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Mechanisms of Familial Pulmonary Fibrosis

2018-02-22 19:05:23 | BioPortfolio

Published on BioPortfolio: 2018-02-22T19:05:23-0500

Clinical Trials [1970 Associated Clinical Trials listed on BioPortfolio]

Linkage Study in Familial Pulmonary Fibrosis

To map the gene (or genes) for familial pulmonary fibrosis.

A Linkage and Association Study in Pulmonary Fibrosis

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 in...

Procurement and Analysis of Specimens From Individuals With Pulmonary Fibrosis

The etiology of pulmonary fibrosis is unknown. Analysis of blood, genomic DNA, and specimens procured by bronchoscopy, lung biopsy, lung transplantation, or post-mortem examination from i...

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)

Effects of Pulmonary Rehabilitation on Functional and Health Status Measures in Pulmonary Fibrosis

The inclusion of Pulmonary Rehabilitation (PR) as part of the management of pulmonary fibrosis although being highly recommended and recommended in guidelines still sees studies exploring ...

PubMed Articles [4242 Associated PubMed Articles listed on BioPortfolio]

A case report of heterozygous TINF2 gene mutation associated with pulmonary fibrosis in a patient with dyskeratosis congenita.

Dyskeratosis congenita (DC) is a rare inherited disease characterized by the classical mucocutaneous triad. Pulmonary fibrosis, bone marrow failure, and solid tumors are the main causes of mortality i...

Dihydroartemisinin alleviates oxidative stress in bleomycin-induced pulmonary fibrosis.

Dihydroartemisinin has been shown to inhibit the development of pulmonary fibrosis in rats, but its mechanism has yet to be elucidated. This study aimed to determine the mechanisms of dihydroartemisin...

A Novel Variant of Desmoplakin Is Potentially Associated with Silicosis Risk.

Two genome-wide association studies and one sequencing study have coincidently reported significant associations of single nucleotide polymorphisms (SNPs) in the desmoplakin (DSP) gene with the risk o...

Astilbin ameliorates pulmonary fibrosis via blockade of Hedgehog signaling pathway.

The nature of pulmonary fibrosis involves inadequate repair of the epithelial cell barrier accompanied by impaired regulation of the fibroblast. Moreover, pulmonary fibrosis currently lacks an effecti...

Inhibition of mTOR ameliorated bleomycin-induced pulmonary fibrosis by regulating epithelial-mesenchymal transition.

Epithelial-mesenchymal transition (EMT) plays a pivotal role in idiopathic pulmonary fibrosis (IPF). In bleomycin-induced pulmonary fibrosis mice, we observed that inhibition of mTOR (mammalia target ...

Medical and Biotech [MESH] Definitions

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

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 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.

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 diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.

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