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Pulmonary function in nonsmoking subjects with alpha1 antitrypsin deficiency (MZ phenotype)

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      Abstract

      We measured pulmonary functions in 10 nonsmoking asymptomatic subjects, ages 40.5 years ± 9.2 years, with alpha1 antitrypsin heterozygous deficiency (phenotype MZ). The subjects were longstanding residents of the greater Los Angeles area. The range of physiologic studies and per cent of normal predicted values were forced vital capacity (FVC), 2.8 to 7.0 liters (86 to 124 per cent predicted); ratio of the forced expiratory volume in 1 second to the FVC, 70 to 86 per cent (86 to 104 per cent predicted); the ratio of the residual volume to total lung capacity, 28 to 44 per cent (94 to 119 per cent predicted); total lung capacity, 4.8 to 9.8 liters (80 to 119 per cent predicted); flow at 50 per cent FVC, 3.1 to 7.8 liters per second (69 to 140 per cent); and volume of isoflow, 7.3 to 26 per cent of forced vital capacity (38 to 137 per cent predicted). In eight patients studied, static deflation pressure volume curves were normal, and at respiratory rate of 60 breaths/min the ratio of dynamic compliance to static compliance did not fall below 84 per cent. We have found that these nonsmoking heterozygotes with alpha1 antitrypsin deficiency have normal pulmonary functions (within 1.67 SD of predicted mean).
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      References

