Therapeutic advances in respiratory disease
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Ther Adv Respir Dis · Dec 2015
ReviewAn update on the efficacy of endobronchial valve therapy in the management of hyperinflation in patients with chronic obstructive pulmonary disease.
Lung volume reduction surgery has been shown to be effective in patients with heterogeneous emphysema, but is also associated with a relatively high perioperative morbidity and mortality. Accordingly, several novel and potentially less invasive methods for bronchoscopic lung volume reduction have been developed. Endobronchial valve (EBV) therapy is one such therapeutic approach in patients with advanced emphysema. ⋯ The choice of EBV therapy largely depends on the distribution of emphysema and the presence or absence of interlobar collateral ventilation. Adequate patient selection and technical success of valve implantation with the intention of lobar exclusion are predictive factors for positive outcomes. This review attempts to highlight the milestones in the development of bronchoscopic lung volume reduction with one-way valve implantation over the past few years.
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Ther Adv Respir Dis · Dec 2015
ReviewThe role of the small airways in the pathophysiology of asthma and chronic obstructive pulmonary disease.
Chronic respiratory diseases, such as asthma and chronic obstructive pulmonary disease (COPD), represent a major social and economic burden for worldwide health systems. During recent years, increasing attention has been directed to the role of small airways in respiratory diseases, and their exact contribution to the pathophysiology of asthma and COPD continues to be clarified. Indeed, it has been suggested that small airways play a distinct role in specific disease phenotypes. ⋯ Most studies have been performed in small population samples, and different techniques to characterize aspects of small airways function have been employed in order to assess inflammation and remodelling. Most methods of assessing small airways dysfunction have been largely confined to research purposes, but some data are encouraging, supporting the utilization of certain techniques into daily clinical practice, particularly for early-stage diseases, when subjects are often asymptomatic and routine pulmonary function tests may be within normal ranges. In this context further clinical trials and real-life feedback on large populations are desirable.
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Ther Adv Respir Dis · Dec 2015
ReviewLumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.
Cystic fibrosis (CF) is a genetic disorder that causes multiorgan morbidity and premature death, most commonly from pulmonary dysfunction. Mutations in the CF transmembrane conductance regulator (CFTR) gene, of which almost 2000 have been described, result in a dysfunctional CFTR protein. This protein is an adenosine triphosphate binding anion channel, present primarily at the surface of epithelial cells. ⋯ F508del is the most common CFTR mutation, present in homozygosity in approximately 50% of patients with CF. The F508del mutation results in multiple CFTR channel defects that require both correction (stabilization of misfolded CFTR and trafficking to the epithelial cell membrane) and potentiation. This article reviews the in vitro and clinical trial data for the potential use of the potentiator, ivacaftor, and the corrector, lumacaftor, in patients with CF.
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Ther Adv Respir Dis · Dec 2015
ReviewThe role of inhaled prostacyclin in treating acute respiratory distress syndrome.
Acute respiratory distress syndrome (ARDS) is a syndrome of acute lung injury that is characterized by noncardiogenic pulmonary edema and severe hypoxemia second to a pathogenic impairment of gas exchange. Despite significant advances in the area, mortality remains high among ARDS patients. High mortality and a limited spectrum of therapeutic options have left clinicians searching for alternatives, spiking interest in selective pulmonary vasodilators (SPVs). ⋯ The most effective and safest dose for yielding a clinically significant increase in PaO2 and reduction in pulmonary artery pressure (PAP) appears to be 20-30 ng/kg/min in adults and 30 ng/kg/min in pediatric patients. iEPO appears to have a ceiling effect above these doses in which no additional benefit may be derived. iNO and iEPO have shown similar efficacy profiles; however, they differ with respect to cost and ease of therapeutic administration. The most beneficial effects of iEPO have been seen in adult patients with secondary ARDS as compared with primary ARDS, most likely due to the difference in etiology of the two disease states, and in patients suffering from baseline right ventricular heart failure. Although iEPO has demonstrated improvements in hemodynamic parameters and oxygenation in ARDS patients, due to the limited number of randomized clinical trials and the lack of studies investigating mortality, the use of iEPO cannot be recommended as standard of care in ARDS. iEPO should be reserved for those refractory to traditional therapies.
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Ther Adv Respir Dis · Oct 2015
ReviewTreatment of emphysema using bronchoscopic lung volume reduction coil technology: an update on efficacy and safety.
In the last decade several promising bronchoscopic lung volume reduction (BLVR) treatments were developed and investigated. One of these treatments is BLVR treatment with coils. ⋯ Seven years ago, the very first patients were treated with BLVR coil treatment and currently large randomized, controlled trials are underway. The aim of this article is to review the available literature and provide an update on the current knowledge on the efficacy and safety of BLVR treatment with coils.