Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements
Background: Cystic fibrosis (CF) is associated with numerous nutritional and health challenges that significantly impact the patient's quality of life and clinical outcomes. Nutritional management plays a vital role in addressing issues such as pancreatic insufficiency, fat malabsorption, vitam...
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Mashhad University of Medical Sciences
2024-12-01
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| Series: | Reviews in Clinical Medicine |
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| Online Access: | https://rcm.mums.ac.ir/article_25507_507e7478ee9c432076772a8c6c4b5bd6.pdf |
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| author | Salehe Akhondian Saeedeh Talebi |
| author_facet | Salehe Akhondian Saeedeh Talebi |
| author_sort | Salehe Akhondian |
| collection | DOAJ |
| description | Background: Cystic fibrosis (CF) is associated with numerous nutritional and health challenges that significantly impact the patient's quality of life and clinical outcomes. Nutritional management plays a vital role in addressing issues such as pancreatic insufficiency, fat malabsorption, vitamin and mineral deficiencies, and complications like CF-related diabetes (CFRD) and liver disease (CFLD). This review provides an overview of the essential nutritional assessment, management strategies, and the role of CFTR modulator therapies in improving the nutritional status and health outcomes in CF patients.Methods: A comprehensive review of the current literature regarding the nutritional needs, deficiencies, and therapies in CF patients was conducted, including a focus on pancreatic enzyme replacement therapy (PERT), vitamin and mineral supplementation, and the management of bone disease, CFRD, and CFLD. Additionally, the impact of CFTR modulators on nutritional outcomes and related complications was examined.Results: Nutritional assessment in CF is multifaceted, incorporating clinical, anthropometric, body composition, and biochemical evaluations. Early intervention strategies, including PERT and tailored supplementation, are critical for managing fat-soluble vitamin deficiencies, mineral imbalances, and maintaining adequate body mass. The prevalence of CFRD and CFLD significantly impacts CF management, necessitating regular screening and appropriate treatments. CFTR modulator therapies have led to improvements in lung function and nutritional status, though regular monitoring is essential to prevent complications such as hypervitaminosis, altered lipid metabolism, and insulin sensitivity.Conclusion: A proactive, individualized approach to nutritional management is essential in CF, emphasizing early intervention, regular screening, and personalized therapy to address the diverse challenges faced by these patients. |
| format | Article |
| id | doaj-art-a818493b2e1d4e23af17d2b7e40fa96f |
| institution | OA Journals |
| issn | 2345-6256 2345-6892 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Mashhad University of Medical Sciences |
| record_format | Article |
| series | Reviews in Clinical Medicine |
| spelling | doaj-art-a818493b2e1d4e23af17d2b7e40fa96f2025-08-20T02:16:44ZengMashhad University of Medical SciencesReviews in Clinical Medicine2345-62562345-68922024-12-01114152110.22038/rcm.2025.85472.152225507Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic AdvancementsSalehe Akhondian0Saeedeh Talebi1Student Research Committee, School of Dentistry, Mashhad University of Medical Science, Mashhad, IranDepartment of Pediatrics, school of medicine, Mashhad University of Medical Science, Mashhad, IranBackground: Cystic fibrosis (CF) is associated with numerous nutritional and health challenges that significantly impact the patient's quality of life and clinical outcomes. Nutritional management plays a vital role in addressing issues such as pancreatic insufficiency, fat malabsorption, vitamin and mineral deficiencies, and complications like CF-related diabetes (CFRD) and liver disease (CFLD). This review provides an overview of the essential nutritional assessment, management strategies, and the role of CFTR modulator therapies in improving the nutritional status and health outcomes in CF patients.Methods: A comprehensive review of the current literature regarding the nutritional needs, deficiencies, and therapies in CF patients was conducted, including a focus on pancreatic enzyme replacement therapy (PERT), vitamin and mineral supplementation, and the management of bone disease, CFRD, and CFLD. Additionally, the impact of CFTR modulators on nutritional outcomes and related complications was examined.Results: Nutritional assessment in CF is multifaceted, incorporating clinical, anthropometric, body composition, and biochemical evaluations. Early intervention strategies, including PERT and tailored supplementation, are critical for managing fat-soluble vitamin deficiencies, mineral imbalances, and maintaining adequate body mass. The prevalence of CFRD and CFLD significantly impacts CF management, necessitating regular screening and appropriate treatments. CFTR modulator therapies have led to improvements in lung function and nutritional status, though regular monitoring is essential to prevent complications such as hypervitaminosis, altered lipid metabolism, and insulin sensitivity.Conclusion: A proactive, individualized approach to nutritional management is essential in CF, emphasizing early intervention, regular screening, and personalized therapy to address the diverse challenges faced by these patients.https://rcm.mums.ac.ir/article_25507_507e7478ee9c432076772a8c6c4b5bd6.pdfcystic fibrosisnutritional assessmentcftr modulatorsbone diseasecfrdcfld |
| spellingShingle | Salehe Akhondian Saeedeh Talebi Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements Reviews in Clinical Medicine cystic fibrosis nutritional assessment cftr modulators bone disease cfrd cfld |
| title | Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements |
| title_full | Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements |
| title_fullStr | Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements |
| title_full_unstemmed | Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements |
| title_short | Integrated Approaches to Managing Cystic Fibrosis: From Nutritional Optimization to Therapeutic Advancements |
| title_sort | integrated approaches to managing cystic fibrosis from nutritional optimization to therapeutic advancements |
| topic | cystic fibrosis nutritional assessment cftr modulators bone disease cfrd cfld |
| url | https://rcm.mums.ac.ir/article_25507_507e7478ee9c432076772a8c6c4b5bd6.pdf |
| work_keys_str_mv | AT saleheakhondian integratedapproachestomanagingcysticfibrosisfromnutritionaloptimizationtotherapeuticadvancements AT saeedehtalebi integratedapproachestomanagingcysticfibrosisfromnutritionaloptimizationtotherapeuticadvancements |