Citations to this article

Assessment of alpha-1-antitrypsin deficiency heterozygosity as a risk factor in the etiology of emphysema. Physiological comparison of adult normal and heterozygous protease inhibitor phenotype subjects from a random population.
D J McDonagh, … , R J Knudson, M D Lebowitz
D J McDonagh, … , R J Knudson, M D Lebowitz
Published February 1, 1979
Citation Information: J Clin Invest. 1979;63(2):299-309. https://doi.org/10.1172/JCI109303.
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Assessment of alpha-1-antitrypsin deficiency heterozygosity as a risk factor in the etiology of emphysema. Physiological comparison of adult normal and heterozygous protease inhibitor phenotype subjects from a random population.

Abstract

For plethysmographic studies of lung mechanics and measurement of pulmonary diffusing capacity, 62 subjects were drawn from a randomly selected population sample. Data obtained from the 24 subjects of heterozygous phenotype for alpha-1-antitrypsin deficiency (PiMZ) were compared by age group with data from 38 normal (PiM) subjects matched for sex, age, and smoking history. Comparison of mean values by age group for lung volumes, diffusing capacity, lung elastic recoil, maximum expiratory flow, and the occurrence of frequency dependence of dynamic compliance revealed no differences between phenotype groups. There was no evidence of an accelerated effect of aging among PiMZ subjects when compared with normal counterparts nor was there evidence of an increased effect of smoking. From these data it appears that the PiMZ phenotype per se is not a risk factor in the development of emphysema.

Authors

D J McDonagh, S P Nathan, R J Knudson, M D Lebowitz

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Citations to this article (21)

Title and authors Publication Year
Longitudinal Decline of Diffusing Capacity of the Lung for Carbon Monoxide in Community Subjects With the PiMZ α1-Antitrypsin Phenotype
GE Silva, S Guerra, S Keim, RA Barbee, DL Sherrill 		Chest 2008
Lung Biology in Health and Disease: Cellular and Molecular Mechanisms
E Sapey, R Stockley 		Lung Biology in Health and Disease: Experimental Models and Therapeutic Potential 2005
Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis
CP Hersh, M Dahl, NP Ly, CS Berkey, BG Nordestgaard, EK Silverman 		Thorax 2004
Genetic risk factors for chronic obstructive pulmonary disease
AJ Sandford, L Joos, PD Paré 		Current Opinion in Pulmonary Medicine 2002
Z and S Mutations of the α 1 -Antitrypsin Gene and the Risk of Chronic Obstructive Pulmonary Disease
AJ Sandford, TD Weir, JJ Spinelli, PD Paré 		American journal of respiratory cell and molecular biology 1999
Genes, oxidative stress, and the risk of chronic obstructive pulmonary disease
H Koyama, DM Geddes 		Thorax 1998
OBSTRUCTIVE PULMONARY DISEASE
MS Stulbarg, JA Frank 		Radiologic Clinics of North America 1998
Frequency and significance of phenotypes for alpha1-antitrypsin deficiency in type 1 autoimmune hepatitis
A J Czaja 		Digestive Diseases and Sciences 1998
The Trends in Airway Obstructive Disease Morbidity in the Tucson Epidemiological Study
MD Lebowitz 		American journal of respiratory and critical care medicine 1989
The Flaccid Lung Syndrome and α1-Protease Inhibitor Deficiency
KD Laros, I Biemond, EC Klasen 		Chest 1988
Southwestern Internal Medicine Conference: Pulmonary Emphysema: Current Concepts of Pathogenesis
JC Weissler 		The American Journal of the Medical Sciences 1987
Alpha1-Antitrypsin Pi-Types in 965 COPD Patients
J Lieberman, B Winter, A Sastre 		Chest 1986
Rheumatoid arthritis and small airways function
JD Mountz, RA Turner, RL Collins, KR Gallup, EL Semble 		Arthritis & Rheumatism 1984
The effects of pressurized aerosols on respiratory symptoms and physiology
MD Lebowitz 		Science of The Total Environment 1983
A Case of Rare Heterozygous α1-Antitrypsin Phenotype: IS
K Bencze, X Baur 		Clinical Chemistry and Laboratory Medicine 1982
Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects
JE Gadek, HG Klein, PV Holland, RG Crystal 		Journal of Clinical Investigation 1981
Evaluation of lung elastic recoil by exponential curve analysis
RJ Knudson, WT Kaltenborn 		Respiration Physiology 1981
Biochemistry, Pathology and Genetics of Pulmonary Emphysema
L Andreotti, D Cammelli, A Bussotti, P Arcangeli 		Biochemistry, Pathology and Genetics of Pulmonary Emphysema 1981
Danazol-induced Augmentation of Serum α1-Antitrypsin Levels in Individuals with Marked Deficiency of this Antiprotease
JE Gadek, JD Fulmer, JA Gelfand, MM Frank, TL Petty, RG Crystal 		Journal of Clinical Investigation 1980
Lung Distensibility and Airway Function in Intermediate Alpha1-Antitrypsin Deficiency (PiMZ)
NB Pride, SF Tattersall, RP Pereira, D Hunter, G Blundell 		Chest 1980
Lung distensibility and airway function in intermediate alpha 1-antitrypsin deficiency (Pi MZ)
SF Tattersall, RP Pereira, D Hunter, G Blundell, NB Pride 		Thorax 1979

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