scholarly journals Maximal mid-expiratory flow detects early lung disease in α1-antitrypsin deficiency

2017 ◽  
Vol 49 (3) ◽  
pp. 1602055 ◽  
Author(s):  
James A. Stockley ◽  
Asem M. Ismail ◽  
Siân M. Hughes ◽  
Ross Edgar ◽  
Robert A. Stockley ◽  
...  

Pathological studies suggest that loss of small airways precedes airflow obstruction and emphysema in chronic obstructive pulmonary disease (COPD). Not all α1-antitrypsin deficiency (AATD) patients develop COPD, and measures of small airways function might be able to detect those at risk.Maximal mid-expiratory flow (MMEF), forced expiratory volume in 1 s (FEV1), ratio of FEV1/forced vital capacity (FVC), health status, presence of emphysema (computed tomography (CT) densitometry) and subsequent decline in FEV1 were assessed in 196 AATD patients.FEV1/FVC, FEV1 % predicted and lung densitometry related to MMEF % pred (r2=0.778, p<0.0001; r2=0.787, p<0.0001; r2=0.594, p<0.0001, respectively) in a curvilinear fashion. Patients could be divided into those with normal FEV1/FVC and MMEF (group 1), normal FEV1/FVC and reduced MMEF (group 2) and those with spirometrically defined COPD (group 3). Patients in group 2 had worse health status than group 1 (median total St George's Respiratory Questionnaire (SGRQ) 23.15 (interquartile range (IQR) 7.09–39.63) versus 9.67 (IQR 1.83–22.35); p=0.006) and had a greater subsequent decline in FEV1 (median change in FEV1 −1.09% pred per year (IQR −1.91–0.04% pred per year) versus −0.04% pred per year (IQR −0.67–0.03% pred per year); p=0.007).A reduction in MMEF is an early feature of lung disease in AATD and is associated with impaired health status and a faster decline in FEV1.

BMJ Open ◽  
2020 ◽  
Vol 10 (6) ◽  
pp. e036045
Author(s):  
Diana Crossley ◽  
James Stockley ◽  
Charlotte E Bolton ◽  
Nicholas S Hopkinson ◽  
Ravi Mahadeva ◽  
...  

ObjectivesTo establish a database network for the study of alpha-1 antitrypsin deficiency (AATD) and compare the results to CT lung density as the most direct measure of emphysema.DesignA central electronic database was established to permit the upload of anonymised patient data from remote sites. Prospectively collected CT data were recorded onto disc, anonymised, analysed at the coordinating centre and compared with the clinical features of the disease.SettingTertiary referral centres with expertise in the management of AATD focused on academic Biomedical Research Units and Wellcome Clinical Research Facilities.ParticipantsData were collected from 187 patients over 1 year from eight UK academic sites. This included patient demographics, postbronchodilator physiology, health status and CT. Analysis was undertaken at the coordinating centre in Birmingham.ResultsPatient recruitment in the 12 months reached 94% of target (set at 200) covering the whole spectrum of the disease from those with normal lung function to very severe chronic obstructive lung disease. CT scan suitable for analysis was available from 147 (79%) of the patients. CT density, analysed as the threshold for the lowest 15% of lung voxels, showed statistically significant relationships with the objective physiological parameters of lung function as determined by spirometric Global Initiative for Chronic Obstructive Lung Disease (GOLD) severity staging (p<0.001) and carbon monoxide gas transfer (p<0.01). Density also correlated with subjective measures of quality of life (p=0.02).ConclusionsEstablishment of the network for data collection and its transfer was highly successful facilitating future collaboration for the study of this rare disease and its management. CT densitometry correlated well with the objective clinical features of the disease supporting its role as the specific marker of the associated emphysema and its severity. Correlations with subjective measures of health, however, were generally weak indicating other factors play a role.


