Smoking and Chronic Obstructive Lung Disease in Alpha1-Antitrypsin Deficiency

CHEST Journal ◽  
1971 ◽  
Vol 60 (3) ◽  
pp. 214-221 ◽  
Author(s):  
Charles Mittman ◽  
Jack Lieberman ◽  
Fred Marasso ◽  
Armando Miranda
Keyword(s):  
Lung Disease ◽  
Obstructive Lung Disease ◽  
Chronic Obstructive Lung Disease ◽  
Chronic Obstructive ◽  
Antitrypsin Deficiency ◽  
Alpha1 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 Alpha1 antitrypsin (AT) deficiency with both cirrhosis and chronic obstructive lung disease in two sibs

1971 ◽  
Vol 5 (8) ◽  
pp. 427-427 ◽  
Author(s):  
John F T Glasgow ◽  
Albert Hercz ◽  
Henry Levison ◽  
Matthew J Lynch ◽  
Andrew Sass-Kortsak
Keyword(s):  
Lung Disease ◽  
Obstructive Lung Disease ◽  
Chronic Obstructive Lung Disease ◽  
Chronic Obstructive ◽  
At Deficiency ◽  
Alpha1 Antitrypsin

ALPHA1-ANTITRYPSIN DEFICIENCY AND DISEASE

PEDIATRICS ◽  
1970 ◽  
Vol 46 (6) ◽  
pp. 837-840
Author(s):  
Chester A. Alper ◽  
A. Myron Johnson
Keyword(s):  
Early Onset ◽  
Obstructive Lung Disease ◽  
Protein Concentration ◽  
Proteolytic Enzymes ◽  
Chronic Obstructive Lung Disease ◽  
Chronic Obstructive ◽  
Genetic Typing ◽  
Antitrypsin Deficiency ◽  
Serum Inhibitor ◽  
Alpha1 Antitrypsin

The major serum inhibitor of proteolytic enzymes is the α1-globulin, α1-antitrypsin. Hereditary deficiency of this protein is immediately recognizable on inspection of stained serum electrophoretic strips (agarose, agar, and cellulose acetate, as well as paper) in that the normal α1 band is absent (Fig. 1). Although the protein concentration of the α1-globulin zone is usually subnormal in deficient sera, it is not always so; and, the ultimate criterion for the establishment of the diagnosis must be made by genetic typing, as will be shown. Almost coincidental with the discovery of α1-antitrypsin deficiency by Laurell and Eriksson,1 in Sweden, was their observation that many persons with this abnormality had early onset chronic obstructive lung disease.

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