{"doi":"10.1002/ppul.25781","title":"Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients","abstract":"<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Cystic fibrosis (CF) is a multisystem disorder that results in the buildup of mucus in various organs. Ninety percent of CF patients are classified as pancreatic insufficient, leading to malabsorption of nutrients and fat‐soluble vitamins without the assistance of exogenous pancreatic enzymes. This study was designed to determine if serum 25‐hydroxyvitamin D concentrations were impacted by initiation of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA).</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Serum 25‐hydroxyvitamin D concentrations were measured before and 1 year post‐ELX/TEZ/IVA initiation. A Wilcoxon signed‐rank test was used to compare values.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Seventy‐six patients were included in the final analysis. The average age of our population was 25.8 years (<jats:italic>SD</jats:italic> = 13.2 years) with a majority being male, homozygous F508del, pancreatic insufficient, and not modulator‐naive. The median increase of serum vitamin D concentration after initiating ELX/TEZ/IVA was 5 ng/ml (interquartile range = −4, 13; <jats:italic>p</jats:italic> = .0035).</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>We suggest that ELX/TEZ/IVA may improve fat‐soluble vitamin absorption, specifically serum 25‐hydroxyvitamin D. These results may lead to adjustments in vitamin supplementation in patients receiving cystic fibrosis transmembrane conductance regulator modulator therapy.</jats:p></jats:sec>","journal":"Pediatric Pulmonology","year":2022,"id":627526,"datarank":0.4943755299006494,"base_score":3.295836866004329,"endowment":3.295836866004329,"self_citation_contribution":0.4943755299006494,"citation_network_contribution":0.0,"self_endowment_contribution":0.4943755299006494,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":26,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1624188,"name":"Natalie K. Ketchen","orcid":null,"position":1,"is_corresponding":false},{"id":1624189,"name":"Leah N. Rasmussen","orcid":null,"position":2,"is_corresponding":false},{"id":1096726,"name":"Alyssa R. Bartels","orcid":"0000-0002-3988-2843","position":3,"is_corresponding":false},{"id":766972,"name":"Sachinkumar B. Singh","orcid":"0000-0002-7935-2003","position":4,"is_corresponding":false},{"id":1493241,"name":"Brittany A. Wright","orcid":"0000-0001-6620-1727","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients","abstract":"<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Cystic fibrosis (CF) is a multisystem disorder that results in the buildup of mucus in various organs. Ninety percent of CF patients are classified as pancreatic insufficient, leading to malabsorption of nutrients and fat‐soluble vitamins without the assistance of exogenous pancreatic enzymes. This study was designed to determine if serum 25‐hydroxyvitamin D concentrations were impacted by initiation of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA).</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Serum 25‐hydroxyvitamin D concentrations were measured before and 1 year post‐ELX/TEZ/IVA initiation. A Wilcoxon signed‐rank test was used to compare values.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Seventy‐six patients were included in the final analysis. The average age of our population was 25.8 years (<jats:italic>SD</jats:italic> = 13.2 years) with a majority being male, homozygous F508del, pancreatic insufficient, and not modulator‐naive. The median increase of serum vitamin D concentration after initiating ELX/TEZ/IVA was 5 ng/ml (interquartile range = −4, 13; <jats:italic>p</jats:italic> = .0035).</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>We suggest that ELX/TEZ/IVA may improve fat‐soluble vitamin absorption, specifically serum 25‐hydroxyvitamin D. These results may lead to adjustments in vitamin supplementation in patients receiving cystic fibrosis transmembrane conductance regulator modulator therapy.</jats:p></jats:sec>","is_dataset_classified":null,"base_score":3.295836866004329,"endowment":3.295836866004329,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"34859619","pmcid":null,"openalex_id":"https://openalex.org/W3215953147","authors":[],"funders":[],"total_grants":0,"fwci":2.451,"citation_percentile":0.90227675,"influential_citations":0,"citation_trend":[{"year":2022,"count":5},{"year":2023,"count":7},{"year":2024,"count":5},{"year":2025,"count":7},{"year":2026,"count":2}],"oa_status":"closed","license":"http://onlinelibrary.wiley.com/termsAndConditions#vor","oa_locations":[{"url":"https://onlinelibrary.wiley.com/doi/pdf/10.1002/ppul.25781","host_type":"publisher"},{"url":"https://onlinelibrary.wiley.com/doi/full-xml/10.1002/ppul.25781","host_type":"publisher"},{"url":"https://doi.org/10.1002/ppul.25781","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/34859619","host_type":"repository"}],"fields_of_study":["Cystic Fibrosis Research Advances","Vitamin D Research Studies","Folate and B Vitamins Research","Adolescent","Adult","Aminophenols","Benzodioxoles","Child","Chloride Channel Agonists","Cystic Fibrosis","Cystic Fibrosis Transmembrane Conductance Regulator","Female","Humans","Indoles","Male","Mutation","Pyrazoles","Pyridines","Pyrrolidines","Quinolones","Vitamin D","Vitamins","Young Adult"],"mesh_terms":["Adolescent","Adult","Aminophenols","Child","Cystic Fibrosis","Female","Humans","Indoles","Male","Mutation","Pyrazoles","Pyridines","Pyrrolidines","Vitamin D","Vitamins","Quinolones","Cystic Fibrosis Transmembrane Conductance Regulator","Benzodioxoles","Young Adult","Chloride Channel Agonists"],"keywords":["Ivacaftor","Cystic fibrosis","Medicine","Cystic fibrosis transmembrane conductance regulator","Vitamin D and neurology","Interquartile range","Gastroenterology","Malabsorption","Internal medicine","Population","Mucus","Endocrinology","Biology","Cftr Modulators","Vitamins And Nutrition"],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-04T17:51:08.362856Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}