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Pulmonary Fibrosis and Telomerase Mutation Study

Completed
Conditions
Pulmonary Fibrosis
Registration Number
NCT01501578
Lead Sponsor
Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
Brief Summary

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.

Detailed Description

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.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
81
Inclusion Criteria
  • Diffuse interstitial lung disease on CT scan
  • Telomerase mutation analysis
Exclusion Criteria
  • Presence of connective tissue disease, or pneumoconiosis or drug induced lung disease

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Description of imaging patternat baseline only

Description of imaging pattern on representative CT scan at diagnosis.

Secondary Outcome Measures
NameTimeMethod
Pulmonary function testsfrom diagnosis to last follow-up, for an average of one year

analyze the respiratory function

Pathology of the lungat baseline only

description of pathological pattern

Trial Locations

Locations (1)

Louis Pradel Hospital (Bâtiment A4)

🇫🇷

Lyon, France

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