Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ)

Thorax

Volumn 63, Issue 12, 2008, Pages 1091-1095

  Tanash, H A ^a,b   Nilsson, P M ^a   Nilsson, J A ^a   Piitulainen, E ^a  

^a LUND UNIVERSITY   (Sweden)

^b LUND UNIVERSITY HOSPITAL   (Sweden)

Author keywords

[No Author keywords available]

Indexed keywords

ADULT; AGED; ALPHA 1 ANTITRYPSIN DEFICIENCY; ARTICLE; CAUSE OF DEATH; DISEASE COURSE; EMPHYSEMA; FEMALE; FOLLOW UP; HUMAN; LIVER CANCER; LIVER CIRRHOSIS; MAJOR CLINICAL STUDY; MALE; MALIGNANT TRANSFORMATION; MORTALITY; PRIORITY JOURNAL; PROGNOSIS; RESPIRATORY TRACT DISEASE; SMOKING; SWEDEN;

EID: 57149107426     PISSN: 00406376     EISSN: 14683296     Source Type: Journal    
DOI: 10.1136/thx.2008.095497     Document Type: Article

References (22)

1. American Thoracic Society/European Respiratory Society. Statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003;168:818- 900.
2. Brandy M, Nukiwa T, Crystal RG. Molecular basis of alpha-1-antitrypsin deficiency. Am J Med 1988;84:13-31.
3. Cox DW, Billingsley GD, Mansfield T. DNA restriction-site polymorphisms associated with the alpha 1-antitrypsin gene. Am J Hum Genet 1987;41:891-906.
4. Eriksson S. Studies in alpha 1-antitrypsin deficiency. Acta Med Scand Suppl 1965;432:1-85.
5. Tobin MJ, Cook PJ, Hutchison DC. 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;77:14-27.
6. Larsson C. Natural history and life expectancy in severe alpha-1-antitrypsin deficiency, Pi Z. Acta Med Scand 1978;204:345- 51.
7. Sharp H. Alpha-one antitrypsin deficiency. Hosp Pract 1971;6:83.
8. Sveger T. Liver disease in alpha 1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med 1976;294:1316-21.
9. Eriksson S, Carlson J, Velez R. Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency. N Engl J Med 1986;314:736-9.
10. Elzouki AN, Eriksson S. Risk of hepatobiliary disease in adults with severe alpha 1-antitrypsin deficiency (PiZZ): is chronic viral hepatitis B or C an additional risk factor for cirrhosis and hepatocellular carcinoma? Eur J Gastroenterol Hepatol 1996;8:989-94.
11. Eriksson S. Alpha-1 antitrypsin deficiency: natural course and therapeutic strategies. In: Boyer J, Blum HE, Maier KP, et al., eds Cirrhosis and its development. Falk Symposium 115. Dordrecht, The Netherlands: Kluwer Academic, 2000:305-13.
12. Piitulainen E, Tornling G, Eriksson S. Effect of age and occupational exposure to airway irritants on lung function in non-smoking individuals with alpha 1-antitrypsin deficiency (PiZZ). Thorax 1997;52:244-8.
13. Seersholm N, Kok-Jensen A, Dirksen A. Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases. Thorax 1994;49:695-8.
14. Seersholm N, Kok-Jensen A. Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency. Thorax 1998;53:265-8.
15. Stockley BA, Luisetti M. Miravitlles M, et al. Alpha-1-antitrypsin deficiency International Registry group. Ongoing research in Europe: Alpha-1 -antitrypsin deficiency International Registry (AIR) objectives and development. Eur Respir J 2007;29:582-6.
16. Jeppsson JO, Franzen B. Typing of genetic variants of alpha 1-antitrypsin by electrofocusing. Clin Chem 1982;28:219-25.
17. Quanjer P. Standardized lung function testing. Official statement of the European Respiratory Society. Eur Respir J Suppl 1993;16:1-100.
18. Statistics Sweden (Bureau of Statistics in Stockholm). 1991-2006 www.ssd.scb.se.
19. Black LF, Kueppers F. Alpha-1-Antitrypsin deficiency in nonsmokers. Am Rev Respir Dis 1978;117:421-8.
20. Stoller JK, Tomashefski J Jr, Crystal RG, et al. Mortality in individuals with severe deficiency of alpha1-antitrypsin: findings from the National Heart, Lung, and Blood Institute Registry. Chest 2005;127:1196-204.
21. Bemspang E, Sveger T, Piitulainen E. Respiratory symptoms and lung function in 30-year-old individuals with alpha-1-antitrypsin deficiency. Respir Med 2007;101:1971-6.
22. Silverman EK, Pierce JA, Province MA, et al. Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates. Ann Intern Med 1989;111:982-91.