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Genetics of Pulmonary Fibrosis
Seminars in Respiratory and Critical Care Medicine, 2002Pulmonary fibrosis is a devastating disease that occurs in association with environmental exposures, several inherited diseases, collagen vascular disease, as well as in an idiopathic form. Epithelial cell damage, excessive fibroblast proliferation, upregulation of extracellular matrix production, and attenuated matrix degradation are shared ...
Ganesh Raghu, Mark M. Wurfel
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Pulmonary fibrosis in children
The Indian Journal of Pediatrics, 1981The functional integrity of the lung is largely dependant on the connective tissue and capillary structure of the gas exchanging parts. Pulmonary fibrosis as a result of several interstinal diseases have major physiological implications. We report three cases of pulmonary fibrosis in children.
Anbu Chetty, Ved Prakash Choudhry
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Pathobiology of pulmonary fibrosis
American Journal of Physiology-Lung Cellular and Molecular Physiology, 1990Pulmonary fibrosis is characterized by an increase in lung matrix and alterations in the numbers and spatial relationships of lung parenchymal cells. The increase in matrix results from a proliferation and “activation” of fibroblasts (FB) with increased production and deposition of matrix macromolecules at sites of lung injury.
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Diseases of the Chest, 1951
1)The term Familial Pulmonary Fibrosis is felt to be a definite entity. 2)This disease is a familial response to chronic pulmonary insult in the nature of bilateral pulmonary fibrosis. 3)The disease, once it manifests itself clinically, is usually progressive to death. 4)The age incidence is from 30 to 55 years.
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1)The term Familial Pulmonary Fibrosis is felt to be a definite entity. 2)This disease is a familial response to chronic pulmonary insult in the nature of bilateral pulmonary fibrosis. 3)The disease, once it manifests itself clinically, is usually progressive to death. 4)The age incidence is from 30 to 55 years.
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Autophagy and Pulmonary Fibrosis
2020Pulmonary fibrosis is a progressive chronic inflammatory disease with a poor clinical outcome. Although pirfenidone and nintedanib have been approved by FDA to treat idiopathic pulmonary fibrosis (IPF), these drugs can only slow the progression of IPF. Autophagy plays an important role in the pathogenesis of pulmonary fibrosis.
Ke Li, Xiaoxi Lv, Zhuo-Wei Hu
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Revue des Maladies Respiratoires, 2015
The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the last 10 years, mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis.
Caroline Kannengiesser +5 more
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The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the last 10 years, mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis.
Caroline Kannengiesser +5 more
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The Myofibroblast in Pulmonary Fibrosis*
Chest, 2002The pathogenesis of pulmonary fibrosis remains incompletely understood. Studies of associated inflammation have led to the discovery of a number of cytokines and chemokines that are found to be important either directly or indirectly for the fibrotic process.
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Clinics in Chest Medicine, 1982
SUMMARY Idiopathic pulmonary fibrosis is a precise syndrome defined by clinical, roentgenographic, physiologic, and histopathologic features. Clinically, most patients have a nonproductive cough and dyspnea on exertion. The most common physical finding is the presence of crackles over the lower portions of both lungs; also, there may be clubbing ...
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SUMMARY Idiopathic pulmonary fibrosis is a precise syndrome defined by clinical, roentgenographic, physiologic, and histopathologic features. Clinically, most patients have a nonproductive cough and dyspnea on exertion. The most common physical finding is the presence of crackles over the lower portions of both lungs; also, there may be clubbing ...
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Human Pathology, 1970
Abstract The histopathologic changes in diffuse pulmonary fibrosis are discussed and current nomenclature reviewed. The importance of alveolitis due to hypersensitivity to inhaled organic proteins (allergic alveolitis) has been recognized only recently. The features that distinguish allergic alveolitis from other types of diffuse interstitial fibrosis
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Abstract The histopathologic changes in diffuse pulmonary fibrosis are discussed and current nomenclature reviewed. The importance of alveolitis due to hypersensitivity to inhaled organic proteins (allergic alveolitis) has been recognized only recently. The features that distinguish allergic alveolitis from other types of diffuse interstitial fibrosis
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