Respiratory medicine
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Respiratory medicine · Jul 2020
ReviewFocus on the pulmonary involvement and genetic patterns in Birt-Hogg-Dubè syndrome: Literature review.
Brit-Hogg-Dubé syndrome (BHD) is a rare disorder that is estimated to affects about 600 families in the World. The disease-causing mutations is on FLCN gene which codes for folliculin. This protein has a role in different organs as skin, kidney and lung, thanks to the interaction with type I and II cadherins, RhoA activity and the regulation of AMPK, mTORC1 pathways and cell adhesion. The aim of our study is to focus on the manifestation of the syndrome, especially the pulmonary involvement, then on genetical analysis and on the available treatments. ⋯ BHD syndrome is rare and usually the first manifestations appear in early age. In patients with these clinical and radiological characteristics we suggest taking a careful medical history, though the diagnosis of BHD syndrome should be confirmed with the analysis of FLCN gene.
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Respiratory medicine · Jul 2020
ReviewSevere respiratory SARS-CoV2 infection: Does ACE2 receptor matter?
SARS-CoV-2 is a novel virus of the Coronaviridiae family that represents a major global health issue. Mechanisms implicated in virus/host cells interaction are central for cell infection and replication that in turn lead to disease onset and local damage. ⋯ Molecular mechanisms that promote interaction between SARS-CoV-2 virus and host with particular focus on virus cell entry receptor ACE2 are described. We further explore the impact of underlying medical conditions and therapies including renin-angiotensin inhibitors on modulating ACE 2, which is the major SARS-CoV-2 cell entry receptor.
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Respiratory medicine · Jun 2020
Letter Case ReportsCoronavirus Disease 2019-related dyspnea cases difficult to interpret using chest computed tomography.
Patients with Coronavirus Disease 2019 (COVID-19) often have clinical characteristics, such as chest tightness and dyspnea. Continuous, unresolved dyspnea often indicates the progression of lung lesions. The mechanism that underlies the chest distress and dyspnea in patients with COVID-19 is still unclear. ⋯ We found that extensive inflammation of the bilateral and respiratory bronchioles in patients with COVID-19 due to excessive activation of proinflammatory cytokines and chemotactic aggregation of T-lymphocytes at the site of inflammation are possible mechanisms underlying chest distress and dyspnea in patients with COVID-19. Short-time and lose-dose use of corticosteroid may be helpful to treat chest tightness and dyspnea in mild COVID-19 patients. Through this study, we aimed to improve our understanding of the pathogenesis of COVID-19.
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Respiratory medicine · Mar 2020
A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex.
The mechanistic target of rapamycin inhibitors (mTORi) sirolimus and everolimus stabilize lung function in patients with pulmonary lymphangioleiomyomatosis (LAM) but do not induce remission. Pre-clinical studies suggest that simvastatin in combination with sirolimus induces LAM cell death. The objective of this study was to assess the safety of simvastatin with either sirolimus or everolimus in LAM patients. ⋯ The combination of simvastatin with mTORi in LAM patients is safe and well-tolerated from an adverse events perspective. The addition of simvastatin, however, was associated with decline in FEV1 and the efficacy of this combination should be explored in larger trials.
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Respiratory medicine · Mar 2020
Case ReportsBreaking hearts and taking names: A case of sarcoidosis related effusive-constrictive pericarditis.
Pericardial involvement of sarcoidosis is a rare cause for acute heart failure, and usually occurs as a result of the development of a pericardial effusion leading to cardiac tamponade. Even rarer still, is the manifestation of constrictive pericarditis. We report a case of sarcoidosis with lung, pleural, and pericardial involvement with effusive-constrictive pericarditis leading to cardiac tamponade. ⋯ We report a case demonstrating unique manifestations of sarcoidosis; effusive-constrictive pericarditis presenting with acute congestive heart failure.