scholarly journals Alpha-1 Antitrypsin Deficiency and Chronic Obstructive Pulmonary Disease (COPD) Phenotypes in a Canadian Population: From the Canadian Obstructive Lung Disease (CanCOLD) Cohort Study

2020 ◽  
Author(s):  
N. Gupta ◽  
N. Gaudreault ◽  
S. Thériault ◽  
P.Z. Li ◽  
C. Henry ◽  
...  
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Cohort Study ◽  
Lung Disease ◽  
Obstructive Lung Disease ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Canadian Population ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Alpha 1-antitrypsin deficiency in patients with chronic obstructive pulmonary disease patients: is systematic screening necessary?

2019 ◽  
Vol 12 (1) ◽  
Author(s):  
Cláudia Henrique da Costa ◽  
Arnaldo José Noronha Filho ◽  
Rosa Maria Fernambel Marques e Silva ◽  
Thaís Ferrari da Cruz ◽  
Valeria de Oliveira Monteiro ◽  
...  
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Pulmonary Disease ◽  
Chronic Obstructive ◽  
Systematic Screening ◽  
Obstructive Pulmonary Disease ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Relationship of CT densitometry to lung physiological parameters and health status in alpha-1 antitrypsin deficiency: initial report of a centralised database of the NIHR rare diseases translational research collaborative

BMJ Open ◽  
2020 ◽  
Vol 10 (6) ◽  
pp. e036045
Author(s):  
Diana Crossley ◽  
James Stockley ◽  
Charlotte E Bolton ◽  
Nicholas S Hopkinson ◽  
Ravi Mahadeva ◽  
...  
Keyword(s):  
Health Status ◽  
Lung Function ◽  
Lung Disease ◽  
Clinical Features ◽  
Obstructive Lung Disease ◽  
Chronic Obstructive Lung Disease ◽  
Chronic Obstructive ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

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.

scholarly journals One-year Prevalence, Comorbidities, and Cost of Hospitalizations for Alpha-1 Antitrypsin Deficiency among Patients with Chronic Obstructive Pulmonary Disease in the United States

10.36469/9799 ◽  
2017 ◽  
Vol 5 (1) ◽  
pp. 65-74
Author(s):  
Christopher M. Blanchette ◽  
Emily Zacherle ◽  
Joshua M. Noone ◽  
Bryce A. Van Doren ◽  
Debosree Roy ◽  
...  
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Liver Disease ◽  
Length Of Stay ◽  
Alcoholic Liver Disease ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Patient Demographics ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Objectives: Little is known about severe chronic obstructive pulmonary disease (COPD) exacerbations among patients with Alpha-1 Antitrypsin Deficiency (AATD). We assessed inpatients with AATD and COPD among a sample of COPD inpatients to ascertain demographic, clinical and economic differences in the course of disease and treatment. Methods: Using data from the 2009 Nationwide Inpatient Sample (NIS), we identified COPD (ICD-9-CM: 491.xx, 492.xx, or 496.xx) patients with AATD (273.4). We compared patient demographics and healthcare outcomes (eg, length of stay, inpatient death, type and number of procedures, and cost of care) between COPD patients with and without alpha-1 antitrypsin deficiency. Frequencies and percentages for patient demographics were compared using bivariate statistics (eg, chi-square test). Recognizing the non-parametric nature of length of stay and cost, we calculated median values and interquartile ranges for these variables for each group of patients. Finally, the risk of inpatient death was estimated using logistic regression. Results: Of 840 242 patients with COPD (10.8% of the NIS sample population), 0.08% (684) had a primary or secondary diagnosis code for AATD. COPD+AATD were younger (56 vs 70, p&lt;0.0001) and as a result, less likely to be covered by Medicare (44% vs 62%, p&lt;0.0001). AATD patients were also more likely to have comorbid non-alcoholic liver disease (7% vs 2%, p&lt;0.0001), depression (17% vs 13%, p=0.0328), and pulmonary circulation disorders (7% vs 4%, p=0.0299). Patients with AATD had a 14% longer length of stay (IRR = 1.14, 95% CI 1.07, 1.21) and a mean cost of $1487 (p=0.0251) more than COPD inpatients without AATD. Conclusions: AATD is associated with increased mean length of stay and cost, as well as higher frequency of comorbid non-alcoholic liver disease, depression, and pulmonary circulation disorders. Future research should assess other differences between AATD and the general COPD population such as natural history of disease, treatment responsiveness and disease progression.

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