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A Linkage and Association Study in Pulmonary Fibrosis

2014-08-27 03:15:10 | BioPortfolio

Summary

The purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis. Finding the genes that cause pulmonary fibrosis is the first step at developing better methods for early diagnosis and improved treatment for pulmonary fibrosis. The overall hypothesis is that inherited genetic factors predispose individuals to develop pulmonary fibrosis.

Description

Familial Pulmonary Fibrosis (FPF) is a sub-category of the idiopathic interstitial pneumonias (IIPs). IIPs are progressive lung conditions, with limited treatment options and unknown etiology. Though the IIPs have been associated with both genetic risk factors and environmental exposures, the molecular mechanism underlying disease progression remain poorly understood. This investigation seeks to identify a group of genetic loci that play a role in the development of familial interstitial pneumonia (FIP) or FPF, where 2 or more cases of IIP are seen within a family.

Study Design

Observational Model: Family-Based, Time Perspective: Cross-Sectional

Conditions

Idiopathic Pulmonary Fibrosis

Location

National Jewish Health
Denver
Colorado
United States
80206

Status

Recruiting

Source

National Jewish Health

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:15:10-0400

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Pirfenidone for the Treatment of Patients With Pulmonary Fibrosis/Idiopathic Pulmonary Fibrosis (PF/IPF)

To assess the long-term safety and efficacy of oral pirfenidone in doses of up to 40 mg/kg/d in a limited number of patients with pulmonary fibrosis/idiopathic pulmonary fibrosis (PF/IPF)

OASIS-IPF (Idiopathic Pulmonary Fibrosis) Study

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Detection of Integrin avb6 in Idiopathic Pulmonary Fibrosis Using PET/CT

The investigators wish to evaluate the feasibility of [18F]FP-R01-MG-F2 PET/CT scanning in patients with Idiopathic Pulmonary Fibrosis.

PubMed Articles [4251 Associated PubMed Articles listed on BioPortfolio]

Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema.

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To determine whether computer-derived computed tomography measures, specifically measures of pulmonary vessel-related structures, can better predict functional decline and survival in idiopathic pulmo...

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Cardiovascular comorbidities are frequent in patients with idiopathic pulmonary fibrosis (IPF), and many patients with IPF receive treatment with statins to reduce cardiovascular risk.

Abnormal Bolus Reflux Is Associated With Poor Pulmonary Outcome in Patients With Idiopathic Pulmonary Fibrosis.

Gastroesophageal reflux (GER) is postulated to play a role in idiopathic pulmonary fibrosis (IPF). However, the value of objective GER measures in predicting IPF disease progression is unclear. We aim...

Medical and Biotech [MESH] Definitions

A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.

A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.

A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis.

A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.

Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.

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