Skip to main content
PMC home page
Thorax logoLink to Thorax
. 1994 Jul;49(7):695–698. doi: 10.1136/thx.49.7.695

Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases.

N Seersholm 1, A Kok-Jensen 1, A Dirksen 1
PMCID: PMC475060  PMID: 8066566

Abstract

BACKGROUND--Previous estimates of the survival times of patients with alpha 1-antitrypsin deficiency have been based on selected patients. METHODS--The survival times of 397 patients with severe alpha 1-antitrypsin deficiency identified by pulmonary impairment (index cases) or through family studies (non-index cases) were compared. RESULTS--The overall median survival time was 54.5 years with no significant difference between men and women. Survival for index cases was less than for the non-index cases regardless of smoking history (49.4 years and 69.3 years respectively). When index and non-index cases were analysed separately there was no difference between the survival of smokers and never smokers in the index group. In the non-index group smokers had a shorter survival time than never smokers. The survival time of never smokers was similar to that of the normal Danish population. CONCLUSIONS--The prognosis of severe alpha 1-antitrypsin deficiency is better than previously assumed and, although smoking is a major risk factor, the development of emphysema in patients with severe alpha 1-antitrypsin deficiency is multifactorial.

Full text

PDF
696

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Brantly M. L., Paul L. D., Miller B. H., Falk R. T., Wu M., Crystal R. G. Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis. 1988 Aug;138(2):327–336. doi: 10.1164/ajrccm/138.2.327. [DOI] [PubMed] [Google Scholar]
  2. Brantly M., Nukiwa T., Crystal R. G. Molecular basis of alpha-1-antitrypsin deficiency. Am J Med. 1988 Jun 24;84(6A):13–31. doi: 10.1016/0002-9343(88)90154-4. [DOI] [PubMed] [Google Scholar]
  3. Buist A. S., Burrows B., Eriksson S., Mittman C., Wu M. The natural history of air-flow obstruction in PiZ emphysema. Report of an NHLBI workshop. Am Rev Respir Dis. 1983 Feb;127(2):S43–S45. doi: 10.1164/arrd.1983.127.2P2.S43. [DOI] [PubMed] [Google Scholar]
  4. CUTLER S. J., EDERER F. Maximum utilization of the life table method in analyzing survival. J Chronic Dis. 1958 Dec;8(6):699–712. doi: 10.1016/0021-9681(58)90126-7. [DOI] [PubMed] [Google Scholar]
  5. Eriksson S. Studies in alpha 1-antitrypsin deficiency. Acta Med Scand Suppl. 1965;432:1–85. [PubMed] [Google Scholar]
  6. Fagerhol M. K., Cox D. W. The Pi polymorphism: genetic, biochemical, and clinical aspects of human alpha 1-antitrypsin. Adv Hum Genet. 1981;11:1-62, 371-2. [PubMed] [Google Scholar]
  7. Hubbard R. C., Crystal R. G. Antiproteases and antioxidants: strategies for the pharmacologic prevention of lung destruction. Respiration. 1986;50 (Suppl 1):56–73. doi: 10.1159/000195089. [DOI] [PubMed] [Google Scholar]
  8. Kueppers F., Black L. F. Alpha1-antitrypsin and its deficiency. Am Rev Respir Dis. 1974 Aug;110(2):176–194. doi: 10.1164/arrd.1974.110.2.176. [DOI] [PubMed] [Google Scholar]
  9. Lange P. Development and prognosis of chronic obstructive pulmonary disease with special reference to the role of tobacco smoking. An epidemiologic study. Dan Med Bull. 1992 Feb;39(1):30–48. [PubMed] [Google Scholar]
  10. Larsson C. Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. Acta Med Scand. 1978;204(5):345–351. doi: 10.1111/j.0954-6820.1978.tb08452.x. [DOI] [PubMed] [Google Scholar]
  11. Peto R., Pike M. C., Armitage P., Breslow N. E., Cox D. R., Howard S. V., Mantel N., McPherson K., Peto J., Smith P. G. Design and analysis of randomized clinical trials requiring prolonged observation of each patient. II. analysis and examples. Br J Cancer. 1977 Jan;35(1):1–39. doi: 10.1038/bjc.1977.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Silverman E. K., Pierce J. A., Province M. A., Rao D. C., Campbell E. J. Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates. Ann Intern Med. 1989 Dec 15;111(12):982–991. doi: 10.7326/0003-4819-111-12-982. [DOI] [PubMed] [Google Scholar]
  13. Silverman E. K., Province M. A., Campbell E. J., Pierce J. A., Rao D. C. Variability of pulmonary function in alpha-1-antitrypsin deficiency: residual family resemblance beyond the effect of the Pi locus. Hum Hered. 1990;40(6):340–355. doi: 10.1159/000153958. [DOI] [PubMed] [Google Scholar]
  14. Sveger T. The natural history of liver disease in alpha 1-antitrypsin deficient children. Acta Paediatr Scand. 1988 Nov;77(6):847–851. doi: 10.1111/j.1651-2227.1988.tb10767.x. [DOI] [PubMed] [Google Scholar]
  15. Sveger T. alpha 1-antitrypsin deficiency in early childhood. Pediatrics. 1978 Jul;62(1):22–25. [PubMed] [Google Scholar]
  16. Tobin M. J., Cook P. J., Hutchison D. C. Alpha 1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest. 1983 Jan;77(1):14–27. doi: 10.1016/0007-0971(83)90002-5. [DOI] [PubMed] [Google Scholar]
  17. Vaillant L., Weisbecker M. C., de Muret A., Lorette G. Pemphigus auto-immun associé à un déficit en alpha 1 antitrypsine. Ann Dermatol Venereol. 1988;115(10):1017–1021. [PubMed] [Google Scholar]

Articles from Thorax are provided here courtesy of BMJ Publishing Group

RESOURCES