2018 ◽  
Vol 35 (2) ◽  
pp. 37-42
Author(s):  
R. A. Kostareva ◽  
Ya. B. Khovaeva ◽  
A. I. Podyanova

Aim. To study the left atrium (LA) remodeling in patients with different body mass, suffering from chronic obstructive lung disease (COLD). Materials and methods. Seventy two patients with COLD, who were divided into 3 groups, underwent echocardiography: group 1– patients with normal body mass (n = 31), group 2 – patients with excessive weight (n = 21), group 3 – patients with degree I obesity (n = 20). Results. Among patients with COLD, belonging to group 3, indexed parameters of minimum (15.43 ± 1.62 ml/м2, р = 0.004) and presystolic (20.52 ± 1.88 ml/м², р = 0.03) volumes of LA increased; these patients also demonstrated growth of maximum upper-lower size of LA with elevation of its linear maximum and minimum sizes, when compared with patients of group 1. Patients with different body mass, suffering from COLD, have similar volumes of passive and active depletion and their corresponding fractions. COLD patients with excessive body mass and obesity showed moderate pulmonary hypertension. Conclusions. Patients with obesity, suffering from COLD, have significantly elevated indexed parameters of presystolic and minimum LA volumes, which can be considered as early markers of impaired function of LA. Increased minimum LA volume can be one of the factors of gas exchange impairment.


2020 ◽  
Vol 24 (2) ◽  
pp. 202-206
Author(s):  
M. Atassi ◽  
A. C. F. Kava ◽  
C. Nejjari ◽  
M. C. Benjelloun ◽  
M. El Biaze ◽  
...  

BACKGROUND: Chronic obstructive lung disease (COPD) is the third most common cause of death in the world. Factors other than smoking, such as socio-economic status, could be involved in the development of COPD.OBJECTIVE: To investigate the association between chronic airflow obstruction and socio-economic status in Morocco.DESIGN: Questionnaires were administered and spirometry tests performed as part of the BOLD (Burden of Obstructive Lung Disease) Study carried out in Fez, Morocco. Socio-economic status was evaluated using a wealth score (0–10) based on household assets. The ratio of forced expiratory volume in 1 s (FEV1) to forced vital capacity (FVC) was used to measure airflow obstruction.RESULTS: A total of 760 subjects were included in the analysis. The mean age was 55.3 years (standard deviation [SD] 10.2); the average wealth score was 7.54 (SD 1.63). After controlling for other factors and potential confounders, FEV1/FVC increased by 0.4% (95%CI 0.01–0.78; P < 0.04) per unit increase in wealth score. Ageing, tobacco smoking, underweight, history of tuberculosis and asthma were also independently associated with a higher risk of airflow obstruction.CONCLUSION: Our findings suggest that airflow obstruction is associated with poverty in Morocco. Further investigations are needed to better understand the mechanisms of this association.


2021 ◽  
Vol 28 (11) ◽  
pp. 1-7
Author(s):  
Orein Fernandes ◽  
Cherishma D'Silva ◽  
Don Gregory Mascarenhas ◽  
Sydney Roshan Rebello

Background/Aims Mucus hypersecretion and altered lung functions leads to adverse clinical outcomes in chronic obstructive pulmonary disease. The aim of this study was to compare the effects of the Lung Flute and threshold positive expiratory pressure devices on sputum quantity and pulmonary functions in chronic obstructive pulmonary disease patients. Methods A total of 50 patients with chronic obstructive pulmonary disease were randomly divided into two groups. Group 1 used the Lung Flute device and group 2 used a threshold positive expiratory pressure device. Sputum quantity was measured post-intervention on a daily basis. Forced expiratory volume in the first second, forced vital capacity, and peak expiratory flow rate were evaluated on day 1 and day 6. Results The mean sputum quantity in group 1 was 11.40 ml and it was 11.04 ml in group 2. Between-group comparison demonstrated a significant improvement in forced expiratory volume in the first second for group 1 compared to group 2 (P<0.005). Conclusions The Lung Flute was found to be slightly more effective than the threshold positive expiratory pressure device for airway clearance and also had a positive effect on pulmonary functions in patients with chronic obstructive pulmonary disease.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
David M. MacDonald ◽  
Ken M. Kunisaki ◽  
Timothy J. Wilt ◽  
Arianne K. Baldomero

