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Substantial challenges remain in our understanding of fibrotic lung diseases. Nowhere is this more true than in the elucidation and verification of the pathogenetic basis upon which they develop. Scientific progress, most recently in the field of experimental therapy, has relied closely on interpreting data derived from animal modeling. Such models are used(More)
One distinctive outcome of interstitial lung diseases in childhood is the abnormal accumulation of pulmonary extracellular matrix. The clinical consequence of such excessive connective tissue accumulation is known as pulmonary fibrosis. While numerous aspects of its pathogenesis have become familiar, many key events involved in its inception and progression(More)
Of the myriad proteolytic enzymes implicated in the development of lung disease, neutrophil elastase has undoubtedly some of the most versatile effects. Although its key physiologic role is in innate host defense, it can also participate in tissue remodeling and possesses secretagogue actions that are now recognized as important to local inflammatory(More)
Pulmonary aspergillosis encompasses a heterogeneous group of mycoses that result from either colonisation or pathogenic damage of lung tissue by Aspergillus fungi. These clinical entities range from relatively benign saprophytic hypersensitivity associated with fungal inhabitation to life threatening invasive disease. The diagnosis of pulmonary disorders(More)
We present a rare case of chronic Pneumocystis jiroveci infection presenting as multiple persistent granulomatous pulmonary nodules over a 12 month period in a patient with follicular lymphoma undergoing treatment with Rituximab, Cyclophosphamide, Vincristine, and Prednisolone chemotherapy. Remarkably during this period the patient remained asymptomatic.(More)
BACKGROUND In ventricular dilatation or hypertrophy, an elevated end-diastolic pressure is often assumed to be secondary to increased myocardial stiffness, but stiffness is rarely measured in vivo because of difficulty. We measured in vitro passive stiffness of volume- or pressure-overloaded myocardium mainly from congenital heart disease. METHODS AND(More)
A widening spectrum of increasingly advanced bronchoscopic techniques is available for the diagnosis and treatment of various bronchopulmonary diseases. The evolution of computed tomography (CT)-multidetector CT in particular-has paralleled these advances. The resulting development of two-dimensional and three-dimensional (3D) postprocessing techniques has(More)
Neutrophil elastase is a serine protease stored in the azurophilic granules of leukocytes. It has been implicated in the pathology of several lung diseases and is generally presumed to contribute to the tissue destruction and extracellular matrix damage associated with these conditions. To delineate the role of neutrophil elastase in pulmonary inflammation(More)
Interstitial lung disease (ILD) is a very important complication of the idiopathic inflammatory myositides (IIM), with a prevalence of approximately 40 %. Characteristic HRCT changes, most commonly NSIP, together with a restrictive ventilatory defect and an associated decline in DLco support the presence of ILD. The strongest risk factors are the presence(More)