ReviewNutrients2021
The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status.
Review in Nutrients, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 69 citations in OpenAlex.
- Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis: A Systematic Review and Meta-analysis.JAMA network open · 2022Pooled it
- CFTR regulates lipid metabolism by relieving adipose inflammation in obesity.International journal of obesity (2005) · 2026Article
- Review
- Article
- Beta cell function and insulin sensitivity during elexacaftor/tezacaftor/ivacaftor therapy in people with cystic fibrosis.Frontiers in endocrinology · 2026Article
- Evolving nutrition therapy in cystic fibrosis: Adapting to the CFTR modulator era.Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition · 2025Review
- The State of Weight in Cystic Fibrosis: Understanding Nutritional Status and Individualizing Nutritional Care in the Modulator Era.Nutrients · 2025Review
- Height Velocity in Pediatric Cystic Fibrosis Under Triple CFTR Modulator Therapy: A Real-Life Monocentric Experience.Journal of clinical medicine · 2025Article
- Population Pharmacokinetics of Elexacaftor, Tezacaftor and Ivacaftor in a Real-World Cohort of Adults with Cystic Fibrosis.Clinical pharmacokinetics · 2025Observational
- Challenges to Optimizing Nutrition in Children With Cystic Fibrosis.Current gastroenterology reports · 2025Review
- Role of the microbiota-gut-lung axis in the pathogenesis of pulmonary disease in children and novel therapeutic strategies.Frontiers in immunology · 2025Review
- Cystic fibrosis-related bone disease: an update on screening, diagnosis, and treatment.Therapeutic advances in endocrinology and metabolism · 2025Review
- Faecal lipid profile as a new marker of fat maldigestion, malabsorption and microbiota.Pediatric research · 2024Observational
- Genetic modifiers of body mass index in individuals with cystic fibrosis.American journal of human genetics · 2024Article
- Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis.Journal of lipid research · 2024Article
- Nutritional Trends in Cystic Fibrosis: Insights from the Italian Cystic Fibrosis Patient Registry.Journal of clinical medicine · 2024Article
- Ventromedial hypothalamic nucleus subset stimulates tissue thermogenesis via preoptic area outputs.Molecular metabolism · 2024Article
- Changes in fecal lipidome after treatment with ivacaftor without changes in microbiome or bile acids.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- The Changing Landscape of Treatment for Cystic Fibrosis Related Diabetes.Journal of clinical & translational endocrinology · 2024Article
- Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis.ERJ open research · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with cystic fibrosis (CF) are at increased risk of malnutrition and growth failure due to multiple factors as a result of suboptimal or absent function of the CFTR chloride channel protein. Dysfunctional CFTR contributes to increased energy expenditure, exocrine pancreatic insufficiency causing impaired dietary macronutrient digestion and absorption, intestinal dysbiosis, and impaired bile acid homeostasis. Poor nutritional status as a result of these mechanisms is associated with decreased lung function, worse clinical outcomes, and ultimately, increased mortality. Nutritional interventions addressing these mechanisms, such as pancreatic enzyme-replacement therapy and enteral caloric supplementation, have improved nutritional status and, by association, clinical outcomes. In the last decade, the advent of medications targeting defective CFTR proteins has revolutionized the care of patients with CF by reducing the overall impact of CFTR dysfunction. Below, we summarize the effects of highly effective CFTR modulators on nutritional status overall as well as specific factors including bile acid metabolism, pancreatic function, energy expenditure, and intestinal dysbiosis. The future of CF nutrition care will require a paradigm shift away from focusing on methods addressing CFTR dysfunction such as excess calorie provision and toward an individualized, holistic approach in the context of specific mutations and CFTR-directed therapy.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.