Registry Study on Cystic Fibrosis in Chinese Children-a Multicenter, Prospective Cohort Study
试验速览
- 阶段
- 不适用
- 状态
- 尚未招募
- 发起方
- 入组人数
- 100
- 主要终点
- Change from baseline in lung function on the spirometry
研究概览
简要总结
This study is a multicenter, prospective cohort study of patients diagnosed with cystic fibrosis, the clinical information of recruited patients, including clinical manifestations, lung function, chest imaging, quality of life and other indicators, will be followed for 10 years.
详细描述
All new cases of cystic fibrosis which was confirmed or probably diagnosed at each center from the beginning of the study are made the investigation of the clinical manifestations, sweat test and CFTR mutations by the standard diagnostic process.Then all the patients' following clinical data will be followed for 10 years (once per six month): clinical manifestations, lung function, chest imaging (once per year), quality of life and other indicators.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- — 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Age 0~18 years old
- •Any organ system symptoms consistent with CF, such as chronic sinopulmonary disease, gastrointestinal and nutritional abnormalities, obstructive azoospermia or having siblings with CF
- •CFTR dysfunction indicated by elevated sweat chloride levels ≥60 mmol/L twice, or one sweat chloride levels ≥40 mmol/L plus presence of two pathogenic CFTR mutations on different alleles
- •Probable CF patients with sweat chloride levels among 40~59 mmol/L plus with presence of 0-1 pathogenic CFTR mutation
- •Consent to provide the related clinical specimen to the certain hospital
- •The guardians of the patients fully understand the purpose of the study, volunteer their children to participate in this study and sign informed consent.
排除标准
- •Subject will be excluded if she or he has one of the following:
- •It is unable to provide complete medical records or the current condition can not accept the diagnosis process.
- •She or he does not agree to participate in the test.
结局指标
主要结局
Change from baseline in lung function on the spirometry
时间窗: ten years
forced expiratory volume at one second (FEV1) in Liter
次要结局
未报告次要终点
研究者
Kunling Shen
Chief of China National Clinical Research Center for Respiratory Diseases
Beijing Children's Hospital