Respiration 

About: Respiration is an academic journal published by Karger Publishers. The journal publishes majorly in the area(s): COPD & Respiratory disease. It has an ISSN identifier of 0025-7931. Over the lifetime, 5967 publications have been published receiving 113512 citations.

Fit Yourself and Take Your Lungs to Heart

Abstract: nificantly reduces odds of hospital admissions and deaths following acute exacerbations, as well as consistently improves the quality of life and exercise tolerance of COPD patients [6] . However, no prospective studies on the role of physical rehabilitation in influencing the number and severity of future exacerbations have been performed yet. The paper by Ramponi et al. [7] , published in this issue of Respiration , aims to assess whether a 9-week pulmonary rehabilitation program may affect cardiovascular response to exercise in COPD patients. In an observational prospective trial, according to the ATS/ERS recommendations, 27 patients with COPD were referred to a rehabilitation program consisting of 3-hour sessions, three times a week, with a minimum of 21 sessions required. Data obtained show a significant improvement in maximal exercise tolerance, such as peak oxygen uptake, and in some cardiovascular parameters following rehabilitation. Leg fatigue was also significantly reduced, supporting the reported positive effect of physical exercise on muscle function. The novel aspect of the study is to compare cardiopulmonary exercise variables at ‘submaximal’ exercise levels, isometabolic and isoventilatory levels (‘isolevels’) before and after rehabilitation. Of note, the O 2 pulse (peak oxygen uptake/heart rate) and tidal volume were significantly higher after rehabilitation. Furthermore, tidal volume changes correlated significantly with changes in O 2 pulse. The authors concluded that the most likely explanation for their observations was an improvement in cardiovascular function due to a reduction It has been estimated that physical inactivity is worldwide responsible for 6–10% of the major non-communicable diseases. Furthermore, sedentary lifestyle causes 9% of premature mortality [1] . Moreover, recent studies have consistently shown that messages emphasizing the benefits of being active are more effective at changing physical activity behaviour than those stressing the consequences of inactivity [2] . Chronic obstructive pulmonary disease (COPD) is a complex disease mainly characterized by structural abnormalities of the airways and lungs, but it is very often associated with concomitant comorbidities. The presence of comorbidities strongly influences not only the severity of symptoms, but also the risk of hospitalization and death [3] . The relationship between COPD and cardiovascular disease is particularly notable and of clinical relevance, as cardiovascular disease represents the most common comorbidity and the leading cause of hospitalization in patients with mild-to-moderate COPD [4] . Exercise-based pulmonary rehabilitation is a well-established intervention for patients with COPD. Physical training, by improving skeletal muscle function, positively influences exercise tolerance and symptoms; this is mainly due to a reduction in lung dynamic hyperinflation but also to a desensitization to central dyspnea [5] . There is now emerging evidence to support the efficacy of exercise-based pulmonary rehabilitation also in the management of COPD exacerbations. An up-to-date Cochrane meta-analysis shows that pulmonary rehabilitation sigPublished online: May 30, 2013

Genotype and phenotype in cystic fibrosis.

Abstract: Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene which encodes a protein expressed in the apical membrane of exocrine epithelial cells. CFTR functions principally as a cAMP-induced chloride channel and appears capable of regulating other ion channels. Besides the most common mutation, ΔF508, accounting for about 70% of CF chromosomes worldwide, more than 850 mutant alleles have been reported to the CF Genetic Analysis Consortium. These mutations affect CFTR through a variety of molecular mechanisms which can produce little or no functional CFTR at the apical membrane. This genotypic variation provides a rationale for phenotypic effects of the specific mutations. The extent to which various CFTR alleles contribute to clinical variation in CF is evaluated by genotype-phenotype studies. These demonstrated that the degree of correlation between CFTR genotype and CF phenotype varies between its clinical components and is highest for the pancreatic status and lowest for pulmonary disease. The poor correlation between CFTR genotype and severity of lung disease strongly suggests an influence of environmental and secondary genetic factors (CF modifiers). Several candidate genes related to innate and adaptive immune response have been implicated as pulmonary CF modifiers. In addition, the presence of a genetic CF modifier for meconium ileus has been demonstrated on human chromosome 19q13.2. The phenotypic spectrum associated with mutations in the CFTR gene extends beyond the classically defined CF. Besides patients with atypical CF, there are large numbers of so-called monosymptomatic diseases such as various forms of obstructive azoospermia, idiopathic pancreatitis or disseminated bronchiectasis associated with CFTR mutations uncharacteristic for CF. The composition, frequency and type of CFTR mutations/variants parallel the spectrum of CFTR-associated phenotypes, from classic CF to mild monosymptomatic presentations. Expansion of the spectrum of disease associated with the CFTR mutant genes creates a need for revision of the diagnostic criteria for CF and a dilemma for setting nosologic boundaries between CF and other diseases with CFTR etiology.

Acute exacerbations of idiopathic pulmonary fibrosis.

Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and ultimately fatal disease, with a highly variable course in individual patients. Episodes of rapid deterioration are not uncommon, often following a period of stability. In cases of uncertain etiology, with typical clinical and high-resolution computed tomography (HRCT) features, the term ‘acute exacerbation of IPF' (AE-IPF) has been coined to describe a combination of diffuse alveolar damage and preexisting usual interstitial pneumonia. In 2007, a consensus definition and diagnostic criteria were proposed. Although the presence of overt infection is currently an exclusion criterion, it appears likely that occult infection, reflux and thoracic surgical procedures are all trigger factors for AE-IPF. The development of new, usually bilateral infiltrates (ground-glass attenuation with variable admixed consolidation) is a defining HRCT feature. The outcome is poor with a short-term mortality in excess of 50% despite therapy. A number of pathophysiologic pathways are activated, with immunologic dysregulation, epithelial damage and circulating fibrocytes all believed to play a pathogenetic role. Acute exacerbations are less prevalent in other fibrotic lung diseases than in IPF and may have a better outcome, with the exception of acute exacerbations of rheumatoid lung. In AE-IPF, the exclusion of alternative causes of rapid deterioration, including heart failure and infection, is the main goal of investigation. Empirical high-dose corticosteroid steroid therapy is generally used in AE-IPF, without proven benefit.

Interstitial Lung Disease Induced by Drugs and Radiation

Abstract: An ever-increasing number of drugs can reproduce variegated patterns of naturally occurring interstitial lung disease (ILD), including most forms of interstitial pneumonias, alveolar involvement and, rarely, vasculitis. Drugs in one therapeutic class may collectively produce the same pattern of involvement. A few drugs can produce more than one pattern of ILD. The diagnosis of drug-induced ILD (DI-ILD) essentially rests on the temporal association between exposure to the drug and the development of pulmonary infiltrates. The histopathological features of DI-ILD are generally consistent, rather than suggestive or specific to the drug etiology. Thus, the diagnosis of DI-ILD is mainly made by the meticulous exclusion of all other possible causes. Drug dechallenge produces measurable improvement in symptoms and imaging in the majority of patients, whereas corticosteroid therapy is indicated if symptoms are present or drug dechallenge is without an effect. Rechallenge is justified in a minority of patients, and is discouraged for diagnostic purposes only. Pneumotox ® (www.pneumotox.com) provides updated information on drug-induced respiratory disease.

Transbronchial Cryobiopsy: A New Tool for Lung Biopsies

Abstract: Background: Specimens from transbronchial lung biopsies lack sufficient quality due to crush artifact and are generally too small for diagnosis of diffuse lung diseases. Flexible cr