Idiopathic Interstitial Pneumopathy : Genetic and Environmental Determinants From Infancy to Elderly
试验速览
- 阶段
- 不适用
- 状态
- 终止
- 发起方
- 入组人数
- 1,600
- 试验地点
- 4
- 主要终点
- The phenotypic description idiopathic lung disease
研究概览
简要总结
The main objective is to describe the phenotypic features of the paediatric and adult patients with Idiopathic Interstitial Pneumopathy/Pneumopathy Interstitial Diffuse (IIP/PID), at diagnosis and during the follow-up. These data will be critical for the description of the natural history of the various forms of IIP/PID.
详细描述
The French RaDiCo-PID : Idiopathic Interstitial Pneumopathy registry is an ongoing observational prospective and retrospective cohort with longitudinal long-term follow-up includes pediatric and adult patients with Idiopathic Interstitial Lung Disease (ILD) from the reference and competence centers.
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
- •Clinical criteria: chronic respiratory insufficiency manifestations including dyspnea/tachypnea, cough, and cyanosis during exercise or at rest
- •Radiological criteria: characteristic chest High-Resolution Computed Tomography (HRCT) abnormalities including widespread ground glass or alveolar attenuation, reticulation often associated with traction bronchiectasis, and honeycombing
- •Functional criteria: pulmonary function test abnormalities reflecting a restrictive pattern and including: loss of lung volume, vital capacity (VC), total lung capacity (TLC); reduction in the diffusion capacity of the lung for carbon monoxide (DLCO), gas exchange abnormalities, and altered ventilatory response to exercise
- •Patients (parents/guardians for paediatric/patients) having given an informed consent to participate in the protocol
- •Patients affiliated to the "Regime National d'Assurance Maladie"
排除标准
- •Patients with diffuse parenchymal lung diseases caused by drug toxicity, immunodeficiency, proliferative disorders including histiocytosis, and metabolic disorders
- •Patients (parents/guardians for paediatric patient) not able to approve/understand the protocol
研究组 & 干预措施
children or adults with Idiopathic Lung Disease
结局指标
主要结局
The phenotypic description idiopathic lung disease
时间窗: Up to 10 years
Phenotypic description will be measure demographic data, environmental data, socio-professionnal data, medical history, comorbidities, clinical examination, biological assessment (hematology; biochemistry; hemostasis...), pulmonary biopsy, bronchial-pulmonary imaging; symptom description; respiratory function (arterial blood gas, pulmonary fonction testing, six minute-walk testing, cardiopulmonary exercise testing, polysomnography), treatments, quality of life questionnaire (SF36 and SF10)
次要结局
- Identify gene factors involved in disease initiation and progression(Up to 10 years)
- Investigate the extent to which environmental and co-morbidity factors may influence disease severity and outcome(Up to 10 years)
- Identify and validate biomarkers for disease diagnosis and progression(Up to 10 years)