Expert review of respiratory medicine
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Expert Rev Respir Med · Apr 2011
ReviewInhaled iloprost for therapy in pulmonary arterial hypertension.
Iloprost (Ventavis, Bayer Schering Pharma, Germany) is a synthetic prostacyclin that is used in its inhalative form for the therapy of pulmonary arterial hypertension. Long-term therapy can increase exercise capacity and quality of life. The use of modern nebulizers especially designed for the administration of iloprost guarantees the pulmonary deposition of the required doses and systematically minimizes side effects. Regarding existing data, inhalative iloprost acts in effective and safe combination with other classes of medication; indeed, such combination therapy is frequently necessary in pulmonary arterial hypertension.
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Expert Rev Respir Med · Apr 2011
ReviewPulmonary hypertension in children with sickle cell disease.
Pulmonary hypertension and cor pulmonale have long been known to be complications of sickle cell disease, thought mostly to affect the adult population. Recently, pediatric studies in sickle cell patients have uncovered a similar prevalence of elevated tricuspid regurgitation jet velocities, a finding consistent with increased right-sided pressures, when compared with adult counterparts. ⋯ We review the recent literature in an attempt to discuss the prevalence, etiology and implications of an elevated tricuspid regurgitation jet velocity in pediatric sickle cell patients. We also suggest screening, diagnostics, treatment and follow-up plans that may improve the disease burden of pulmonary hypertension in both pediatric and adult sickle cell populations.
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Pulmonary arterial hypertension (PAH) is a progressive disease that results in elevation of the pulmonary artery pressure and ultimately development of right ventricular failure. Baseline and periodic invasive hemodynamics via right heart catheterization are generally obtained in order to characterize and follow the extent of hemodynamic derangement, response to therapy, and as an end point in clinical trials of novel therapy. Therapy for PAH is steadily evolving, with considerable uncertainty regarding optimal dosing and comparative efficacy of the available therapeutic agents. ⋯ Such techniques provide novel insights into cardiovascular pathophysiology during activity and may prove to be useful in allowing early detection of hemodynamic changes between clinic visits. Hemodynamics obtained from implantable monitors have been used during PAH medical therapy in pilot studies and could be used as an end point in clinical trials of PAH therapy. Future efforts should focus on proof of clinical utility of implantable monitoring and refinement of cardiac output estimation by pressure waveform analysis.
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Expert Rev Respir Med · Apr 2011
ReviewPulmonary hypertension, nitric oxide and nitric oxide-releasing compounds.
Nitric oxide (NO) is an important molecule in the mammalian cardiovascular system and the elucidation of its biological role has led to the awarding of a Nobel prize in medicine. There have been many significant discoveries in NO biology over the past two decades and translation of these developments from bench to bedside has allowed an explosion of therapies for pulmonary hypertension. This article provides an overview of the NO pathway in the pulmonary circulation in maintenance of normal vascular tone as well as its dysregulation in pulmonary hypertension, and a discussion of therapies based on inhaled NO or manipulation of its downstream signaling pathways. Several therapies such as phosphodiesterase inhibitors are already in use, and various experimental therapies are also discussed.
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At present, therapeutic interventions to treat acute lung injury (ALI) or acute respiratory distress syndrome (ARDS) remain largely limited to lung-protective strategies, as no real molecular-pathophysiologic-driven therapeutic intervention has yet become available. This is in part the result of the heterogeneous nature of the etiological processes that contribute to the state of ALI/ARDS. This article sets out to understand the development of ALI resulting from indirect pulmonary insults, such as extrapulmonary sepsis and trauma, shock, burn injury or mass transfusion, as opposed to direct pulmonary challenges, such as pneumonia, aspiration or lung contusion. Here, we consider not only the experimental and clinical data concerning the roles of various immune (neutrophil, macrophage, lymphocyte and dendritic) as well as nonimmune (epithelial and endothelial) cells in orchestrating the development of ALI resulting from indirect pulmonary stimuli, but also how these cell populations might be targeted therapeutically.