Phenotype of Pulmonary Fibrosis Associated With a Mutation of Telomerase
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 81
- 试验地点
- 1
- 主要终点
- Description of imaging pattern
研究概览
简要总结
This study is an observational and retrospective study of patients with pulmonary fibrosis associated or not with telomerase mutation.
The purpose of this study is to describe in detail the cases with telomerase mutation in terms of features on CT scan, respiratory function and evolution, in comparison to control subjects with idiopathic pulmonary fibrosis and no telomerase mutation identified or family history.
详细描述
Two "control" subjects will be enrolled for one subject with telomerase mutation.
The data are all the results of investigations conducted for the diagnosis of idiopathic pulmonary fibrosis and during routine follow up of patients.
The CT scans will reviewed centrally to homogenize the description.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Diffuse interstitial lung disease on CT scan
- •Telomerase mutation analysis
排除标准
- •Presence of connective tissue disease, or pneumoconiosis or drug induced lung disease
结局指标
主要结局
Description of imaging pattern
时间窗: at baseline only
Description of imaging pattern on representative CT scan at diagnosis.
次要结局
- Pulmonary function tests(from diagnosis to last follow-up, for an average of one year)
- Pathology of the lung(at baseline only)
研究者
Vincent COTTIN
Principal Investigator
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires