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| Content Provider | Springer Nature : BioMed Central |
|---|---|
| Author | Zhou, Wangji Wang, Yaqi Yang, Yanli Sun, Yanyan Cheng, Chongsheng Dai, Jinrong Meng, Shuzhen Chen, Keqi Zhao, Yang Liu, Xueqi Zhang, Dingding Liu, Song Zhu, Weiguo Liu, Yaping Xu, Kai-Feng Tian, Xinlun |
| 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 < 50%, and high CF-ABLE and 3-year prognostic scores were associated with mortality in patients with CF in China. Conclusions We reported the progression and mortality of patients with CF in China, which was a rare and relatively unknown population in the past. Baseline FEV1% 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. |
| Related Links | https://ojrd.biomedcentral.com/counter/pdf/10.1186/s13023-024-03522-1.pdf |
| Ending Page | 9 |
| Page Count | 9 |
| Starting Page | 1 |
| File Format | HTM / HTML |
| ISSN | 17501172 |
| DOI | 10.1186/s13023-024-03522-1 |
| Journal | Orphanet Journal of Rare Diseases |
| Issue Number | 1 |
| Volume Number | 20 |
| Language | English |
| Publisher | BioMed Central |
| Publisher Date | 2025-01-07 |
| Access Restriction | Open |
| Subject Keyword | Medicine Public Health Pharmacology Toxicology Human Genetics Chinese Cystic fibrosis Lung function Prognosis Mortality Medicine/Public Health Pharmacology/Toxicology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Pharmacology (medical) Genetics (clinical) |
| Journal Impact Factor | 3.4/2023 |
| 5-Year Journal Impact Factor | 3.9/2023 |
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