Login to your account

Username *
Password *
Remember Me

Blog With Right Sidebar

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis.

Related Articles

Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis.

Dis Model Mech. 2013 Jan;6(1):9-17

Authors: Kropski JA, Lawson WE, Young LR, Blackwell TS

Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal lung disease for which there is no known treatment. Although the traditional paradigm of IPF pathogenesis emphasized chronic inflammation as the primary driver of fibrotic remodeling, more recent insights have challenged this view. Linkage analysis and candidate gene approaches have identified four genes that cause the inherited form of IPF, familial interstitial pneumonia (FIP). These four genes encode two surfactant proteins, surfactant protein C (encoded by SFTPC) and surfactant protein A2 (SFTPA2), and two components of the telomerase complex, telomerase reverse transcriptase (TERT) and the RNA component of telomerase (TERC). In this review, we discuss how investigating these mutations, as well as genetic variants identified in other inherited disorders associated with pulmonary fibrosis, are providing new insights into the pathogenesis of common idiopathic interstitial lung diseases, particularly IPF. Studies in this area have highlighted key roles for epithelial cell injury and dysfunction in the development of lung fibrosis. In addition, genetic approaches have uncovered the importance of several processes - including endoplasmic reticulum stress and the unfolded protein response, DNA-damage and -repair pathways, and cellular senescence - that might provide new therapeutic targets in fibrotic lung diseases.

PMID: 23268535 [PubMed - in process]

Lung sonography.

Related Articles

Lung sonography.

J Ultrasound Med. 2013 Jan;32(1):165-71

Authors: Volpicelli G

Abstract
Lung sonography represents an emerging and useful technique in the management of some pulmonary diseases. For many years, sonography of the thorax was limited to the study of pleural effusion and thoracic superficial masses because alveolar air and bones of the thoracic cage limit the propagation of the ultrasound beam. Only recently has it been highlighted that lung sonography is highly sensitive to variations of the pulmonary content and balance between air and fluids, like a real lung densitometer. Dynamic and static analysis of a combination of sonographic artifacts and real images makes accurate diagnosis of many lung disorders possible, particularly when lung sonography is applied in the emergency and critical care settings. Sonography is useful in the diagnosis of lung diseases in which the alveolar air content is impaired and interstitial and alveolar fluids are increased and also when air or fluids are collected in the pleural space. This article analyzes the basic principles of lung ultrasonography and all of the supposed limitations to its diagnostic usefulness. Moreover, the article reviews the three main fields of lung sonography application: interstitial, alveolar, and pleural syndromes.

PMID: 23269722 [PubMed - in process]

[Respiratory bronchiolitis and respiratory bronchiolitis-associated interstitial lung disease].

Related Articles

[Respiratory bronchiolitis and respiratory bronchiolitis-associated interstitial lung disease].

Rev Med Suisse. 2012 Nov 21;8(363):2228-32

Authors: Ogna A, Noirez L, Lazor R, Nicod LP

Abstract
The increasing use of chest CT imaging in medical practice rises the likelihood of the general practitioner to be confronted with cases of interstitial lung disease. Respiratory bronchiolitis (RB) and respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) are two smoking-related lung damages that may have important implications for the patient's management. The authors present in this paper a review of current knowledge of the epidemiology, clinical features, prognosis, and treatment options of RB and RB-ILD.

PMID: 23240299 [PubMed - indexed for MEDLINE]

Human metapneumovirus infections are associated with severe morbidity in hospitalized children of all ages.

Related Articles

Human metapneumovirus infections are associated with severe morbidity in hospitalized children of all ages.

Epidemiol Infect. 2013 Jan 7;:1-11

Authors: Hahn A, Wang W, Jaggi P, Dvorchik I, Ramilo O, Koranyi K, Mejias A

Abstract
SUMMARY The impact of human metapneumovirus (HMPV) in children aged >5 years and the risk factors associated with disease severity for all ages have not been well characterized. A retrospective cohort study of 238 children aged 0-15 years hospitalized over a 3-year period was performed. Medical records were reviewed for demographic information, clinical parameters and outcomes. Multivariable analyses were performed to identify independent factors associated with worse disease severity assessed by length of hospital stay (LOS), need for ICU care, respiratory support, and a disease severity score. Pulmonary diseases were associated with all outcomes of care, while congenital heart disease (CHD) and neuromuscular disorders were associated with longer LOS, and CHD and trisomy 21 were associated with worse severity scores independent of other covariables. Fever, retractions, use of steroids and albuterol were also associated with enhanced disease severity. Understanding the determinants of HMPV disease in children may help design targeted preventive strategies.

PMID: 23290557 [PubMed - as supplied by publisher]

Systems biology of lung development and regeneration: current knowledge and recommendations for future research.

Related Articles

Systems biology of lung development and regeneration: current knowledge and recommendations for future research.

Wiley Interdiscip Rev Syst Biol Med. 2013 Jan 4;

Authors: Hagood JS, Ambalavanan N

Abstract
The lung begins as a simple outpouching of the foregut and develops by stages into a highly complex organ, the proper function of which is essential to life for terrestrial mammals. Interruption of normal lung development can result in death or chronic disease. Conversely, repair after lung injury, as well as many acquired diseases, involves recapitulation, often aberrant, of developmental pathways. The principal paradigms in lung development are branching morphogenesis and alveolar septation, but others, such as vasculogenesis, are critical. These are partially understood at the level of cellular differentiation and molecular signaling, but a true systems biology analysis of lung development and lung repair/regeneration, including bioinformatics analysis and integration of data from unbiased and complementary '-omics' level studies, is still lacking. The past decade has seen increasing numbers of genomic, proteomic, metabolomics, and epigenomic studies of lung development and lung remodeling. In many cases, these studies have confirmed the importance of pathways uncovered painstakingly through single-molecule approaches, but they have also uncovered novel and unexpected pathways and new paradigms such as noncoding RNA. Future studies will need to combine data from multiple repositories and apply novel mathematical and computational models in order to establish a systems-level understanding of this remarkable organ. WIREs Syst Biol Med 2013. doi: 10.1002/wsbm.1205 For further resources related to this article, please visit the WIREs website.

PMID: 23293056 [PubMed - as supplied by publisher]

Search