The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.
Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations
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The source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“Treatment with ELX/TEZ/IVA led to robust and clinically meaningful improvements in lung function (as assessed by percent predicted forced expiratory volume in 1 second [ppFEV 1 ]), CFTR function (as assessed by sweat chloride concentration), and respiratory symptoms (as assessed by Cystic Fibrosis Questionnaire-Revised respiratory domain score), exceeding improvements seen with the dual CFTR Modul”
From Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study (2023) · cites this paper as [11]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“These results demonstrated that ELX/TEZ/IVA is a superior treatment option for people with CF with ≥1 F508del-CFTR allele.”
From Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study (2023) · cites this paper as [11]
Experimental — accuracy not yet measuredIn the citing paper
“Treatment with ELX/TEZ/IVA led to robust and clinically meaningful improvements in lung function (as assessed by percent predicted forced expiratory volume in 1 second [ppFEV 1 ]), CFTR function (as assessed by sweat chloride concentration), and respiratory symptoms (as assessed by Cystic Fibrosis Questionnaire-Revised respiratory domain score), exceeding improvements seen with the dual CFTR Modul”
From Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study (2023) · cites this paper as [11]
In the citing paper
“Treatment with ELX/TEZ/IVA led to robust and clinically meaningful improvements in lung function (as assessed by percent predicted forced expiratory volume in 1 second [ppFEV 1 ]), CFTR function (as assessed by sweat chloride concentration), and respiratory symptoms (as assessed by Cystic Fibrosis Questionnaire-Revised respiratory domain score), exceeding improvements seen with the dual CFTR Modul”
From Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study (2023) · cites this paper as [11]
In the citing paper
“Treatment with ELX/TEZ/IVA led to robust and clinically meaningful improvements in lung function (as assessed by percent predicted forced expiratory volume in 1 second [ppFEV 1 ]), CFTR function (as assessed by sweat chloride concentration), and respiratory symptoms (as assessed by Cystic Fibrosis Questionnaire-Revised respiratory domain score), exceeding improvements seen with the dual CFTR Modul”
From Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study (2023) · cites this paper as [11]
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Citations
Cites
Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele
Middleton, P. G., Mall, M. A., Dřevínek, P., Lands, L. C., McKone, E. F., Polineni, D., Ramsey, B. W., Taylor-Cousar, J. L., Tullis, E., Vermeulen, F., et al. (2019) · New England Journal of Medicine · doi:10.1056/nejmoa1908639 · Cited by 2,198
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
Self-citationHeijerman, H. G. M., McKone, E. F., Downey, D. G., Van Braeckel, E., Rowe, S. M., Tullis, E., Mall, M. A., Welter, J. J., Ramsey, B. W., McKee, C. M., et al. (2019) · The Lancet · doi:10.1016/s0140-6736(19)32597-8 · Cited by 1,414
Self-citation
Flagged first, then by citations
Research works printed but not linked: 13
- De Boeck, 2016, Progress in therapies for cystic fibrosis, Lancet Respir Med, 4, 662, 10.1016/S2213-2600(16)00023-0 · DOI
- Riordan, 1989, Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA, Science, 245, 1066, 10.1126/science.2475911 · DOI · Paper page
- Dalemans, 1991, Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation, Nature, 354, 526, 10.1038/354526a0 · DOI · Paper page
- Van Goor, 2009, Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770, Proc Natl Acad Sci U S A, 106, 18825, 10.1073/pnas.0904709106 · DOI
- Van Goor, 2016, Nonclinical profile of the CFTR corrector VX-661, Pediatr Pulmonol, 51
- Rowe, 2017, Tezacaftor-ivacaftor in residual-function heterozygotes with cystic fibrosis, N Engl J Med, 377, 2024, 10.1056/NEJMoa1709847 · DOI · Paper page
- Taylor-Cousar, 2017, Tezacaftor–ivacaftor in patients with cystic fibrosis homozygous for Phe508del, N Engl J Med, 377, 2013, 10.1056/NEJMoa1709846 · DOI · Paper page
- Taylor-Cousar, 2019, Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles, ERJ Open Res, 5, 10.1183/23120541.00082-2019 · DOI · Paper page
- Elborn, 2016, Cystic fibrosis, Lancet, 388, 2519, 10.1016/S0140-6736(16)00576-6 · DOI · Paper page
- Davies, 2018, VX-659–Tezacaftor–ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles, N Engl J Med, 379, 1599, 10.1056/NEJMoa1807119 · DOI · Paper page
- Boyle, 2014, A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a Phe508del CFTR mutation: a phase 2 randomised controlled trial, Lancet Respir Med, 2, 527, 10.1016/S2213-2600(14)70132-8 · DOI
- Keating, 2018, VX-445–Tezacaftor–ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles, N Engl J Med, 379, 1612, 10.1056/NEJMoa1807120 · DOI · Paper page
- Wainwright, 2015, Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR, N Engl J Med, 373, 220, 10.1056/NEJMoa1409547 · DOI · Paper page
Cited by
Sorted by citations
Bower, J. K., Volkova, N., Ahluwalia, N., Sahota, G., Xuan, F., Chin, A., Weinstock, T. G., Ostrenga, J., & Elbert, A. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2023.03.002 · Cited by 140
Self-citation
Metadata
- Authors
- Munck, A., Kerem, E., Ellemunter, H., Campbell, D., Wang, L. T., Ahluwalia, N., Owen, C. A., & Wainwright, C.
- ORCID
- Ellemunter, Helmut
- Venue
- Journal of Cystic Fibrosis
- Publisher
- Elsevier BV
- Published
- 2020-11
- Volume
- 19
- Issue
- 6
- Pages
- 962-968
- ISSN
- 1569-1993
- Cited by
- 33
- References
- 3
- Language
- en
- Licence
- Funders
- Vertex Pharmaceuticals
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