| Title: |
Progression and mortality of patients with cystic fibrosis in China |
| Authors: |
Wangji Zhou, Yaqi Wang, Yanli Yang, Yanyan Sun, Chongsheng Cheng, Jinrong Dai, Shuzhen Meng, Keqi Chen, Yang Zhao, Xueqi Liu, Dingding Zhang, Song Liu, Weiguo Zhu, Yaping Liu, Kai-Feng Xu, Xinlun Tian |
| Source: |
Orphanet Journal of Rare Diseases, Vol 20, Iss 1, Pp 1-9 (2025) |
| Publisher Information: |
BMC, 2025. |
| Publication Year: |
2025 |
| Collection: |
LCC:Medicine |
| Subject Terms: |
Chinese, Cystic fibrosis, Lung function, Prognosis, Mortality, Medicine |
| More Details: |
Abstract Background Patients with cystic fibrosis (CF) are rare in China and differ significantly from the Caucasian populations in terms of clinical and genetic characteristics. However, the progression and mortality of Chinese patients with CF have not been well described. Results 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%, P = 0.001) and a higher body mass index (19.6 vs. 17.7 kg/m2, P = 0.045). According to the mixed-effects model, for patients ≤ 30 years of age at diagnosis, FEV1% predicted decreased 1.17% per year. The generalized linear regression model showed that higher baseline FEV1% 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 FEV1% predicted |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
1750-1172 |
| Relation: |
https://doaj.org/toc/1750-1172 |
| DOI: |
10.1186/s13023-024-03522-1 |
| Access URL: |
https://doaj.org/article/8b97e47d284a4f328ad91ecf868882cb |
| Accession Number: |
edsdoj.8b97e47d284a4f328ad91ecf868882cb |
| Database: |
Directory of Open Access Journals |
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