The DOI matches its Crossref record; 30 of the 30 references it cites were found in a registry or an index.
Replacement Therapy for Alpha1-Antitrypsin Deficiency Associated with Emphysema
Abstract
In patients with alpha 1-antitrypsin deficiency, the development of emphysema is believed to be caused by the unchecked action of proteases on lung tissue. We evaluated the feasibility, safety, and biochemical efficacy of intermittent infusions of alpha 1-antitrypsin in the treatment of patients with alpha 1-antitrypsin deficiency. Twenty-one patients were given 60 mg of active plasma-derived alpha 1-antitrypsin per kilogram of body weight, once a week for up to six months. After a steady state had been reached, the group had trough serum levels of alpha 1-antitrypsin of 126 +/- 1 mg per deciliter as compared with 30 +/- 1 mg per deciliter before treatment, and serum anti-neutrophil elastase capacities of 13.3 +/- 0.1 microM as compared with 5.4 +/- 0.1 microM. The alpha 1-antitrypsin level in the epithelial-lining fluid of the lungs was 0.46 +/- 0.16 microM before treatment, and the anti-neutrophil elastase capacity was 0.81 +/- 0.13 microM. Six days after infusion, alpha 1-antitrypsin levels (1.89 +/- 0.17 microM) and anti-neutrophil elastase capacities (1.65 +/- 0.13 microM) in the lining fluid were significantly increased (P less than 0.0001). Because of the chronicity of the disorder and the lack of sensitive measures of lung destruction, the clinical efficacy of this therapy could not be studied rigorously. No changes in lung function were observed in our patients over six months of treatment. The only important adverse reactions to the 507 infusions were four episodes of self-limited fever. This study demonstrates that infusions of alpha 1-antitrypsin derived from plasma are safe and can reverse the biochemical abnormalities in serum and lung fluid that characterize this disorder. Together with lifetime avoidance of cigarette smoking, replacement therapy with alpha 1-antitrypsin may be a logical approach to long-term medical treatment.
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Cites
Diffusion-In-Gel Methods for Immunological Analysis II (Part 1 of 4)
Ouchterlony, Ö. (1962) · Chemical Immunology and Allergy · doi:10.1159/000313795 · Cited by 2,973
HUMAN PLASMA PROTEINASE INHIBITORS
Travis, J., & Salvesen, G. S. (1983) · Annual Review of Biochemistry · doi:10.1146/annurev.bi.52.070183.003255 · Cited by 2,104
The Electrophoretic α; 1 -Globulin Pattern of Serum in α; 1 -Antitrypsin Deficiency
Laurell, C.-B., & Eriksson, S. (1963) · Scandinavian Journal of Clinical and Laboratory Investigation · doi:10.1080/00365516309051324 · Cited by 1,393
Estimation of volume of epithelial lining fluid recovered by lavage using urea as marker of dilution
Rennard, S. I., Basset, G., Lecossier, D., O'Donnell, K. M., Pinkston, P., Martin, P. G., & Crystal, R. G. (1986) · Journal of Applied Physiology · doi:10.1152/jappl.1986.60.2.532 · Cited by 1,131
Self-citationLiver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 Infants
Sveger, T. (1976) · New England Journal of Medicine · doi:10.1056/nejm197606102942404 · Cited by 796
Beatty, K., Bieth, J., & Travis, J. (1980) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)85615-6 · Cited by 756
Structure and variation of human α1–antitrypsin
Carrell, R. W., Jeppsson, J.-O., Laurell, C.-B., Brennan, S. O., Owen, M. C., Vaughan, L., & Boswell, D. R. (1982) · Nature · doi:10.1038/298329a0 · Cited by 617
Enzyme-linked immunoassay (ELISA) for connective tissue components
Rennard, S. I., Berg, R., Martin, G. R., Foidart, J. M., & Robey, P. G. (1980) · Analytical Biochemistry · doi:10.1016/0003-2697(80)90300-0 · Cited by 605
Natural History and Life Expectancy in Severe Alpha1‐Antitrypsin Deficiency, Pi Z
Larsson, C. (1978) · Acta Medica Scandinavica · doi:10.1111/j.0954-6820.1978.tb08452.x · Cited by 484
Pulmonary Emphysema and Alpha1‐Antitrypsin Deficiency
ERIKSSON, S. (1964) · Acta Medica Scandinavica · doi:10.1111/j.0954-6820.1964.tb00567.x · Cited by 459
Cited by
Survival and FEV 1 Decline in Individuals with Severe Deficiency of α 1 -Antitrypsin
The Alpha-1-Antitrypsin Deficiency Registry Study Group (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.158.1.9712017 · Cited by 435
Therapies for rare diseases: therapeutic modalities, progress and challenges ahead
Tambuyzer, E., Vandendriessche, B., Austin, C. P., Brooks, P. J., Larsson, K., Miller Needleman, K. I., Valentine, J., Davies, K., Groft, S. C., Preti, R., et al. (2020) · Nature Reviews Drug Discovery · doi:10.1038/s41573-019-0049-9 · Cited by 357
McElvaney, N. G., Nakamura, H., Birrer, P., Hébert, C. A., Wong, W. L., Alphonso, M., Baker, J. B., Catalano, M. A., & Crystal, R. G. (1992) · Journal of Clinical Investigation · doi:10.1172/jci115994 · Cited by 299
Self-citationTaggart, C., Cervantes-Laurean, D., Kim, G., McElvaney, N. G., Wehr, N., Moss, J., & Levine, R. L. (2000) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)61505-x · Cited by 269
Pastore, N., Blomenkamp, K., Annunziata, F., Piccolo, P., Mithbaokar, P., Maria Sepe, R., Vetrini, F., Palmer, D., Ng, P., Polishchuk, E., et al. (2013) · EMBO Molecular Medicine · doi:10.1002/emmm.201202046 · Cited by 142
Metadata
- Authors
- Wewers, M. D., Casolaro, M. A., Sellers, S. E., Swayze, S. C., McPhaul, K. M., Wittes, J. T., & Crystal, R. G.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1987-04-23
- Volume
- 316
- Issue
- 17
- Pages
- 1055-1062
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2323886210
- Cited by
- 641
- References
- 30
- FWCI
- 40.19
- Type
- journal article
- Language
- en
- Open access
- closed
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- Wewers, M. D., Casolaro, M. A., Sellers, S. E., Swayze, S. C., McPhaul, K. M., Wittes, J. T., & Crystal, R. G. (1987). Replacement Therapy for Alpha1-Antitrypsin Deficiency Associated with Emphysema. New England Journal of Medicine. https://doi.org/10.1056/nejm198704233161704
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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