منابع مشابه
Genetic Causes of Interstitial Lung Disease in Children
Interstitial lung diseases (ILDs) occur through out childhood and adult life. In neonates and infants, defects in lung development and growth and defects in surfactant production and function are common features with the environmental factors being less important. For older children, genetic background combined with environmental exposure is central to the pathophysiology of ILD. In adults, env...
متن کاملGenetic Susceptibility to Interstitial Lung Disease Associated with Systemic Sclerosis
Systemic sclerosis (SSc) is a connective tissue disease that is characterized by tissue fibrosis, microvasculopathy, and autoimmunity. Interstitial lung disease (ILD) is a common complication of SSc and is one of the frequent causes of mortality in SSc. Although the exact etiology of SSc remains unknown, clinical and experimental investigations have suggested that genetic and environmental fact...
متن کاملInterstitial lung disease in children – genetic background and associated phenotypes
Interstitial lung disease in children represents a group of rare chronic respiratory disorders. There is growing evidence that mutations in the surfactant protein C gene play a role in the pathogenesis of certain forms of pediatric interstitial lung disease. Recently, mutations in the ABCA3 transporter were found as an underlying cause of fatal respiratory failure in neonates without surfactant...
متن کاملInherited interstitial lung disease.
This article focuses on recent advances in the identification of genes and genetic polymorphisms that have been implicated in the development of human interstitial lung diseases. It focuses on the inherited mendelian diseases in which pulmonary fibrosis is part of the clinical phenotype and the genetics of familial idiopathic pulmonary fibrosis and other rare inherited interstitial lung disease...
متن کامل[Interstitial lung disease].
This concise article summarizes recent advances in the field of interstitial lung disease (ILD) with particular focus on clinically relevant findings. As a novel treatment option for idiopathic pulmonary fibrosis (IPF), pirfenidone has been granted marketing authorization in the European Union for the treatment of mild to moderate IPF. In contrast, the FDA refused to approve pirfenidone for the...
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ژورنال
عنوان ژورنال: Clinics in Chest Medicine
سال: 2012
ISSN: 0272-5231
DOI: 10.1016/j.ccm.2011.11.001