Swiss Primary Ciliary Dyskinesia Registry
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 800
- 试验地点
- 1
- 主要终点
- Lung function measurements
研究概览
简要总结
The Swiss Primary Ciliary Dyskinesia (PCD) Registry is a national patient registry that collects information on diagnosis, symptoms, treatment and follow-up of patients with PCD in Switzerland and provides data for national and international monitoring and research.
详细描述
The Swiss Primary Ciliary Dyskinesia registry (CH-PCD) was founded in 2013 as a collaborative project between epidemiologists and adult and paediatric pulmonologists. It started as a pilot project in the canton of Bern and in 2014 was extended to include all Switzerland. The data centre of the SPCDR is located at the Institute of Social and Preventive Medicine at the University of Bern. It contributes data to the international PCD registry and other international studies such as the international PCD (iPCD) cohort.
Objectives of the CH-PCD:
The Swiss Primary Ciliary Dyskinesia Registry collects information on diagnosis, symptoms, treatment and follow-up of patients with PCD in Switzerland and provides data for national and international monitoring and research. In particular, it aims to:
- Identify all patients diagnosed with PCD in Switzerland.
- Collect population based data (incidence, prevalence, time trends and regional trends).
- Document diagnostic evaluations, treatments and participation in clinical trials.
- Document the clinical course of PCD, quality of life, morbidity and mortality.
- Establish a research platform for clinical, epidemiological and basic research.
Study design:
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Other
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients diagnosed with primary ciliary dyskinesia
- •Signed informed consent or assent
排除标准
- 未提供
结局指标
主要结局
Lung function measurements
时间窗: every 3 months up to 80 years
Spirometric indices, particularly Forced expiratory volume in 1 sec (FEV1) and Forced vital capacity (FVC) z-scores calculated based on Global Lung Function Initiative (GLI) reference values
Chest computed tomography
时间窗: every 3 months up to 80 years
Radiological findings from chest computed tomography
Height
时间窗: every 3 months up to 80 years
Height z-scores calculated based on available national and international references
Vital status
时间窗: every 3 months up to 80 years
Vital status of patient: has the patient died or is he/she still alive and in case of death what was the cause?
Clinical symptoms frequency
时间窗: once a year up to 80 years
Frequency of respiratory symptoms recorded with a patient or parent-reported questionnaire
BMI
时间窗: every 3 months up to 80 years
Body Mass Index (BMI) z-scores calculated based on available national and international references
Measurement of nasal nitric oxide using breath analyzer
时间窗: at study entry (in case patient is <5 years old at study entry, then the outcome will be assessed at age 5
Results of nasal nitric oxide measurement, performed for diagnosis
Cilia ultrastructure identified using electron microscopy analysis
时间窗: at study entry
Results of electron microscopy analysis of cilia, performed for diagnosis
Ciliary beat frequency and beat pattern using video microscopy analysis
时间窗: at study entry
Results of video microscopy analysis of cilia, performed for diagnosis
Microbiological culture of respiratory samples
时间窗: every 3 months up to 80 years
Results of microbiology cultures of respiratory samples (sputum, cough swabs, throat swabs, ear swabs, bronchoalveolar lavage) expressed in a qualitative (growth or no growth of pathogens) or semiquantitative way (the type of sample depends on the age of the patient and the clinic the sample was collected).
Sinus computed tomography
时间窗: every 3 months up to 80 years
Radiological findings from sinus computed tomography
Antibiotic resistance of microbes isolated in microbiological cultures
时间窗: every 3 months up to 80 years
Information on antibiotic resistance of microbes isolated in microbiological cultures (see outcome 7) of respiratory samples, expressed either semiquantitatively or by the value of minimum inhibitory concentration.
次要结局
未报告次要终点