scholarly journals Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin Deficiency at the Age of 42

2021 ◽  
Vol Volume 16 ◽  
pp. 3477-3485
Author(s):  
Georg Rüdiger Schramm ◽  
Behrouz Mostafavi ◽  
Eeva Piitulainen ◽  
Per Wollmer ◽  
Hanan A Tanash
Keyword(s):  
Health Status ◽  
Lung Function ◽  
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 Lung function and CT lung densitometry in 37- to 39-year-old individuals with alpha-1-antitrypsin deficiency

2018 ◽  
Vol Volume 13 ◽  
pp. 3689-3698 ◽  
Author(s):  
Behrouz Mostafavi ◽  
Sandra Diaz ◽  
Eeva Piitulainen ◽  
Berend Stoel ◽  
Per Wollmer ◽  
...  
Keyword(s):  
Lung Function ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Lung Densitometry

scholarly journals Long-term evolution of lung function in individuals with alpha-1 antitrypsin deficiency from the Spanish registry (REDAAT)

2018 ◽  
Vol Volume 13 ◽  
pp. 1001-1007 ◽  
Author(s):  
Cristina Esquinas ◽  
Sonia Serreri ◽  
Miriam Barrecheguren ◽  
Esther Rodríguez ◽  
Alexa Nuñez ◽  
...  
Keyword(s):  
Lung Function ◽  
Long Term Evolution ◽  
Term Evolution ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Neutrophil elastase gene expression and relation with lung function in Alpha-1 Antitrypsin deficiency patients

2017 ◽  
Author(s):  
Nerea Matamala ◽  
Gema Gomez-Mariano ◽  
Beatriz Lara ◽  
Maria Teresa Martinez ◽  
Esther Rodriguez ◽  
...  
Keyword(s):  
Gene Expression ◽  
Lung Function ◽  
Neutrophil Elastase ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals Heritability of Lung Function in Severe Alpha-1 Antitrypsin Deficiency

2009 ◽  
Vol 67 (1) ◽  
pp. 38-45 ◽  
Author(s):  
D.L. DeMeo ◽  
E.J. Campbell ◽  
M.L. Brantly ◽  
A.F. Barker ◽  
E. Eden ◽  
...  
Keyword(s):  
Lung Function ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin
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