{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T03:15:37Z","timestamp":1789269337291,"version":"build-2803163510"},"reference-count":52,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T00:00:00Z","timestamp":1774915200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T00:00:00Z","timestamp":1774915200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100004835","name":"Zhejiang University","doi-asserted-by":"publisher","award":["B202311"],"award-info":[{"award-number":["B202311"]}],"id":[{"id":"10.13039\/501100004835","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Cystic fibrosis (CF) is a life\u2010threatening genetic disorder characterized by dysfunctional mucus production, leading to severe respiratory and digestive complications. CF transmembrane conductance regulator (CFTR) modulators are highly effective targeted therapies for CF; however, they are not yet available in mainland China, resulting in no published reports on their use in Chinese CF patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To report a case of CF patient with compound heterozygous CFTR mutations (c.2909G\u232aA\/c.2491\u20102A\u232aG) treated withelexacaftor\/tezacaftor\/ivacaftor (ETI) in China and summarize the diagnosis and treatment status based on a literature review, exploring the potential efficacy of CFTR modulators in the Chinese population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Case Report: Clinical data and treatment response of a CF patient receiving ETI were analyzed. Literature Review: PubMed, CNKI, and Wanfang were searched (Jan 2010\u2010Aug 2025) to identify CF cases reported in China. A total of 71 cases were included for analysis of diagnosis, treatment, and outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Case Outcome: The patient showed significant clinical improvement within 2 weeks after ETI initiation. Literature Analysis: The 71 cases demonstrated heterogeneity in diagnosis and treatment, with most relying on supportive therapies and lacking gene\u2010targeted interventions. Status Summary: CF diagnosis rates are low in China, genetic testing is insufficient, and CFTR modulator accessibility is limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study presents the first report of CFTR modulator efficacy in a Chinese patient, indicating potential treatment benefits. It underscores the urgent need to improve CF awareness, establish a CF genetic mutation database, and expedite CFTR modulator approval\/access to promote personalized treatment and outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71590","type":"journal-article","created":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T06:27:32Z","timestamp":1775024852000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Current Status and the Need for\n                    <i>CFTR<\/i>\n                    Modulator Therapy in Cystic Fibrosis Patients in Mainland China: A Case Report and Literature Review"],"prefix":"10.1002","volume":"61","author":[{"given":"Yuxiao","family":"Sun","sequence":"first","affiliation":[{"name":"Department of Pediatrics, The First Affiliated Hospital Zhejiang University School of Medicine Hangzhou Zhejiang 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