Alpha-1-antitrypsin deficient man presenting with lung function decline associated with dust exposure: a case report

We hypothesised that exposure to workplace aerosols may lead to lung function impairment among cement production workers.Our study included 4966 workers in 24 cement production plants. Based on 6111 thoracic aerosol samples and information from questionnaires we estimated arithmetic mean exposure levels by plant and job type. Dynamic lung volumes were assessed by repeated spirometry testing during a mean follow-up time of 3.5 years (range 0.7–4.6 years). The outcomes considered were yearly change of dynamic lung volumes divided by the standing height squared or percentage of predicted values. Statistical modelling was performed using mixed model regression. Individual exposure was classified into quintile levels limited at 0.09, 0.89, 1.56, 2.25, 3.36, and 14.6 mg·m−3, using the lowest quintile as the reference. Employees that worked in administration were included as a second comparison group.Exposure was associated with a reduction in forced expiratory volume in 1 s (FEV1), forced expiratory volume in 6 s and forced vital capacity. For FEV1% predicted a yearly excess decline of 0.84 percentage points was found in the highest exposure quintile compared with the lowest.Exposure at the higher levels found in this study may lead to a decline in dynamic lung volumes. Exposure reduction is therefore warranted.

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Prevalence and impact of co-morbidity in COPD due to Alpha 1 Antitrypsin deficiency

Internal Medicine Review ◽

10.18103/imr.v1i1.20 ◽

2016 ◽

Vol 1 (1) ◽

Author(s):

Anilkumar Pillai ◽

Anita Pye ◽

Robert A Stockley ◽

Alice M Turner

Keyword(s):

Lung Function ◽

Multivariate Analyses ◽

Prognostic Score ◽

Chronic Obstructive ◽

Lung Function Decline ◽

Gold Stage ◽

Co Morbidity ◽

Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin

BackgroundAlpha 1 antitrypsin deficiency (AATD) is a rare genetic cause of chronic obstructive pulmonary disease (COPD). There are a number of conditions which are recognized to be common as co-morbidities in COPD unrelated to AATD, and a co-morbidity specific prognostic score exists (COTE index). In this manuscript we sought to describe the COPD related, and unrelated, co-morbidities seen in AATD and assess their impact on outcome. The relevant literature is also reviewed.MethodsAll PiZZ AATD patients who have COPD from the UK AATD registry were selected, and graded for severity of COPD using the GOLD criteria. Medical notes were reviewed to ascertain co-morbidity, and used to calculate the COTE score. Each co-morbid condition was tested for association with GOLD stage and subsequent death. Multivariate analyses, adjusting for smoking, age and GOLD stage, were used to ascertain independent relationships to death. COTE score was compared between GOLD groups and between survivors and those that died. Selected co-morbid diseases were also assessed using multivariate analyses for their relation to lung function decline.ResultsThe most common co-morbid disease in AATD was bronchiectasis (31.1% of patients). Some common COPD related conditions were seen, such as osteoporosis (11.9%). Liver disease was observed in 5.5% of patients. In univariate analyses the presence of osteoporosis, depression or gastro-oesophageal reflux (GORD) associated with GOLD stage, being more common in group D patients (all p<0.05). No co-morbid disease was associated with death after adjustment for co-variates. COTE scores were generally low and did not differ between survivors and those that died.ConclusionCo-morbid disease is common in AATD, but differs from usual COPD, and has little impact on mortality and lung function decline. The COTE index is not valid for use in AATD.

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Longitudinal lung function decline and wood dust exposure in the furniture industry

European Respiratory Journal ◽

10.1183/09031936.00146806 ◽

2008 ◽

Vol 31 (2) ◽

pp. 334-342 ◽

Cited By ~ 24

Author(s):

G. Jacobsen ◽

V. Schlunssen ◽

I. Schaumburg ◽

E. Taudorf ◽

T. Sigsgaard

Keyword(s):

Lung Function ◽

Dust Exposure ◽

Wood Dust ◽

Furniture Industry ◽

Lung Function Decline

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The Natural History of Lung Function in Severe Deficiency of Alpha-1 Antitrypsin Following Orthotopic Liver Transplantation: A Case Report - See more at: http://journal.copdfoundation.org/#sthash.rILbXrxo.dpuf

Chronic Obstructive Pulmonary Diseases Journal of the COPD Foundation ◽

10.15326/jcopdf.2.4.2015.0141 ◽

2015 ◽

Vol 2 (4) ◽

pp. 290-295

Author(s):

Vickram Tejwani ◽

Xiao-Feng Wang ◽

James K Stoller

Keyword(s):

Liver Transplantation ◽

Case Report ◽

Lung Function ◽

Natural History ◽

Orthotopic Liver Transplantation ◽

Severe Deficiency ◽

History Of ◽

Alpha 1 Antitrypsin

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Accelerated lung function decline in an aluminium manufacturing industry cohort exposed to PM2.5: an application of the parametric g-formula

Occupational and Environmental Medicine ◽

10.1136/oemed-2019-105908 ◽

2019 ◽

Vol 76 (12) ◽

pp. 888-894 ◽

Cited By ~ 1

Author(s):

Andreas M Neophytou ◽

Sadie Costello ◽

Sally Picciotto ◽

Elizabeth M Noth ◽

Sa Liu ◽

...

