A bibliometric analysis of cystic fibrosis transmembrane conductance regulators.

Abstract

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.