Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases
N Seersholm, A Kok-Jensen, A Dirksen, N Seersholm, A Kok-Jensen, A Dirksen
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.
References
- Hum Hered. 1990;40(6):340-55
- Br J Dis Chest. 1983 Jan;77(1):14-27
- Ann Dermatol Venereol. 1988;115(10):1017-21
- Pediatrics. 1978 Jul;62(1):22-5
- Dan Med Bull. 1992 Feb;39(1):30-48
- Am Rev Respir Dis. 1988 Aug;138(2):327-36
- Br J Cancer. 1977 Jan;35(1):1-39
- Am J Med. 1988 Jun 24;84(6A):13-31
- Acta Med Scand. 1978;204(5):345-51
- Acta Med Scand Suppl. 1965;432:1-85
- Acta Paediatr Scand. 1988 Nov;77(6):847-51
- Am Rev Respir Dis. 1974 Aug;110(2):176-94
- J Chronic Dis. 1958 Dec;8(6):699-712
- Ann Intern Med. 1989 Dec 15;111(12):982-91
- Am Rev Respir Dis. 1983 Feb;127(2):S43-5
- Adv Hum Genet. 1981;11:1-62, 371-2
- Respiration. 1986;50 Suppl 1:56-73
Source: PubMed