Keyword(s):

Lung Function ◽

Manufacturing Industry ◽

Fine Particulate Matter ◽

Forced Expiratory Volume ◽

Dust Exposure ◽

Lung Function Decline ◽

Exposure Distribution ◽

The Usa ◽

Composite Risk ◽

Over Time

ObjectiveOccupational dust exposure has been associated with accelerated lung function decline, which in turn is associated with overall morbidity and mortality. In the current study, we assess potential benefits on lung function of hypothetical interventions that would reduce occupational exposure to fine particulate matter (PM2.5) while adjusting for the healthy worker survivor effect.MethodsAnalyses were performed in a cohort of 6485 hourly male workers in an aluminium manufacturing company in the USA, followed between 1996 and 2013. We used the parametric g-formula to assess lung function decline over time under hypothetical interventions while also addressing time-varying confounding by underlying health status, using a composite risk score based on health insurance claims.ResultsA counterfactual scenario envisioning a limit on exposure equivalent to the 10th percentile of the observed exposure distribution of 0.05 mg/m3 was associated with an improvement in forced expiratory volume in one second (FEV1) equivalent to 37.6 mL (95% CI 13.6 to 61.6) after 10 years of follow-up when compared with the observed. Assuming a linear decrease and (from NHANES reference values), a 20 mL decrease per year for a 1.8 m-tall man as they age, this 37.6 mL FEV1 loss over 10 years associated with observed exposure would translate to approximately a 19% increase to the already expected loss per year from age alone.ConclusionsOur results indicate that occupational PM2.5 exposure in the aluminium industry accelerates lung function decline over age. Reduction in exposure may mitigate accelerated loss of lung function over time in the industry.

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Lung function decline: Screening of alpha-1 antitrypsin gene in a population exposed to coal dust

International Journal for Biotechnology and Molecular Biology Research ◽

10.5897/ijbmbr11.051 ◽

2011 ◽

Vol 2 (11) ◽

Cited By ~ 1

Author(s):

M. Bhattacharjee

Keyword(s):

Lung Function ◽

Coal Dust ◽

Lung Function Decline ◽

Alpha 1 Antitrypsin

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Faculty Opinions recommendation of Lung function decline and outcomes in an adult population.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.30409.486908 ◽

2006 ◽

Author(s):

Harald Groeben

Keyword(s):

Lung Function ◽

Adult Population ◽

Lung Function Decline

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Faculty Opinions recommendation of Genome-wide study identifies two loci associated with lung function decline in mild to moderate COPD.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.717986716.793472285 ◽

2013 ◽

Author(s):

Craig Hersh

Keyword(s):

Lung Function ◽

Lung Function Decline ◽

Genome Wide ◽

Genome Wide Study

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Lung Function and Quality of Life in Workers with Chemical and Dust Exposure

Revista de Chimie ◽

10.37358/rc.18.2.6104 ◽

2018 ◽

Vol 69 (2) ◽

pp. 346-349 ◽

Cited By ~ 1

Author(s):

Marina Ruxandra Otelea ◽

Oana Cristina Arghir ◽

Corina Zugravu ◽

Eugenia Naghi ◽

Sabina Antoniu ◽

...

Keyword(s):

Quality Of Life ◽

Occupational Exposure ◽

Lung Function ◽

Lung Diseases ◽

Dust Exposure ◽

Chronic Lung Diseases ◽

Respiratory Exposure ◽

Increased Risk ◽

Occupational Settings

Regarding the widely distribution of respiratory exposure hazards in occupational settings, workers have an increased risk for chronic lung diseases. For assessing the quality of life and lung function in workers exposed to chemicals and dust, St George�s Respiratory Questionnaire (SGRQ) and spirometry were performed among 40 patients, admitted in Occupational Clinic Department of Colentina Hospital, Bucharest, Romania, during February, 2017. SGRQ showed different predictors for patients according to their occupational exposure and total symptoms score correlated better with decreased spirometric parameters in defining lung function deterioration. Quality of life is earlier affected than lung function deterioration and emphasises the need of more sensitive methods for an earlier identification and better evaluation of respiratory hazards in different workplaces.

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Air Trapping Is Associated with Heterozygosity for Alpha-1 Antitrypsin Mutations in Patients with Asthma

Respiration ◽

10.1159/000513072 ◽

2021 ◽

pp. 1-10

Author(s):

Marina Aiello ◽

Marianna Ghirardini ◽

Laura Marchi ◽

Annalisa Frizzelli ◽

Roberta Pisi ◽

...

Keyword(s):

Lung Function ◽

Serum Concentration ◽

Forced Expiratory Volume ◽

Small Airway ◽

University Hospital ◽

Air Trapping ◽

Asthmatic Patients ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin ◽

Few Data

Background: Alpha-1 antitrypsin deficiency (AATD) is a hereditary disorder involving lungs, characterized by low serum concentration of the protein alpha-1 antitrypsin (AAT) also called proteinase inhibitor (PI). Asthma is common in AATD patients, but there are only few data on respiratory function in asthmatic patients with AATD. Objectives: The aim of the study was to evaluate lung function in asthmatic outpatients with mutation in the SERPINA1 gene coding for AAT versus asthmatic subjects without mutation. Methods: We performed the quantitative analysis of the serum concentration of AAT in 600 outpatients affected by mild to moderate asthma from the University Hospital of Parma, Italy. Fifty-seven of them underwent the genetic analysis subsequently; they were subdivided into mutated and non-mutated subjects. All the mutated patients had a heterozygous genotype, except 1 (PI*SS). We assessed the lung function through a flow-sensing spirometer and the small airway parameters through an impulse oscillometry system. Results: The values of forced vital capacity (% predicted) and those of the residual volume to total lung capacity ratio (%) were, respectively, lower and higher in patients mutated versus patients without mutation, showing a significantly greater air trapping (p = 0.014 and p = 0.017, respectively). Moreover, patients with mutation in comparison to patients without mutation showed lower forced expiratory volume in 3 s (% predicted) and forced expiratory volume in 6 s (L) spirometric values, reflecting a smaller airways contribution. Conclusions: In asthmatic patients, heterozygosity for AAT with PI*MZ and PI*MS genotypes was associated with small airway dysfunction and with lung air trapping.

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