European respiratory review : an official journal of the European Respiratory Society
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The loss of pulmonary vessels has been shown to be related to the severity of pulmonary hypertension in patients with chronic obstructive pulmonary disease (COPD). The severity of hypoxaemia is also related to pulmonary hypertension and pulmonary vascular resistance in these patients, suggesting that the hypoxic condition probably plays an important role in this form of pulmonary hypertension. However, pulmonary hypertension also develops in patients with mild COPD without hypoxaemia. ⋯ It has recently been demonstrated that pulmonary vascular remodelling, resulting in pulmonary hypertension in COPD patients, can develop independently from parenchymal destruction and loss of lung vessels. We wonder whether the changes in the lung microenvironment due to hypoxia and vessel loss have a causative role in the development of pulmonary hypertension in patients with COPD. Herein we review the pathobiological features of the pulmonary vasculature in COPD patients and suggest that pulmonary hypertension can occur with and without emphysematous lung tissue destruction and with and without loss of lung vessels.
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Haemoptysis is a potentially life-threatening condition with the need for prompt diagnosis. In about 10-20% of all cases the bleeding source remains unexplained with the standard diagnostic approach. The aim of this article is to show the necessity of widening the diagnostic approach to haemoptysis with consideration of pulmonary venous stenosis as a possible cause of even severe haemoptysis and haemoptoe. ⋯ Clinical findings are unspecific and may be misleading. Pulmonary venous stenosis can be detected using several imaging techniques, yet three-dimensional magnetic resonance-angiography and three-dimensional contrast-enhanced computed tomography are the most appropriate. Pulmonary venous stenosis should be considered in patients with haemoptysis.
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Prone positioning has been used for many years in patients with acute lung injury (ALI)/acute respiratory distress syndrome (ARDS), with no clear benefit for patient outcome. Meta-analyses have suggested better survival in patients with an arterial oxygen tension (PaO2 )/inspiratory oxygen fraction (FIO2 ) ratio <100 mmHg. A recent randomised controlled trial was performed in ARDS patients after a 12-24 h stabilisation period and severity criteria (PaO2 /FIO2 <150 mmHg at a positive end-expiratory pressure ≥5 cmH2O). ⋯ The reasons for this dramatic effect are not clear but probably involves a reduction in ventilator-induced lung injury due to prone positioning, for which there is ample evidence in experimental and clinical studies. The aims of this article are to discuss: the rationale of prone positioning in patients with ALI/ARDS; the evidence of its use based on trial analysis; and the limitations of its use as well as the current place of prone positioning in the management of patients with ALI/ARDS. From the currently available data, prone positioning should be used as a first-line therapy in patients with severe ALI/ARDS.
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Pirfenidone is an orally active, small molecule that inhibits synthesis of profibrotic and inflammatory mediators. It was approved for the treatment of adults with mild-to-moderate idiopathic pulmonary fibrosis in the European Union based on the results of two pivotal phase III, double-blind, randomised, placebo-controlled clinical trials (CAPACITY) demonstrating efficacy and safety, and supported by two Japanese clinical trials (SP2 and SP3). Currently, there is increasing interest in experience with pirfenidone in patients relating to the real-world setting. ⋯ New data from the RECAP extension study also provided longer term data for pirfenidone and promising continuation rates with treatment. Pirfenidone is also being evaluated in specialist centre cohorts providing important information on real-world efficacy and safety. Increasing experience with pirfenidone in everyday clinical practice is helping to establish \expert guidance on the management of known adverse events, together with practical recommendations, to ensure adherence to treatment so that the possible longer term benefits of pirfenidone treatment in reducing lung function decline can be maximised.
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This systematic review evaluated evidence for two dry powder formulations, colistimethate sodium and tobramycin, for the treatment of chronic Pseudomonas aeruginosa in cystic fibrosis, as part of the UK national recommendation process for new technologies. Electronic bibliographic databases were searched in May 2012 (MEDLINE, MEDLINE in-Process, EMBASE, Cochrane Library databases, CINAHL, Web of Science, Conference Proceedings Citation Index and BIOSIS Previews). Relevant outcomes included rate and extent of microbial response (e.g. sputum density of P. aeruginosa), lung function (e.g. forced expiratory volume in 1 s (FEV1)), frequency, severity of acute exacerbations and adverse events. ⋯ Whilst short-term results showed that both dry powder drugs were non-inferior to nebulised tobramycin, there was no long-term follow-up and no phase 3 trials compared nebulised and dry powder colistimethate sodium. The use of FEV1 as the primary end-point may not accurately represent changes in lung health. This review illustrates the difficulty in assessing new technologies where the evidence base is poor.