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However, the progression and mortality of Chinese patients with CF have not been well described.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>This study included all 67 patients from the Peking Union Medical College Hospital CF cohort, with a median followed up time of 5.2 years. Compared to patients diagnosed with CF in childhood, adult-diagnosed patients exhibit a lower proportion of pancreatic exocrine insufficiency (25.0% vs. 77.8%, <jats:italic>P<\/jats:italic>\u2009=\u20090.001) and a higher body mass index (19.6 vs. 17.7\u00a0kg\/m<jats:sup>2<\/jats:sup>, <jats:italic>P<\/jats:italic>\u2009=\u20090.045). According to the mixed-effects model, for patients\u2009\u2264\u200930 years of age at diagnosis, FEV<jats:sub>1<\/jats:sub>% predicted decreased 1.17% per year. The generalized linear regression model showed that higher baseline FEV<jats:sub>1<\/jats:sub>% predicted and occurrence of pulmonary exacerbations were associated with the progression of patients with CF. The survival rates at 5 years and 10 years after the diagnosis were 96.7% and 80.6%, respectively. The log-rank test showed baseline FEV<jats:sub>1<\/jats:sub>% predicted\u2009&lt;\u200950%, and high CF-ABLE and 3-year prognostic scores were associated with mortality in patients with CF in China.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>We reported the progression and mortality of patients with CF in China, which was a rare and relatively unknown population in the past. Baseline FEV<jats:sub>1<\/jats:sub>% predicted is associated with progression and mortality. Pulmonary exacerbations can accelerate the decline in lung function. The CF-ABLE and 3-year prognostic scores are applicable for predicting poor prognosis in patients with CF in China.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-024-03522-1","type":"journal-article","created":{"date-parts":[[2025,1,7]],"date-time":"2025-01-07T10:26:44Z","timestamp":1736245604000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Progression and mortality of patients with cystic fibrosis in China"],"prefix":"10.1186","volume":"20","author":[{"given":"Wangji","family":"Zhou","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaqi","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanli","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanyan","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chongsheng","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinrong","family":"Dai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuzhen","family":"Meng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keqi","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xueqi","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dingding","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Song","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiguo","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaping","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai-Feng","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4307-6665","authenticated-orcid":false,"given":"Xinlun","family":"Tian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,1,7]]},"reference":[{"issue":"18","key":"3522_CR1","doi-asserted-by":"publisher","first-page":"1693","DOI":"10.1056\/NEJMra2216474","volume":"389","author":"H Grasemann","year":"2023","unstructured":"Grasemann H, Ratjen F. 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