Found 42 datasets matching "fibrosis".
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Treatment of idiopathic pulmonary fibrosis patients has evolved very slowly; the fundamental approach of corticosteroids alone or in combination with other immunosuppressive agents has had little...
Search relevance: 144.94 | Views last month: 0 -
Idiopathic pulmonary fibrosis is a chronic and usually progressive lung disorder of unknown etiology. A growing body of evidence suggests that, in contrast to other interstitial lung diseases, IPF...
Search relevance: 141.02 | Views last month: 0 -
The increase in numbers of adults with cystic fibrosis (CF) has allowed us to identify previously unrecognized chronic complications of CF, as well as appreciate unique presentations of cystic...
Search relevance: 136.16 | Views last month: 0 -
The cystic fibrosis transmembrane conductance regulator (CFTR) has been known for the past 11 years to be a membrane glycoprotein with chloride channel activity. Only recently has the...
Search relevance: 133.34 | Views last month: 1 -
A recent review article suggested that idiopathic pulmonary fibrosis (IPF) is a disease that is associated more with abnormal wound healing than with inflammation. Data derived from transgenic and...
Search relevance: 132.75 | Views last month: 0 -
The identification of the cystic fibrosis (CF) gene opened the way for gene therapy. In the ten years since then, proof of principle in vitro and then in animal models in vivo has been followed by...
Search relevance: 132.25 | Views last month: 2 -
Cystic fibrosis is a genetic disease that is associated with abnormal sweat electrolytes, sino-pulmonary disease, exocrine pancreatic insufficiency, and male infertility. Insights into...
Search relevance: 128.24 | Views last month: 1 -
Background Endoplasmic reticulum retention of misfolded cystic fibrosis transmembrane conductance regulator (CFTR) mutants and their rapid degradation is the major cause of cystic...
Search relevance: 124.39 | Views last month: 0 -
Background Attempts at gene therapy for the pulmonary manifestations of Cystic Fibrosis have relied mainly on airway delivery. However the efficiency of gene transfer and expression in...
Search relevance: 116.85 | Views last month: 7 -
Background Prostanoids are known to participate in the process of fibrogenesis. Because lung fibroblasts produce prostanoids and are believed to play a central role in the pathogenesis...
Search relevance: 99.43 | Views last month: 0 -
Research and surveillance reports have documented a significant increase in coal workers’ pneumoconiosis, including the most severe forms of progressive massive fibrosis and rapidly progressive...
Search relevance: 93.98 | Views last month: 0 -
Systemic sclerosis (SSc) is a multisystem connective tissue disease characterised phenotypically by fibrosis and ischaemic atrophy. Its aetiology is most likely multifactorial. A genetic...
Search relevance: 75.07 | Views last month: 2 -
Background Liver fibrosis is the common sequel of chronic liver diseases. Recent studies have identified hepatic stellate cells as the primary cell type mediating hepatic fibrogenesis....
Search relevance: 74.25 | Views last month: 1 -
Background To learn about the molecular etiology of strabismus, we are studying the genetic basis of 'congenital fibrosis of the extraocular muscles' (CFEOM). These syndromes are...
Search relevance: 73.56 | Views last month: 0 -
The importance of balanced pro-inflammatory and anti-inflammatory mechanisms in diffuse lung disease
The lung responds to a variety of insults in a remarkably consistent fashion but with inconsistent outcomes that vary from complete resolution and return to normal to the destruction of normal...
Search relevance: 66.61 | Views last month: 1 -
The pathogenesis of cryptogenic fibrosing alveolitis (CFA) involves injury, an immune/inflammatory response and fibrosis. The cause of the injury is unknown, but the identification of serum...
Search relevance: 66.38 | Views last month: 0 -
This review provides a detailed overview of the current state of knowledge about the ultrastructure and dynamics of liver sinusoidal endothelial fenestrae. Various aspects of liver sinusoidal...
Search relevance: 64.95 | Views last month: 1 -
Background Excessive loss of bile acids in stool has been reported in patients with cystic fibrosis. Some data suggest that a defect in mucosal bile acid transport may be the mechanism...
Search relevance: 63.45 | Views last month: 0 -
Secretory leukocyte proteinase inhibitor and elafin are two low-molecular-mass elastase inhibitors that are mainly synthesized locally at mucosal sites. It is thought that their physicochemical...
Search relevance: 61.24 | Views last month: 0 -
Aim To demonstrate the effects of combined inhaled nitric oxide and surfactant replacement as treatment for acute respiratory distress syndrome. This treatment has not previously been...
Search relevance: 60.43 | Views last month: 0