Abstract Background Bilirubin is a potent antioxidant and higher serum bilirubin levels have been associated with improved COPD outcomes. We performed a systematic review to evaluate the association between serum bilirubin levels and lung function (FEV1), prevalence/incidence of COPD, acute exacerbations of COPD, respiratory health status, and mortality. Methods MEDLINE® and Embase were searched using Ovid® (search updated October 1st, 2019). We included studies that measured serum bilirubin levels and outcomes of interest in adults with or without underlying lung disease. We excluded studies of those with liver disease or drug-induced elevations in bilirubin. We used the Newcastle–Ottawa scale to assess individual study risk of bias (ROB) and the US Agency for Healthcare Research and Quality—Evidence Based Practice tool to assess overall strength of evidence (SOE). Two authors independently determined eligibility, performed data abstraction, assessed ROB, and determined SOE. Results Thirteen studies (5 low risk of bias, 3 moderate and 5 high risk) were included. We found low strength of evidence for the association between higher bilirubin levels and lower risk of acute exacerbations of COPD (2 studies), mortality (3 studies), COPD diagnosis (4 studies), and lung function (FEV1) (8 studies). We found insufficient evidence on the relationship between serum bilirubin and respiratory health status/exercise capacity (1 study) and airflow obstruction (FEV1/FVC ratio) (4 studies). Conclusion Higher bilirubin levels may be associated with lower mortality and improved COPD outcomes. Randomized trials are needed to evaluate the effect of medications that raise serum bilirubin on COPD outcomes. PROSPERO registration: CRD42019145747.


2021 ◽  
Vol 12_suppl ◽  
pp. 204062232110101
Author(s):  
Igor Barjaktarevic ◽  
Michael Campos

Management of lung disease in patients with alpha-1 antitrypsin deficiency (AATD) includes both non-pharmacological and pharmacological approaches. Lifestyle changes with avoidance of environmental pollutants, including tobacco smoke, improving exercise levels and nutritional status, all encompassed under a disease management program, are crucial pillars of AATD management. Non-pharmacological therapies follow conventional treatment guidelines for chronic obstructive pulmonary disease. Specific pharmacological treatment consists of administering exogenous alpha-1 antitrypsin (AAT) protein intravenously (augmentation therapy). This intervention raises AAT levels in serum and lung epithelial lining fluid, increases anti-elastase capacity, and decreases several inflammatory mediators in the lung. Radiologically, augmentation therapy reduces lung density loss over time, thus delaying disease progression. The effect of augmentation therapy on other lung-related outcomes, such as exacerbation frequency/length, quality of life, lung function decline, and mortality, are less clear and questions regarding dose optimization or route of administration are still debatable. This review discusses the rationale and available evidence for these interventions in AATD.


2021 ◽  
Vol 31 (1) ◽  
Author(s):  
Sandeep Bansal ◽  
Martin Anderson ◽  
Antonio Anzueto ◽  
Nicola Brown ◽  
Chris Compton ◽  
...  

AbstractChronic obstructive pulmonary disease (COPD) treatment guidelines do not currently include recommendations for escalation directly from monotherapy to triple therapy. This 12-week, double-blind, double-dummy study randomized 800 symptomatic moderate-to-very-severe COPD patients receiving tiotropium (TIO) for ≥3 months to once-daily fluticasone furoate/umeclidinium/vilanterol (FF/UMEC/VI) 100/62.5/25 mcg via ELLIPTA (n = 400) or TIO 18 mcg via HandiHaler (n = 400) plus matched placebo. Study endpoints included change from baseline in trough forced expiratory volume in 1 s (FEV1) at Days 85 (primary), 28 and 84 (secondary), health status (St George’s Respiratory Questionnaire [SGRQ] and COPD Assessment Test [CAT]) and safety. FF/UMEC/VI significantly improved trough FEV1 at all timepoints (Day 85 treatment difference [95% CI] 95 mL [62–128]; P < 0.001), and significantly improved SGRQ and CAT versus TIO. Treatment safety profiles were similar. Once-daily single-inhaler FF/UMEC/VI significantly improved lung function and health status versus once-daily TIO in symptomatic moderate-to-very-severe COPD patients, with a similar safety profile.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1098.2-1098
Author(s):  
S. Barsotti ◽  
C. Roncella ◽  
A. Valentini ◽  
L. Cavagna ◽  
R. Castellana ◽  
...  

