ArticleAnnals of the American Thoracic Society2019
CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis.
Article in Annals of the American Thoracic Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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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.
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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.
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Who cites it
16 citing papers in PubMed, 25 citations in OpenAlex.
- Glycemia and Insulin Secretion in Cystic Fibrosis 2 Years After Elexacaftor/Tezacaftor/Ivacaftor: PROMISE-ENDO.The Journal of clinical endocrinology and metabolism · 2025Observational
- Hemoglobin Levels in Children Treated for Cystic Fibrosis with CFTR Modulators: A Single Center Retrospective Study.Journal of clinical medicine · 2025Article
- Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis: A Review of Registry-Based Evidence.Journal of clinical medicine · 2025Review
- Elexacaftor-tezacaftor-ivacaftor pharmacokinetics with concurrent tacrolimus administration after lung transplant.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Observational
- Association between CFTR modulators and changes in iron deficiency markers in cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- Glycemia and β-cell function before and after elexacaftor/tezacaftor/ivacaftor in youth and adults with cystic fibrosis.Journal of clinical & translational endocrinology · 2022Article
- Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2022Article
- Iron Deficiency in Cystic Fibrosis: A Cross-Sectional Single-Centre Study in a Referral Adult Centre.Nutrients · 2022Article
- Use of proton pump inhibitors is associated with lower hemoglobin levels in people with cystic fibrosis.Pediatric pulmonology · 2021Article
- Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene.JCI insight · 2021Article
- How Clinically Efficient Is Lumacaftor/Ivacaftor for Cystic Fibrosis Patients? An Updated Literature Review.Cureus · 2020Review
- Airway Inflammation and Host Responses in the Era of CFTR Modulators.International journal of molecular sciences · 2020Review
- Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2020Article
- The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.Annals of the American Thoracic Society · 2020Review
- CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis.Annals of the American Thoracic Society · 2019Article
- Exercise Physiology Across the Lifespan in Cystic Fibrosis.Frontiers in physiology · 2019Review
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
rationaleUnderstanding how cystic fibrosis transmembrane conductance regulator (CFTR) modulators influence comorbid conditions like anemia is of great interest to the cystic fibrosis community.
objectivesTo test the hypothesis that CFTR modulators are associated with higher hemoglobin (Hgb) levels.
methodsAnnualized Hgb and other laboratory, demographic, and anthropometric data were abstracted from the U.S. CF Foundation Patient Registry for adult and pediatric registrants before and after therapy with ivacaftor (IVA) or lumacaftor/ivacaftor (LUM/IVA) between January 2010 and December 2016. Univariate and multivariate linear mixed models were used to examine the effect of IVA on Hgb in patients with G551D-CFTR, and the effect of LUM/IVA on Hgb in F508del-CFTR homozygotes. Linear regression was used to characterize change in mean Hgb over time.
resultsA total of 1,347 registrants (707 males and 640 females) with G551D-CFTR and 12,582 F508del-CFTR homozygotes (6,640 males and 5,942 females) who had never undergone lung transplant and had contemporaneous data regarding Hgb and CFTR modulator use were identified. IVA was associated with average Hgb increases of 0.54 gm/dl (95% confidence interval [CI], 0.39-0.69; P < 0.0001) and 0.18 gm/dl (95% CI, 0.01-0.35; P = 0.037) for males and females, respectively, with G551D-CFTR. LUM/IVA was associated with average Hgb increases of 0.58 gm/dl (95% CI, 0.48-0.68; P < 0.0001) and 0.26 gm/dl (95% CI, 0.20-0.33; P < 0.0001) for male and female F508del-CFTR homozygotes, respectively. In multivariate models, IVA positively affected Hgb in males but not females, and LUM/IVA positively affected Hgb in both sexes.
conclusionsIVA and LUM/IVA use are both associated with higher Hgb levels in patients with CF.
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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.