The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis.

Background: Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research.

Methods: The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management.

Coexistence of obesity and asthma determines a distinct respiratory metabolic phenotype.

Background: Epidemiologic and clinical evidence supports the existence of an obesity-related asthma phenotype. No distinct pathophysiologic elements or specific biomarkers have been identified thus far, but increased oxidative stress has been reported.

Objective: We aimed at verifying whether metabolomics of exhaled breath condensate from obese asthmatic (OA) patients, lean asthmatic (LA) patients, and obese nonasthmatic (ONA) subjects could recognize specific and statistically validated biomarkers for a separate "asthma-obesity" respiratory metabolic phenotype, here defined as "metabotype.

Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial.

Background: Pulmonary hypertension (PH) is a well-known independent prognostic factor in chronic obstructive pulmonary disease (COPD) and a sufficient criterion for lung transplant candidacy. Limited data are currently available on the hemodynamic and clinical effect of phosphodiesterase 5 inhibitors in patients with severe PH associated with COPD. This study assessed the effect of sildenafil on pulmonary hemodynamics and gas exchange in severe PH associated with COPD.

Extended analysis of exhaled and nasal nitric oxide for the evaluation of chronic cough.

Introduction: Chronic cough is usually defined as a cough that lasts for eight weeks or longer. Its etiological diagnosis is not always straightforward, and the measurement of exhaled nitric oxide (FeNO) has been proposed in patients' evaluation. No studies have assessed the usefulness of extended exhaled NO measurement for the evaluation of chronic cough.

Low alveolar and bronchial nitric oxide in severe uncomplicated obesity.

Background: Fractional concentration of exhaled nitric oxide (FeNO) is a recognized biomarker of the lower respiratory tract, where it is produced by the proximal conducting airways and the expansible peripheral bronchoalveolar compartment. We have previously shown that large increase in body mass decreases FeNO. Here we evaluated bronchial and alveolar components of the NO output of the lower respiratory tract in subjects with severe uncomplicated obesity (OB).

NMR metabolomic analysis of exhaled breath condensate of asthmatic patients at two different temperatures.

Exhaled breath condensate (EBC) collection is a noninvasive method to investigate lung diseases. EBC is usually collected with commercial/custom-made condensers, but the optimal condensing temperature is often unknown. As such, the physical and chemical properties of exhaled metabolites should be considered when setting the temperature, therefore requiring validation and standardization of the collecting procedure.

Nocturnal continuous positive airway pressure in severe non-apneic asthma. A pilot study.

Introduction: It has been demonstrated that brief periods of nocturnal continuous positive airway pressure (nCPAP) reduce airway reactivity in animal models and in patients with asthma. The effects of nCPAP in severe uncontrolled non-apneic asthmatic patients are not well known.

Aim: In this open pilot study, we aimed to assess the effect nCPAP on peak flow (PEF) variability and asthma control in this type of patients.

Clinical variability of respiratory pulmonary hypertension: implications for diagnosis and management.

Pulmonary Hypertension (PH) associated to chronic respiratory diseases is currently classified in the 3rd group, as a mild to moderate form of pre-capillary PH that progressively complicates the prognosis of associated pulmonary disease. In clinical practice, however, some unresolved issues in the respiratory PH should be considered: 1) the PH heterogeneity in some respiratory diseases, such as Chronic Obstructive Pulmonary Disease (COPD), where the prevalence of unrecognized left heart disease, or chronic pulmonary thromboembolism may change the clinical classification; 2) the combining form of severe PH which often is not correlated to chronic ventilator impairment, while outcome is strictly related to pulmonary haemodynamics. The recognition of out of proportion respiratory PH in several chronic respiratory diseases which include COPD, Idiopathic Pulmonary Fibrosis (IPF), Combined Pulmonary Fibrosis and Emphysema, Obstructive Sleep Apnea (OSA), Obesity Hypoventilation Syndrome (OHS) may be important for a comprehensive clinical classification of severe respiratory PH, as well as, for the inclusion of these patients in randomized clinical trials on PH targeted therapy.

Adiponectin: an attractive marker for metabolic disorders in Chronic Obstructive Pulmonary Disease (COPD).

Chronic Obstructive Pulmonary Disease (COPD) is a chronic inflammatory lung disease which may be complicated by development of co-morbidities including metabolic disorders. Metabolic disorders commonly associated with this disease contribute to lung function impairment and mortality. Systemic inflammation appears to be a major factor linking COPD to metabolic alterations.

Exhaled nasal nitric oxide during humming: potential clinical tool in sinonasal disease?

The use of nasal nitric oxide (nNO) in sinonasal disease has recently been advocated as a potential tool to explore upper inflammatory airway disease. However, it is currently hampered by some factors including the wide range of measurement methods, the presence of various confounding factors and the heterogeneity of the study population. The contribution of nasal airway and paranasal sinuses communicating with the nose through the ostia represents the main confounding factor.

Adiponectin affects lung epithelial A549 cell viability counteracting TNFα and IL-1ß toxicity through AdipoR1.

Adiponectin (Acrp30) exerts protective functions on metabolic and cellular processes as energy metabolism, cell proliferation and differentiation by two widely expressed receptors, AdipoR1 and AdipoR2. To date, the biological role of Acrp30 in lung has not been completely assessed but altered levels of Acrp30 and modulated expression of both AdipoRs have been related to establishment and progression of chronic obstructive pulmonary disease (COPD) and lung cancer. Here, we investigated the effects of Acrp30 on A549, a human alveolar epithelial cell line, showing how, in a time and dose-dependent manner, it decreases cell viability and increases apoptosis through ERK1/2 and AKT.

