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.
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Selected References
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- BLAKEMORE W. S., FORSTER R. E., MORTON J. W., OGILVIE C. M. A standardized breath holding technique for the clinical measurement of the diffusing capacity of the lung for carbon monoxide. J Clin Invest. 1957 Jan;36(1 Pt 1):1–17. doi: 10.1172/JCI103402. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Barnett T. B., Gottovi D., Johnson A. M. Protease inhibitors in chronic obstructive pulmonary disease. Am Rev Respir Dis. 1975 May;111(5):587–593. doi: 10.1164/arrd.1975.111.5.587. [DOI] [PubMed] [Google Scholar]
- Black L. F., Kueppers F. alpha1-Antitrypsin deficiency in nonsmokers. Am Rev Respir Dis. 1978 Mar;117(3):421–428. doi: 10.1164/arrd.1978.117.3.421. [DOI] [PubMed] [Google Scholar]
- Briscoe W. A., Kueppers F., Davis A. L., Bearn A. G. A case of inherited deficiency of serum alpha-antitrypsin associated with pulmonary emphysema. Am Rev Respir Dis. 1966 Oct;94(4):529–539. doi: 10.1164/arrd.1966.94.4.529. [DOI] [PubMed] [Google Scholar]
- Chan-Yeung M., Ashley M. J., Corey P., Maledy H. Pi phenotypes and the prevalence of chest symptoms and lung function abnormalities in workers employed in dusty industris. Am Rev Respir Dis. 1978 Feb;117(2):239–245. doi: 10.1164/arrd.1978.117.2.239. [DOI] [PubMed] [Google Scholar]
- Cohen B. H., Ball W. C., Jr, Bias W. B., Brashears S., Chase G. A., Diamond E. L., Hsu S. H., Kreiss P., Levy D. A., Menkes H. A. A genetic-epidemiologic study of chronic obstructive pulmonary disease. I. Study design and preliminary observations. Johns Hopkins Med J. 1975 Sep;137(3):95–104. [PubMed] [Google Scholar]
- Cole R. B., Nevin N. C., Blundell G., Merrett J. D., McDonald J. R., Johnston W. P. Relation of alpha-1-antitrypsin phenotype to the performance of pulmonary function tests and to the prevalence of respiratory illness in a working population. Thorax. 1976 Apr;31(2):149–157. doi: 10.1136/thx.31.2.149. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Cooper D. M., Hoeppner V., Cox D., Zamel N., Bryan A. C., Levison H. Lung function in alpha1-antitrypsin heterozygotes (Pi type MZ). Am Rev Respir Dis. 1974 Dec;110(6):708–715. doi: 10.1164/arrd.1974.110.6P1.708. [DOI] [PubMed] [Google Scholar]
- Cox D. W., Hoeppner V. H., Levison H. Protease inhibitors in patients with chronic obstructive pulmonary disease: the alpha-antitrypsin heterozygote controversy. Am Rev Respir Dis. 1976 May;113(5):601–606. doi: 10.1164/arrd.1976.113.5.601. [DOI] [PubMed] [Google Scholar]
- DUBOIS A. B., BOTELHO S. Y., BEDELL G. N., MARSHALL R., COMROE J. H., Jr A rapid plethysmographic method for measuring thoracic gas volume: a comparison with a nitrogen washout method for measuring functional residual capacity in normal subjects. J Clin Invest. 1956 Mar;35(3):322–326. doi: 10.1172/JCI103281. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dosman J., Bode F., Urbanetti J., Antic R., Martin R., Macklem P. T. Role of inertia in the measurement of dynamic compliance. J Appl Physiol. 1975 Jan;38(1):64–69. doi: 10.1152/jappl.1975.38.1.64. [DOI] [PubMed] [Google Scholar]
- ERIKSSON S. PULMONARY EMPHYSEMA AND ALPHA1-ANTITRYPSIN DEFICIENCY. Acta Med Scand. 1964 Feb;175:197–205. doi: 10.1111/j.0954-6820.1964.tb00567.x. [DOI] [PubMed] [Google Scholar]
- Fagerhol M. K., Laurell C. B. The polymorphism of "prealbumins" and alpha-1-antitrypsin in human sera. Clin Chim Acta. 1967 May;16(2):199–203. doi: 10.1016/0009-8981(67)90181-7. [DOI] [PubMed] [Google Scholar]