Background:Interstitial lung disease (ILD), is common in patients with idiopathic inflammatory myopathies (IIM) and strongly impact on patients’ morbidity and mortality. Patients with anti-aminoacyl-transfer RNA-synthetases (anti-ARS) antibodies are associated with an increased risk of ILD.Objectives:Defining the radiological characteristics of IIM patients, with special focus on serological groups, through qualitative, semiquantitative and quantitative analysis of lung CT.Methods:This was a prospective study conducted from 2016 to 2019. Ninety-eight IIM patients (35 men, 63 women) were included. Myositis specific autoantibodies (MSA) were assessed with Myositis Prophyle III (Euroimmune, Lubeck).Each patient had a baseline CT; the total score of Warrick (WS) was obtained at semiquantitative analysis. The radiological scores ILD% (interstitial lung disease %) and PVRS% (pulmonary vascular related structure) were the result of quantitative analysis in 61 patients (CALIPER). Pulmonary function tests (PFTs) included TLC%, FVC% and DLCO% (65 patients). The analysis was conducted in the whole group and divided in subgroups based on their MSA pattern: in particular anti-ARS (Group 1) and patients negative to MSA (Group 2) were analysed.Results:Positive correlations between ILD% and PVRS% (Rho=0.916; ρ=0.000), WS and ILD% (Rho=0.663; ρ=0.000) and WS and PVRS% (Rho=0.637; ρ<0.001) were found.The most relevant inverse correlations were found between ILD% and DLCO% (Rho=-0.590; ρ=0.001), PVRS% and DLCO% (Rho=-0.549; ρ<0.001) and WS and DLCO% (Rho=-0.471; ρ<0.001).Statistically significant higher values of WS, ILD% and PVRS% were found in Group 1 (WS=15, ILD%=11 and PVRS%=3.5), compared to Group 2 (WS=2.5, ILD%=0.84 and PVRS%=2.2). NSIP pattern resulted dominant represented in the two groups (80% Group 1, 75% Group 2). No statistically significant differences of DLCO%, FVC% and TLCO% were found.Conclusion:The inverse correlations between the radiological scores and the functional data TLC% and DLCO% (ρ<0.001) confirm the role of lung CT in the clinical management of ILD in IIM patients, and may represent a promising tool for clinical trials. For the first time anti-ARS and serological negative patients were defined through qualitative, semiquantitative and quantitative analysis of lung CT. Further study should be conducted in order to define the prognostic value of the quantitative analysis of lung CT in the follow up of IIM patients.Disclosure of Interests:None declared


Nutrients ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 2098
Author(s):  
Francisca de Castro Mendes ◽  
Kirstie Ducharme-Smith ◽  
Gustavo Mora-Garcia ◽  
Saleh A. Alqahtani ◽  
Maria Stephany Ruiz-Diaz ◽  
...  

Increasing epidemiological evidence suggests that optimal diet quality helps to improve preservation of lung function and to reduce chronic obstructive pulmonary disease (COPD) risk, but no study has investigated the association of food insecurity (FI) and lung health in the general population. Using data from a representative sample of US adults who participated in the National Health and Nutrition Examination Survey (NHANES) 2007–2012 cycles, we investigated the association between FI with lung function and spirometrically defined COPD in 12,469 individuals aged ≥ 18 years of age. FI (high vs. low) was defined using the US Department of Agriculture’s Food Security Scale). Population-weighted adjusted regression models were used to investigate associations between FI, and forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), their ratio, and spirometrically defined restriction (FVC below the lower limit of normal) and airflow obstruction (COPD). The prevalence of household FI was 13.2%. High household FI was associated with lower FVC (adjusted β-coefficient −70.9 mL, 95% CI −116.6, −25.3), and with higher odds (OR) of spirometric restriction (1.02, 95% CI 1.00, 1.03). Stratified analyses showed similar effect sizes within specific ethnic groups. High FI was associated with worse lung health in a nationally representative sample of adults in the US.


PEDIATRICS ◽  
1983 ◽  
Vol 72 (4) ◽  
pp. 517-522
Author(s):  
S. Godfrey ◽  
E. Bar-Yishay ◽  
I. Arad ◽  
L. I. Landau ◽  
L. M. Taussig

Partial expiratory flow-volume maneuvers have been performed on nine occasions on six infants with a variety of pulmonary problems using a new tech nique for thoracic compression. The infants were placed within an inflatable bag that was, itself, within a canvas bag. By sudden controlled inflation of the inner bag at end inspiration, partial expiratory flow-volume curves were generated and recorded by means of a face mask and pneumotachograph. By comparing these flow results with those airway resistance and lung volume measurements obtained from the infants in whole body plethysmography and by noting the effect of inhaling a helium/oxygen gas mixture, it was possible to partition the airway obstruction between large and small airways. The presence of small airway obstruction was noted in the absence of changes in airway resistance or lung volume in several instances. A complete evaluation of airway function should include this test of forced expiration for greater understanding and treatment of lung disease in infancy.


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