        • Guenter CA
        • Welch MH
        • Russel TB
        • et al.
        The pattern of lung disease associated with alpha1-antitrypsin deficiency.
        Arch Intern Med. 1968; 122: 254
        • Talamo RC
        • Allen JD
        • Kahan MG
        • et al.
        Hereditary alpha1-antitrypsin deficiency.
        N Engl J Med. 1968; 278: 345
        • Eriksson S
        Pulmonary emphysema and alpha1-antitrypsin deficiency.
        Acta Med Scand. 1964; 175: 197
        • Hutchinson DCS
        • Cook PJI
        • Barter CE
        • et al.
        Pulmonary emphysema and alpha1-antitrypsin deficiency.
        Br Med J. 1971; 1: 689
        • Lieberman J
        Heterozygous and homozygous alpha1-antitrypsin in patients with pulmonary emphysema.
        N Engl J Med. 1969; 281: 279
        • Kueppers F
        • Fallat R
        • Larson RK
        Obstructive lung disease and alpha1-antitrypsin deficiency gene heterozygosity.
        Science. 1969; 165: 899
        • Mittman C
        • Lieberman J
        • Rumsfeld J
        Prevalence of abnormal protease inhibitor phenotypes in patients with chronic obstructive lung disease.
        Am Rev Respir Dis. 1974; 109: 295
        • Stevens PM
        • Hirilica VS
        • Johnson PC
        • et al.
        Pathophysiology of heriditary emphysema.
        Ann Intern Med. 1971; 74: 672
        • Webb DR
        • Hyde RW
        • Schwartz RH
        • et al.
        Serum alpha1antitrypsin varients. Prevalence and clinical spirometry.
        Am Rev Respir Dis. 1973; 108: 918
        • Guenter CA
        • Welch MH
        • Ferguson S
        • et al.
        Alpha1-antitrypsin deficiency, Heterozygosity, intermediate levels, and pulmonary disease.
        Chest. 1971; 59: 16S
        • Morse JO
        • Lebowitz MD
        • Knudson RJ
        • et al.
        Alpha1-antitrypsin and obstructive disease—a community study.
        N Engl J Med. 1975; 292: 278
        • Klayton R
        • Fallat R
        • Cohen AB
        Determinants of chronic obstructive pulmonary disease in patients with intermediate levels of alpha1-antitrypsin.
        Am Rev Respir Dis. 1975; 112: 71
        • Welch MH
        • Reinecke ME
        • Hammarsten JF
        • et al.
        Antitrypsin deficiency in pulmonary disease. The significance of intermediate levels.
        Ann Intern Med. 1969; 71: 533
        • Lieberman J
        • Gaidulis L
        • Garoutte B
        • et al.
        Identification and characteristics of the common alpha1-antitrypsin phenotypes.
        Chest. 1972; 62: 557
        • Gelb AF
        • Molony P
        • Klein E
        • et al.
        Sensitivity of volume of isoflow in detection of mild airway obstruction.
        Am Rev Respir Dis. 1975; 112: 401
        • Hutcheon M
        • Griffin P
        • Levison H
        • et al.
        Volume of isoflow. A new test in detection of mild abnormalities of lung mechanics.
        Am Rev Respir Dis. 1974; 110: 458
        • Despas PH
        • LeRoux M
        • Macklem PT
        Site of airway obstruction in asthma as determined by measuring maximal expiratory flow breathing air and helium-oxygen mixture.
        J Clin Invest. 1972; 51: 3235
        • Dosman J
        • Bode F
        • Urbonetti J
        • et al.
        The use of a helium-oxygen mixture during maximum expiratory flow to demonstrate obstruction in small airways in smokers.
        J Clin Invest. 1975; 55: 1090
        • DuBois AB
        • Botelho SY
        • Bedell GN
        • et al.
        A rapid plethysmographic method for measuring thoracic gas volume' A comparison with a nitrogen washout method for measuring functional residual capacity in a normal subject.
        J Clin Invest. 1956; 35: 322
        • DuBois AB
        • Botelho SY
        • Comroe Jr, JH
        A new method for measuring airway resistance in man using a body plethysmograph. Values in normal subjects and in patients with respiratory disease.
        J Clin Invest. 1956; 35: 327
        • Wagner PD
        • Mazzone RW
        • West JB
        Diffusing capacity and anatomic dead space for carbon monoxide (C18O).
        J Appl Physiol. 1972; 31: 847
        • Milic-Emili J
        • Mead J
        • Turner JM
        • et al.
        Improved technique for estimating pleural pressure from esophageal balloons.
        J Appl Physiol. 1964; 19: 207
        • Turner JM
        • Mead J
        • Wohl ME
        Elasticity of human lungs in relation to age.
        J Appl Physiol. 1968; 25: 664
        • Woolcock AJ
        • Vincent NJ
        • Macklem PT
        Frequency dependence of compliance as a test for obstruction in the small airways.
        J Clin Invest. 1969; 48: 1097
        • Schlueter DP
        • Immekus J
        • Stead WJ
        Relationship between maximal inspiratory pressure and total lung capacity (coefficient of retraction) in normal subjects and in paitents with emphysema, asthma, and diffuse pulmonary infiltration.
        Am Rev Respir Dis. 1967; 107: 656
        • Gelb AF
        • Zamel N
        Simplified diagnosis of small airways obstruction.
        N Engl J Med. 1973; 288: 395
        • Buist AS
        • Ross BB
        Predicted values for closing volume using a modified single breath test.
        Am Rev Respir Dis. 1973; 107: 744
        • Goldman HI
        • Becklake MR
        Respiratory function tests. Normal values at medial altitudes and the prediction of normal results.
        Am Rev Tuberc Pul Dis. 1959; 79: 457
        • Morris JF
        • Koski A
        • Johnson LC
        Spirometric standards for healthy nonsmoking adults.
        Am Rev Respir Dis. 1971; 103: 57
        • VanGanse WF
        • Ferris BG
        • Cotes JE
        Cigarette smoking and pulmonary diffusing capacity (transfer factor).
        Am Rev Respir Dis. 1972; 105: 30
        • Cooper DM
        • Hoeppner V
        • Cox D
        • et al.
        Lung function in alpha1-antitrypsin heterozygotes (Pi type MZ).
        Am Rev Respir Dis. 1974; 110: 708
        • Shigeoka JW
        • Hall WJ
        • Hyde RW
        • et al.
        Intermediate alpha1antitrypsin deficiency; Relationship of environment to risk of lung disease.
        Clin Res. 1975; 23: 352A
        • Ostrow DN
        • Cherniack RM
        The mechanical properties of the lungs in intermediate deficiency of alpha1-antitrypsin.
        Am Rev Respir Dis. 1972; 106: 377
        • Mittman C
        • Lieberman J
        • Marasso F
        • et al.
        Smoking and chronic obstructive lung disease in alpha1-antitrypsin dificiency.
        Chest. 1971; 60: 214
        • Mead J
        • Turner JM
        • Macklem PT
        • et al.
        Significance of the relationship between lung recoil and maximum expiratory flow.
        J Appl Physiol. 1967; 22: 95
        • LaPierre JG
        • Ho MF
        • Zamel N
        • et al.
        Effects of age on volume of isoflow and its determinants in healthy persons.
        Am Rev Respir Dis. 1975; 111: 938
        • Otis AB
        • McKerrow CB
        • Bartlett PA
        • et al.
        Mechanical factors in distribution of pulmonary ventilation.
        J Appl Physiol. 1956; 5: 799
        • Niewoehner DE
        • Kleinerman J
        Effects of experimental emphysema and bronchiolitis on lung mechanics and morphometry.
        J Appl Physiol. 1973; 35: 25
        • Mead J
        Contribution of compliance of airways to frequency dependent behavior of lungs.
        J Appl Physiol. 1969; 26: 670
        • Gelb AF
        • Gold WM
        • Wright RR
        • et al.
        Physiologic diagnosis of subclinical emphysema.
        Am Rev Respir Dis. 1973; 107: 50
        • Fletcher CM
        • Tinker CM
        • Hill ID
        A five year prospective field study of early obstructive airway disease.
        in: 11th Aspen Conference. 2nd ed. U.S. Public Health Service Publication 1879. 1968: 249
        • Bates DV
        The fate of the chronic bronchitic. A report of the ten-year follow-up in the Canadian Department of Veteran's Affairs coordinated study of chronic bronchitis.
        Am Rev Respir Dis. 1973; 108: 1043
        • Lieberman J
        Involvement of leukocytic proteases in emphysema and antitrypsin deficiency.
        Arch Environ Health. 1973; 127: 196
        • Galdston M
        • Janoff A
        • Davis AL
        Familial variation of leukocyte lysosomal protease and serum alpha-1-antitrypsin as determinants in chronic obstructive pulmonary disease.
        Am Rev Respir Dis. 1973; 107: 718
        • Rodriguez JR
        • Seals JE
        • Radin A
        • et al.
        The role of leucocyte lysosomal elastase in the pathogenesis of obstructive lung disease.
        Clin Res. 1975; 23: 351A
        • Gelb AF
        • Zamel N
        Effect of aging of lung mechanics in healthy nonsmokers.
        Chest. 1975; 68: 538