Biochemical Efficacy and Safety of Monthly Augmentation Therapy for α1-Antitrypsin Deficiency

JAMA: The Journal of the American Medical Association

Volumn 260, Issue 9, 1988, Pages 1259-1264

  Hubbard, R C ^a   Sellers, S ^a   Czerski, D ^a   Stephens, L ^a   Crystal, R G ^a  

^a NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA 1 ANTITRYPSIN; ELASTASE;

ADULT; ALPHA 1 ANTITRYPSIN DEFICIENCY; CLINICAL ARTICLE; DIAGNOSIS; DRUG BLOOD LEVEL; FEMALE; HEREDITY; HUMAN; INTRAVENOUS DRUG ADMINISTRATION; LUNG FUNCTION; MALE; NEUTROPHIL; PRIORITY JOURNAL;

ADULT; ALPHA 1-ANTITRYPSIN; ALPHA 1-ANTITRYPSIN DEFICIENCY; DRUG ADMINISTRATION SCHEDULE; FEMALE; HUMAN; INFUSIONS, INTRAVENOUS; LUNG; MALE; MIDDLE AGE; MODELS, BIOLOGICAL; PANCREATIC ELASTASE; PULMONARY EMPHYSEMA; TIME FACTORS;

EID: 0023784904     PISSN: 00987484     EISSN: 15383598     Source Type: Journal    
DOI: 10.1001/jama.1988.03410090091037     Document Type: Article

References (26)

1. Gadek JE, Crystal RG: α1-Antitrypsin deficiency, in Stanbury JB, Wyngaarden JB, Fredrickson DS, et al. (eds): Metabolic Basis of Inherited Disease. New York, McGraw-Hill International Book Co, 1982, pp 1450-1467.
2. Kueppers F, Black LF: α1-Antitrypsin and its deficiency. Am Rev Respir Dis 1974;110:176-194.
3. Morse JO: Alpha1-antitrypsin deficiency. N Engl J Med 1978;299:1045-1105.
4. Tobin MJ, Cook PJL, Hutchison DCS: Alpha1-antitrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. Br J Dis Chest 1983; 77:14-27.
5. Carrell RW, Owen MC: α1-Antitrypsin: Structure, variation and disease. Essays Med Biochem 1979;4:83-119.
6. Pierce JA, Eradio B, Tew DA: Antitrypsin phenotypes in St Louis. JAMA 1975;231:609-612.
7. Dykes DD, Miller SA, Polesky HF: Distribution of α1-antitrypsin variants. Adv Hum Genet 1981;11:1-62.
8. Gadek JE, Fells GA, Zimmerman RL, et al.: Antielastases of the human alveolar structures: Implications for the protease-antiprotease theory of emphysema. J Clin Invest 1981;68:889-898.
9. Wewers MD, Casolaro MA, Crystal RG: Comparison of alpha-1-antitrypsin levels and antineutrophil elastase capacity of blood and lung in a patient with the alpha-1-antitrypsin phenotype null-null before and during alpha-1-antitrypsin augmentation therapy. Am Rev Respir Dis 1987; 135:539-543.
10. Janoff A: Elastases and emphysema: Current assessment of the protease-antiprotease hypothesis. Am Rev Respir Dis 1985;132:417-433.
11. Wewers MD, Casolaro MA, Sellers SE, et al.: Replacement therapy for alpha1-antitrypsin deficiency associated with emphysema. N Engl J Med 1987;316:1055-1062.
12. Thurlbeck WM, Henderson JA, Fraser RG, et al.: Chronic obstructive lung disease: A comparison between clinical, roentgenologic, functional and morphologic criteria in chronic bronchitis, emphysema, asthma and bronchiectasis. Medicine 1980; 49:81-145.
13. Jeppsson J-O, Laurell C-B, Fagerhol M: Properties of isolated human α1-antitrypsin of Pi types M, S, and Z. Eur J Biochem 1978;83:143-153.
14. Straus SD, Fells GA, Wewers MD, et al.: Evaluation of recombinant DNA-directed E coli produced α1-antitrypsin as an anti-neutrophil elastase for potential use as replacement therapy of alantitrypsin deficiency. Biochem Biophys Res Commun 1985;130:1177-1184.
15. Rennard SI, Berg R, Martin GR, et al.: Enzyme-linked immunoassay (ELISA) for connective tissue components. Anal Biochem 1980;104: 205-214.
16. Rennard SI, Basset G, Lecossier D, et al.: Estimation of volume of epithelial lining fluid recovered by lavage using urea as a marker of dilution. J Appl Physiol 1986;60:532-538.
17. Ogushi F, Fells GA, Hubbard RC, et al.: Z-type α1-antitrypsin is less competent than M1-type α1-antitrypsin as an inhibitor of neutrophil elastase. J Clin Invest 1987;80:1366-1374.
18. Coan MH, Brockway WJ, Eguizabel H, et al.: Preparation and properties of alpha1-proteinase inhibitor concentrate from human plasma. Vox Sang 1985;48:333-342.
19. Gellis SS, Neefe JR, Stokes J Jr, et al.: Chemical, clinical and immunological studies on the products of human plasma fractionation: XXXVI. Inactivation of the virus of homologous serum hepatitis in solutions of normal human serum albumin by means of heat. J Clin Invest 1948;27:239-244.
20. Murray R, Diefenback WCL: Effect of heat on the agent of homologous serum hepatitis. Proc Soc Exp Med 1953;84:230-231.
21. Beatty K, Bieth J, Travis JL: Kinetics of association of serine proteinases with native and oxidized α-1-proteinase inhibitor and α-1-antichymotrypsin. J Biol Chem 1980;255:3931-3934.
22. Berman M, Weiss MF, Shahn E: Some formal approaches to the analysis of kinetic data in terms of linear compartmental systems. Biophys J 1962; 2:289-316.
23. Gadek JE, Klein HG, Holland PV, et al.: Replacement therapy of alpha1-antitrypsin deficiency: Reversal of protease-antiprotease imbalance within the alveolar structures of Pi Z subjects. J Clin Invest 1981;68:1158-1165.
24. Gadek JE, Fells GA, Wright DG, et al.: Human neutrophil elastase functions as a type III collagen ’collagenase.’ Biochem Biophys Res Commun 1980;95:1815-1822.
25. Saltini C, Hance AJ, Ferrans VJ, et al.: Accurate quantification of cells recovered by bronchoalveolar lavage. Am Rev Respir Dis 1984;10:650-658.
26. Hunninghake GW, Crystal RG: Cigarette smoking and lung destruction: Accumulation of neutrophils in the lungs of cigarette smokers. Am Rev Respir Dis 1983;128:833-838.