https://doi.org/10.4081/monaldi.2024.3004

A bibliometric analysis of cystic fibrosis transmembrane conductance regulators

Early Access
Submitted: March 27, 2024
Accepted: June 3, 2024
Published: July 24, 2024
Cystic fibrosis, CFTR protein, CFTR modulators, bibliometric analysis, scopus
Abstract Views: 484
PDF_EARLY VIEW: 119
SUPPLEMENTARY MATERIAL: 17
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Authors

Bashar Araiqat
bbaraiqat189@med.just.edu.jo
https://orcid.org/0000-0003-4728-6707
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Sebawe Syaj
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Aseel Al-Khatib
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Nour Al-Bzour
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Narmine Hussein
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Sarah Aqel
https://orcid.org/0009-0003-4590-509X
Medical Research Center, Hamad Medical Corporation, Doha, Qatar.
Jamil Ahmad
Department of Urology, Hamad Medical Corporation, Doha, Qatar.

Cystic fibrosis (CF), a multisystem disease primarily affecting the lungs, arises due to pathogenic mutations in the CF transmembrane conductance regulator (CFTR) gene. This study embarked on a bibliometric analysis to survey the use of CFTR modulators in CF treatment. Utilizing the Scopus database, a comprehensive search was executed, incorporating terms related to CF and CFTR modulators. Various document types up to July 19, 2023, were included, with citation counts forming the basis of our analyses. Trends, contributor countries, leading institutions, top authors, journals, keywords, and annual citation trends were evaluated. Our search retrieved 2317 records, predominantly articles. The United States dominated in both publications and citations, followed by the United Kingdom. The University of Alabama, Birmingham, and Vertex Pharmaceuticals, Boston, were among the top institutions. Rowe S.M. was identified as a top-cited author. The Journal of Cystic Fibrosis emerged as the leading journal in terms of publication volume, while the New England Journal of Medicine had the highest citation count. The most-cited article addressed a CFTR potentiator's efficacy in patients with the G551D mutation. The keyword "Cystic fibrosis" appeared most frequently. This bibliometric analysis underscores the significant research focus on CF, especially concerning CFTR modulators. The results highlight the pivotal role of certain countries, institutions, authors, and journals in the progression of CF research, offering insights into current trends and future research directions.

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Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med 2005;352:1992-2001. DOI: https://doi.org/10.1056/NEJMra043184
Ratjen F, Döring G. Cystic fibrosis. Lancet 2003;361:681-9. DOI: https://doi.org/10.1016/S0140-6736(03)12567-6
Hamosh A, FitzSimmons SC, Macek MJ, et al. Comparison of the clinical manifestations of cystic fibrosis in black and white patients. J Pediatr 1998;132:255-9. DOI: https://doi.org/10.1016/S0022-3476(98)70441-X
O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet 2009;373:1891-904. DOI: https://doi.org/10.1016/S0140-6736(09)60327-5
Stewart C, Pepper MS. Cystic fibrosis on the African continent. Genet Med 2016;18:653-62. DOI: https://doi.org/10.1038/gim.2015.157
Yamashiro Y, Shimizu T, Oguchi S, et al. The estimated incidence of cystic fibrosis in Japan. J Pediatr Gastroenterol Nutr 1997;24:544-7. DOI: https://doi.org/10.1002/j.1536-4801.1997.tb00648.x
Guo X, Liu K, Liu Y, et al. Clinical and genetic characteristics of cystic fibrosis in CHINESE patients: a systemic review of reported cases. Orphanet J Rare Dis 2018;13:224. DOI: https://doi.org/10.1186/s13023-018-0968-2
Guo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros 2022;21:456-62. DOI: https://doi.org/10.1016/j.jcf.2022.01.009
Yankaskas JR, Marshall BC, Sufian B, et al. Cystic fibrosis adult care: consensus conference report. Chest 2004;125:1S-39S. DOI: https://doi.org/10.1378/chest.125.1_suppl.1S
Stern RC. The diagnosis of cystic fibrosis. N Engl J Med 1997;336:487-91. DOI: https://doi.org/10.1056/NEJM199702133360707
Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 1998;132:589-95. DOI: https://doi.org/10.1016/S0022-3476(98)70344-0
Middleton PG, Taylor-Cousar JL. Development of elexacaftor - tezacaftor - ivacaftor: highly effective CFTR modulation for the majority of people with Cystic Fibrosis. Expert Rev Respir Med 2021;15:723-35. DOI: https://doi.org/10.1080/17476348.2021.1855980
Skilton M, Krishan A, Patel S, et al. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. Cochrane Database Syst Rev 2019;1:CD009841. DOI: https://doi.org/10.1002/14651858.CD009841.pub3
Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011;365:1663-72. DOI: https://doi.org/10.1056/NEJMoa1105185
De Boeck K. Cystic fibrosis in the year 2020: a disease with a new face. Acta Paediatr 2020;109:893-9. DOI: https://doi.org/10.1111/apa.15155
Reis FJC, Damaceno N. Cystic fibrosis. J Pediatr (Rio J) 1998;74:S76-94. [Article in Portuguese]. DOI: https://doi.org/10.2223/JPED.489
Klimova B, Kuca K, Novotny M, Maresova P. Cystic fibrosis revisited - a review study. Med Chem 2017;13:102-9. DOI: https://doi.org/10.2174/1573406412666160608113235
Leso V, Romano R, Santocono C, et al. The impact of cystic fibrosis on the working life of patients: a systematic review. J Cyst Fibros 2022;21:361-9. DOI: https://doi.org/10.1016/j.jcf.2021.08.011
Begum M, Lewison G, Wright JSF, et al. European non-communicable respiratory disease research, 2002-13: bibliometric study of outputs and funding. PLoS One 2016;11:e0154197. DOI: https://doi.org/10.1371/journal.pone.0154197
Zhong H, Zhou Y, Mei SY, et al. Scars of COVID-19: a bibliometric analysis of post-COVID-19 fibrosis. Front Public Health 2022;10:967829. DOI: https://doi.org/10.3389/fpubh.2022.967829
Bell SC, Mall MA, Gutierrez H, et al. The future of cystic fibrosis care: a global perspective. Lancet Respir Med 2020;8:65-124. DOI: https://doi.org/10.1016/S2213-2600(19)30337-6
Cutting GR. Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet 2015;16:45-56. DOI: https://doi.org/10.1038/nrg3849
Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013;187:680-9. DOI: https://doi.org/10.1164/rccm.201207-1160OE
Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 2007;29:522-6. DOI: https://doi.org/10.1183/09031936.00099506

How to Cite

Araiqat, Bashar, Sebawe Syaj, Aseel Al-Khatib, Nour Al-Bzour, Narmine Hussein, Sarah Aqel, and Jamil Ahmad. 2024. “A Bibliometric Analysis of Cystic Fibrosis Transmembrane Conductance Regulators”. Monaldi Archives for Chest Disease, July. https://doi.org/10.4081/monaldi.2024.3004.
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