity a Problem in New Cystic Fibrosis Treatments?Nutrients · 2024Observational
- Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis.Journal of lipid research · 2024Article
- Predicting weight gain in patients with cystic fibrosis on triple combination modulator.Pediatric pulmonology · 2024Article
- Article
- Longitudinal Profiling of the Intestinal Microbiome in Children with Cystic Fibrosis Treated with Elexacaftor-Tezacaftor-Ivacaftor.medRxiv : the preprint server for health sciences · 2023Article
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Authors and funding
10 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe introduction of the novel therapy, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has been effective in improving weight gain in both clinical trials and real-world studies. However, the magnitude of this effect appears to be heterogeneous across patient subgroups. This study aims to identify potential determinants of heterogeneity in weight gain following 6-month ETI therapy.
methodsWe conducted a multicenter, prospective cohort study enrolling 92 adults with CF at two major CF centers in Italy with follow-up visit at one month and six months from ETI initiation. The treatment's effect on weight changes was evaluated using mixed effect regression models that included subject-specific random intercepts and fixed effects for potential predictors of treatment response, time and a predictor-by-time interaction term.
resultsThe mean weight gain at six months from the start of treatment was 4.6 kg (95% CI: 2.3-6.9) for the 10 patients with underweight, 3.2 kg (95% CI: 2.3-4.0) for the 72 patients with normal weight, and 0.7 kg (95% CI: -1.6-3.0) for the 10 patients with overweight. After six months of ETI treatment, 8 (80%) of the patients with underweight transitioned to the normal weight category, while 11 (15.3%) of the normal-weight patients became overweight. The major determinants of heterogeneity in weight gain were the baseline BMI and the presence of at least one CFTR residual function mutation, explaining 13% and 8% of the variability, respectively.
conclusionsOur results indicate that ETI is highly effective in improving weight gain in underweight subjects with CF. However, our data also suggests the need for close monitoring of excess weight gain to prevent potential cardiometabolic complications.
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