Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients
Abstract Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively an...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2021-07-01
|
| Series: | BMC Pediatrics |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12887-021-02789-8 |
| _version_ | 1819146215563984896 |
|---|---|
| author | Qionghua Chen Yuelin Shen Hui Xu Xiaolei Tang Haiming Yang Shunying Zhao |
| author_facet | Qionghua Chen Yuelin Shen Hui Xu Xiaolei Tang Haiming Yang Shunying Zhao |
| author_sort | Qionghua Chen |
| collection | DOAJ |
| description | Abstract Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. Results Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5–62 months and 0.5–48 months, respectively, and triggered no obvious adverse reactions. Conclusion No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges. |
| first_indexed | 2024-12-22T13:10:23Z |
| format | Article |
| id | doaj.art-f0f1697bec904013a77d9e35859cc912 |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-12-22T13:10:23Z |
| publishDate | 2021-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-f0f1697bec904013a77d9e35859cc9122022-12-21T18:24:45ZengBMCBMC Pediatrics1471-24312021-07-012111810.1186/s12887-021-02789-8Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patientsQionghua Chen0Yuelin Shen1Hui Xu2Xiaolei Tang3Haiming Yang4Shunying Zhao5Department No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthDepartment No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthDepartment No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthDepartment No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthDepartment No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthDepartment No. 2 of Respiratory Medicine, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s HealthAbstract Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. Results Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5–62 months and 0.5–48 months, respectively, and triggered no obvious adverse reactions. Conclusion No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges.https://doi.org/10.1186/s12887-021-02789-8Cystic fibrosisFollow-upChinese children |
| spellingShingle | Qionghua Chen Yuelin Shen Hui Xu Xiaolei Tang Haiming Yang Shunying Zhao Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients BMC Pediatrics Cystic fibrosis Follow-up Chinese children |
| title | Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients |
| title_full | Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients |
| title_fullStr | Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients |
| title_full_unstemmed | Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients |
| title_short | Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients |
| title_sort | prognosis and treatment of 46 chinese pediatric cystic fibrosis patients |
| topic | Cystic fibrosis Follow-up Chinese children |
| url | https://doi.org/10.1186/s12887-021-02789-8 |
| work_keys_str_mv | AT qionghuachen prognosisandtreatmentof46chinesepediatriccysticfibrosispatients AT yuelinshen prognosisandtreatmentof46chinesepediatriccysticfibrosispatients AT huixu prognosisandtreatmentof46chinesepediatriccysticfibrosispatients AT xiaoleitang prognosisandtreatmentof46chinesepediatriccysticfibrosispatients AT haimingyang prognosisandtreatmentof46chinesepediatriccysticfibrosispatients AT shunyingzhao prognosisandtreatmentof46chinesepediatriccysticfibrosispatients |