Climate change, air pollution and extreme events leading to increasing prevalence of allergic respiratory diseases.

The prevalence of asthma and allergic diseases has increased dramatically during the past few decades not only in industrialized countries. Urban air pollution from motor vehicles has been indicated as one of the major risk factors responsible for this increase.Although genetic factors are important in the development of asthma and allergic diseases, the rising trend can be explained only in changes occurred in the environment.

Separating smoking-related diseases using NMR-based metabolomics of exhaled breath condensate.

Nuclear magnetic resonance (NMR)-based metabolomics separates exhaled breath condensate (EBC) profiles of patients affected by pulmonary disease from those of healthy subjects. Here we show the discriminatory ability of NMR-based metabolomics in separating patients exposed to the same risk factor, namely, smoking habit in smoking-related diseases. Fifty duplicated EBC samples from a cohort of current smokers without chronic obstructive pulmonary disease (COPD, henceforth HS), COPD smokers, and subjects with established pulmonary Langerhans cell histiocytosis (PLCH) were analyzed by means of NMR spectroscopy followed by principal component analysis (PCA) and projection to latent structures discriminant analysis (PLS-DA).

Assessing and accessing the small airways; implications for asthma management.

Despite the wealth of experience in the management of asthma, the disease remains inadequately controlled in some patients, who face long-term respiratory impairment and disability. The disease has been characterised as an inflammatory condition affecting first the larger airways and eventually the smaller airways, but there is evidence that peripheral airway involvement defines a particular and more severe phenotype of asthma. For this reason, assessing functional and biological parameters reflective of small airways involvement is important prognostically.

Adiponectin oligomerization state and adiponectin receptors airway expression in chronic obstructive pulmonary disease.

Adiponectin (Acrp30) shows several beneficial properties and circulates as different oligomers. The role of Acrp30 in lung is not fully clear, but a link with chronic obstructive pulmonary disease (COPD) has been highlighted. In this study, we analyzed the anthropometrical and biochemical features and evaluated total Acrp30 levels of a COPD cohort without metabolic complications compared to healthy controls.

Bronchial asthma.

Purpose Of Review: The aim of this review is to underline the need for an adequate clinical and functional evaluation of respiratory function and asthma control in patients undergoing surgical procedures requiring general anesthesia to obtain useful information for an adequate preoperative pharmacological approach.

Recent Findings: It has been shown that baseline uncontrolled clinical/functional conditions of airways represent the most important risk factors for perioperative bronchospasm. In nonemergency conditions, asthma patients should undergo clinical/functional assessment at least 1 week before the surgery intervention to obtain, the better feasible control of asthma symptoms in the single patient.

Right heart and pulmonary thromboembolism from extensive splanchnic vein thrombosis after splenectomy for myeloproliferative disease.

Background: Splenectomy is a risk factor for both portal-vein and chronic thromboembolic pulmonary hypertension. The underlying mechanism is unclear, but may involve a hypercoagulable state.

Methods: We describe 1 patient with polycythemia vera who developed extensive portal thrombosis of the portal, suprahepatic, and inferior cava veins, leading to right heart thromboembolism, with a resultant pulmonary embolism subsequent to splenectomy despite heparin prophylaxis.

Exploring airway diseases by NMR-based metabonomics: a review of application to exhaled breath condensate.

There is increasing evidence that biomarkers of exhaled gases or exhaled breath condensate (EBC) may help in detecting abnormalities in respiratory diseases mirroring increased, oxidative stress, airways inflammation and endothelial dysfunction. Beside the traditional techniques to investigate biomarker profiles, "omics" sciences have raised interest in the clinical field as potentially improving disease phenotyping. In particular, metabonomics appears to be an important tool to gain qualitative and quantitative information on low-molecular weight metabolites present in cells, tissues, and fluids.

Measurement of nasal nitric oxide by hand-held and stationary devices.

Background: Nasal nitric oxide (nNO) is assessed by nasal aspiration/insufflation via one nostril or by nasal silent exhalation through a facemask and is also measured during humming, a manoeuvre that results in increased nNO in the presence of a patent osteomeatal complex. Humming nNO peak is absent in primary ciliary dyskinesia (PCD) and in cystic fibrosis (CF). Hand-held devices are used successfully for exhaled or nNO analysis.

Exhaled nitric oxide and other major exhaled compounds for the diagnosis of metabolic diseases.

Background: Many metabolic diseases including obesity, cardiovascular disease and diabetes share common pathogenetic pathways, which may involve chronic oxidative stress and inflammation. There is increasing evidence that assessment of biomarkers on exhaled gases or exhaled breath condensate may serve as a non-invasive tool to detect abnormalities in metabolic diseases mirroring increased in oxidative stress, systemic inflammation and endothelial dysfunction.

Methods: Among commonly used exhaled biomarkers, nitric oxide (NO) on exhaled air and some constituents of exhaled breath condensate in volatile or non-volatile form may represent suitable markers.

Exhaled nitric oxide monitoring in COPD using a portable analyzer.

Background: The exhaled nitric oxide (FeNO) is a non-invasive marker of airway inflammation in asthma. A very recent statement has suggested FeNO as potential outcome in chronic obstructive pulmonary disease (COPD). Recently, a new hand-held FeNO analyzer (NIOX MINO) has been developed.

Evaluation of a transcutaneous carbon dioxide monitor in severe obesity.

Objective: To determine the reliability of estimating arterial CO(2) pressure (PaCO(2)) using a recently introduced transcutaneous CO(2) pressure (PtcCO(2)) monitor in severe obese patients.

Design: Observational and interventional study.

Setting: District hospital with respiratory ward and bariatric surgery unit.