Exacerbations and duration of smoking abstinence are associated with the annual loss of FEV1 in individuals with PiZZ alpha-1-antitrypsin deficiency

Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder that is associated with a higher risk of chronic obstructive pulmonary disease (COPD) and emphysema. The annual declines in lung function (FEV1) and transfer factor of the lung for carbon monoxide (TLCO) predict all-cause mortality.
Source: Respiratory Medicine - Category: Respiratory Medicine Authors: Source Type: research

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Background. Frailty is a clinically recognized syndrome of decreased physiological reserve and a key contributor to suboptimal clinical outcomes in various lung disease groups. Interstitial lung disease (ILD) is fast approaching chronic obstructive pulmonary disease as the number one indication for lung transplantation worldwide. Our aim was to assess whether frailty is a predictor of mortality in patients with ILD referred for lung transplantation in an Australian cohort. Methods. Consecutive patients with ILD referred or on the waiting list for lung transplantation from May 2013 to December 2017 underwent frailty as...
Source: Transplantation - Category: Transplant Surgery Tags: Original Clinical Science‚ÄĒGeneral Source Type: research
DLCO measures gas movement at the alveolar-capillary interface. The DLCO at CLAD onset predicts post-CLAD survival. We hypothesized that DLCO is a sensitive measure of allograft fibrosis which would be reflected in the CLAD phenotype. Our aim was to describe the trajectory of DLCO measurements after lung transplantation based on CLAD phenotype.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (753) Source Type: research
Eosinophils are associated with the onset of chronic respiratory diseases like asthma and COPD. In lung transplantation, patients with increased bronchoalveolar lavage eosinophils demonstrated a worse chronic lung allograft dysfunction (CLAD)-free survival and overall survival. We investigated the association between blood eosinophilia, graft survival and CLAD-free survival after lung transplantation.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (751) Source Type: research
We report outcomes of SLT vs. BLT for COPD at our center.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (749) Source Type: research
The majority of potential donor lungs are not being used. The primary reason for underutilization is the concern for primary graft dysfunction (PGD). There is currently no clinically available therapy for PGD. Alpha 1 antitrypsin (A1AT) is a serine protease inhibitor with anti-inflammatory and cytoprotective properties. We and others have shown benefits of A1AT in small and large animal lung transplant studies. Before performing clinical trials, evidence of therapeutic efficacy in human lungs would be valuable.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (154) Source Type: research
Lung volume reduction surgery (LVRS) is a therapeutic option in the treatment of chronic obstructive pulmonary disease (COPD), the most common indication for lung transplantation (LTx). LVRS prior to LTx is controversial. Single institution studies report contradicting results, and the impact of undergoing LVRS prior to LTx on outcomes after LTx is unclear. We performed a propensity-matched analysis of outcomes after LTx in patients who underwent LVRS using a national database of LTx.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (927) Source Type: research
Oscillometry (Osc) is a non-invasive test of respiratory impedance that is sensitive to small airways, a region of lung that is not well characterized by conventional pulmonary function tests (PFT) and has been shown to detect chronic obstructive lung disease (COPD) prior to changes in PFT. We posit that Osc could identify small airway obstruction post-lung transplant (LTx) and discriminate between lungs from smoking vs non-smoking donors.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (763) Source Type: research
Conclusions In this proof-of-concept demonstration, we evaluated CT and MRI lung emphysema measurements and observed significantly worse MRI biomarkers of emphysema in patients with AATD compared to patients with COPD, although CT RA950 and diffusing capacity of carbon monoxide were not significantly different, underscoring the sensitivity of MRI measurements of AATD emphysema.
Source: Academic Radiology - Category: Radiology Source Type: research
Abstract PURPOSE: We generated lung morphometry measurements using single-breath diffusion-weighted MRI and three different acinar duct models in healthy participants and patients with emphysema stemming from chronic obstructive lung disease (COPD) and alpha-1 antitrypsin deficiency (AATD). METHODS: Single-breath-inhaled (3) He MRI with five diffusion sensitizations (b-value = 0, 1.6, 3.2, 4.8, and 6.4 s/cm(2) ) was used, and signal intensities were fit using a cylindrical and single-compartment acinar-duct model to estimate MRI-derived mean linear intercept (Lm ) and surface-to-volume ratio (S/...
Source: Magnetic Resonance in Medicine - Category: Radiology Authors: Tags: Magn Reson Med Source Type: research
Smokers are assessed for chronic obstructive pulmonary disease (COPD) using spirometry, with COPD defined by the Global Initiative for Chronic Obstructive Lung Disease (GOLD) as airflow limitation that is not fully reversible with bronchodilators. There is a subset of smokers with normal spirometry (by GOLD criteria), who have a low diffusing capacity of the lung for carbon monoxide (DLCO), a parameter linked to emphysema and small airway disease. The natural history of these "normal spirometry/low DLCO" smokers is unknown. From a cohort of 1570 smokers in the New York City metropolitian area, all of whom had nor...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: COPD and smoking Original Articles: COPD and smoking Source Type: research
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