- Gelb A. F., Gold W. M., Wright R. R., Bruch H. R., Nadel J. A. Physiologic diagnosis of subclinical emphysema. Am Rev Respir Dis. 1973 Jan;107(1):50–63. doi: 10.1164/arrd.1973.107.1.50. [DOI] [PubMed] [Google Scholar]
- Gelb A. F., Klein E., Lieberman J. Pulmonary function in nonsmoking subjects with alpha1 antitrypsin deficiency (MZ phenotype). Am J Med. 1977 Jan;62(1):93–98. doi: 10.1016/0002-9343(77)90354-0. [DOI] [PubMed] [Google Scholar]
- Girard F., Aug F., Camara M., Bohadana A. B., Bagrel A., Museur G., Abraham S. Bilan pulmonaire et déficit hétérozygote en alpha 1-antitrypsine au sein d'une population active générale. Bull Eur Physiopathol Respir. 1978 Jan-Feb;14(1):11–22. [PubMed] [Google Scholar]
- Guenter C. A., Welch M. H., Russell T. R., Hyde R. M., Hammarsten J. F. The pattern of lung disease associated with alpha antitrypsin deficiency. Arch Intern Med. 1968 Sep;122(3):254–257. [PubMed] [Google Scholar]
- Hall W. J., Hyde R. W., Schwartz R. H., Mudholkar G. S., Webb D. R., Chaubey Y. P., Townes P. L. Pulmonary abnormalities in intermediate alpha-1-antitrypsin deficiency. J Clin Invest. 1976 Nov;58(5):1069–1077. doi: 10.1172/JCI108558. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kazazian H. H., Jr A geneticist's view of lung disease. Am Rev Respir Dis. 1976 Mar;113(3):261–266. doi: 10.1164/arrd.1976.113.3.261. [DOI] [PubMed] [Google Scholar]
- Klayton R., Fallat R., Cohen A. B. Determinants of chronic obstructive pulmonary disease in patients with intermediate levels of alpha-antitrypsin. Am Rev Respir Dis. 1975 Jul;112(1):71–75. doi: 10.1164/arrd.1975.112.1.71. [DOI] [PubMed] [Google Scholar]
- Knudson R. J., Clark D. F., Kennedy T. C., Knudson D. E. Effect of aging alone on mechanical properties of the normal adult human lung. J Appl Physiol Respir Environ Exerc Physiol. 1977 Dec;43(6):1054–1062. doi: 10.1152/jappl.1977.43.6.1054. [DOI] [PubMed] [Google Scholar]
- Knudson R. J., Slatin R. C., Lebowitz M. D., Burrows B. The maximal expiratory flow-volume curve. Normal standards, variability, and effects of age. Am Rev Respir Dis. 1976 May;113(5):587–600. doi: 10.1164/arrd.1976.113.5.587. [DOI] [PubMed] [Google Scholar]
- Larson R. K., Barman M. L., Kueppers F., Fudenberg H. H. Genetic and environmental determinants of chronic obstructive pulmonary disease. Ann Intern Med. 1970 May;72(5):627–632. doi: 10.7326/0003-4819-72-5-627. [DOI] [PubMed] [Google Scholar]
- Larsson C., Eriksson S., Dirksen H. Smoking and intermediate alpha1-antitrypsin deficiency and lung function in middle-aged men. Br Med J. 1977 Oct 8;2(6092):922–925. doi: 10.1136/bmj.2.6092.922. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lebowitz M. D., Knudson R. J., Burrows B. Tucson epidemiologic study of obstructive lung diseases. I: Methodology and prevalence of disease. Am J Epidemiol. 1975 Aug;102(2):137–152. doi: 10.1093/oxfordjournals.aje.a112141. [DOI] [PubMed] [Google Scholar]
- Lebowitz M. D., Knudson R. J., Morse J. O., Armet D. Closing volume and flow volume abnormalities in alpha(1)-antitrypsin phenotype groups in a community population. Am Rev Respir Dis. 1978 Jan;117(1):179–181. doi: 10.1164/arrd.1978.117.1.179. [DOI] [PubMed] [Google Scholar]
- Lemen R., Benson M., Jones J. G. Absolute pressure measurements with hand-dipped and manufactured esophageal balloons. J Appl Physiol. 1974 Oct;37(4):600–603. doi: 10.1152/jappl.1974.37.4.600. [DOI] [PubMed] [Google Scholar]
- MILIC-EMILI J., MEAD J., TURNER J. M., GLAUSER E. M. IMPROVED TECHNIQUE FOR ESTIMATING PLEURAL PRESSURE FROM ESOPHAGEAL BALLOONS. J Appl Physiol. 1964 Mar;19:207–211. doi: 10.1152/jappl.1964.19.2.207. [DOI] [PubMed] [Google Scholar]
- Mead J., Turner J. M., Macklem P. T., Little J. B. Significance of the relationship between lung recoil and maximum expiratory flow. J Appl Physiol. 1967 Jan;22(1):95–108. doi: 10.1152/jappl.1967.22.1.95. [DOI] [PubMed] [Google Scholar]
- Morse J. O., Lebowitz M. D., Knudson R. J., Burrows B. A community study of the relation of alpha1-antitrypsin levels to obstructive lung diseases. N Engl J Med. 1975 Feb 6;292(6):278–281. doi: 10.1056/NEJM197502062920602. [DOI] [PubMed] [Google Scholar]
- Morse J. O., Lebowitz M. D., Knudson R. J., Burrows B. Relation of protease inhibitor phenotypes to obstructive lung diseases in a community. N Engl J Med. 1977 May 26;296(21):1190–1194. doi: 10.1056/NEJM197705262962102. [DOI] [PubMed] [Google Scholar]
- Nelson D. C., Burrows B., Knudson R. J. A device for recording flow and volume data in population surveys. J Appl Physiol. 1973 Aug;35(2):304–306. doi: 10.1152/jappl.1973.35.2.304. [DOI] [PubMed] [Google Scholar]
- Ostrow D. N., Cherniack R. M. The mechanical properties of the lungs in intermediate deficiency of 1 -antitrypsin. Am Rev Respir Dis. 1972 Sep;106(3):377–383. doi: 10.1164/arrd.1972.106.3.377. [DOI] [PubMed] [Google Scholar]
- Pierce J. A., Jeppsson J. O., Laurell C. B. alpha-1 Antitrypsin phenotypes determined by isoelectric focusing of the cysteine-antitrypsin mixed disulfide in serum. Anal Biochem. 1976 Jul;74(1):227–241. doi: 10.1016/0003-2697(76)90327-4. [DOI] [PubMed] [Google Scholar]
- Rawlings W., Jr, Kreiss P., Levy D., Cohen B., Menkes H., Brashears S., Permutt S. Clinical, epidemiologic, and pulmonary function studies in alpha,-antitrypsin-deficient subjects of Pi Z type. Am Rev Respir Dis. 1976 Nov;114(5):945–953. doi: 10.1164/arrd.1976.114.5.945. [DOI] [PubMed] [Google Scholar]
- Richardson R. H., Guenter C. A., Welch M. H., Hyde R. M., Hammarsten J. F. The pattern of inheritance of alpha 1 antitrypsin deficiency and associated pulmonary disease. Am Rev Respir Dis. 1969 Nov;100(5):619–625. doi: 10.1164/arrd.1969.100.5.619. [DOI] [PubMed] [Google Scholar]
- Schleusener A., Talamo R. C., Paré J. A., Thurlbeck W. M. Familial emphysema. Am Rev Respir Dis. 1968 Oct;98(4):692–696. doi: 10.1164/arrd.1968.98.4.692. [DOI] [PubMed] [Google Scholar]
- Stevens P. M., Hnilica V. S., Johnson P. C., Bell R. L. Pathophysiology of hereditary emphysema. Ann Intern Med. 1971 May;74(5):672–680. doi: 10.7326/0003-4819-74-5-672. [DOI] [PubMed] [Google Scholar]
- Szczeklik A., Stankowska K., Frydecka I. Cardiopulmonary function in 1 -antitrypsin heterozygotes exposed to severe air pollution. Am Rev Respir Dis. 1973 Feb;107(2):289–291. doi: 10.1164/arrd.1973.107.2.289. [DOI] [PubMed] [Google Scholar]
- Talamo R. C., Langley C. E., Levine B. W., Kazemi H. Genetic vs. quantitative analysis of serum alpha 1 -antitrypsin. N Engl J Med. 1972 Nov 23;287(21):1067–1069. doi: 10.1056/NEJM197211232872104. [DOI] [PubMed] [Google Scholar]
- Turner J. M., Mead J., Wohl M. E. Elasticity of human lungs in relation to age. J Appl Physiol. 1968 Dec;25(6):664–671. doi: 10.1152/jappl.1968.25.6.664. [DOI] [PubMed] [Google Scholar]
- Webb D. R., Hyde R. W., Schwartz R. H., Hall W. J., Condemi J. J., Townes P. L. Serum alpha 1-antitrypsin variants. Prevalence and clinical spirometry. Am Rev Respir Dis. 1973 Oct;108(4):918–925. doi: 10.1164/arrd.1973.108.4.918. [DOI] [PubMed] [Google Scholar]