Alpha-1 antitrypsin: Now available, but do we need it?

Swiss Medical Weekly

Volumn 138, Issue 13-14, 2008, Pages 191-196

  Russi, Erich W ^a  

^a UNIVERSITY HOSPITAL ZURICH   (Switzerland)

Author keywords

Pulmonary emphysema; Replacement therapy; 1 antitrypsin deficiency

Indexed keywords

ALPHA 1 ANTITRYPSIN; LEUKOCYTE ELASTASE; PLACEBO;

ALPHA 1 ANTITRYPSIN DEFICIENCY; ANAPHYLAXIS; CLINICAL TRIAL; DIAGNOSTIC PROCEDURE; DISEASE SEVERITY; DRUG EFFECT; DRUG TOLERABILITY; EMPHYSEMA; ENZYME ACTIVITY; ENZYME INHIBITION; ENZYME REPLACEMENT; EPIDEMIOLOGICAL DATA; GENE FREQUENCY; GENETIC DISORDER; HEPATITIS; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; INCIDENCE; LUNG; LUNG ALVEOLUS; LUNG EMPHYSEMA; LUNG FLUID; LUNG FUNCTION; MORBIDITY; NONHUMAN; PRION DISEASE; PROTEIN FUNCTION; RISK FACTOR; SHORT SURVEY; SMOKING; SURVIVAL RATE;

EID: 42149182337     PISSN: 14247860     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Short Survey

References (26)

1. 1-antitrypsin deficiency. Scan J Clin Lab Invest. 1963;132-40.
2. 1-antitrypsin deficiency. Acta Med Scand. 1965;S432:1-85.
3. 1-antitrypsin deficiency - a model for conformational diseases. N Engl J Med. 2002;346:45-53.
4. 1-antitrypsin polymerisation and the serpinopathies: pathobiology and prospects for therapy. J Clin Invest. 2002;110:1585-90.
5. De Serres FJ, Blanco I, Fernandez-Bustillo E. Estimated numbers and prevalence of PI*S and PI*Z deficiency alleles of alpha1-antitrypsin deficiency in Asia. Eur Respir J. 2006;27:77-84.
6. 1-antitrypsin deficiency. Thorax. 2004;59:164-9.
7. Sveger T. Liver disease in alpha-1 antitrypsin deficiency detected by screening of 200 000 infants. N Engl J Med. 1976;294:1316-21.
8. O'Brien ML, Buist NR, Murphey WH. Neonatal screening for alpha 1 antitrypsin deficiency. J Pediatr. 1978;92:1006-10.
9. Lieberman J, Winter B, Sastre A. Alpha 1-antitrypsin Pi-types in 965 COPD patients. Chest. 1986;89:370-3.
10. 1-antitrypsin deficiency - clinical manifestations and natural history. Thorax. 2004;59:441-5.
11. Dowson LJ, Guest PJ, Stockley RA. The relationship of chronic sputum expectoration to physiologic, radiologic, and health status characteristics in alpha(1)-antitrypsin deficiency (Pi Z). Chest. 2002;122:1247-55.
12. Piitulainen E, Eriksson S. Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (Pi ZZ). Eur Respir J. 1999;13:247-51.
13. Seersholm N, Kok-Jensen A, Dirksen A. Decline in FEV1 among patients with severe hereditary alpha 1-antitrypsin deficiency type Pi Z. Am J Respir Crit Care Med. 1995;152:1922-5.
14. 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.
15. Sveger T, Piitulainen E, Arborelius M Jr. Lung function in adolescents with alpha-1-antitrypsin deficiency. Acta Paediatr. 1994;83:1170-93.
16. Larsson C. Natural history and life expectancy in severe alpha-1 deficiency, PiZ. Acta Med Scand. 1978;204:345-51.
17. 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.
18. http://www.goldcopd.com/
19. Tutic M, Bloch KE, Lardinois D, Brack T, Russi EW, Weder W. Long-term results after lung volume reduction surgery in patients with alpha1-antitrypsin deficiency. J Thorac Cardiovasc Surg. 2004;128:408-13.
20. Tutic M, Lardinois D, Imfeld S, Korom S, Boehler A, Speich R, et al. Lung-volume reduction surgery as an alternative or bridging procedure to lung transplantation. Ann Thorac Surg. 2006;82(1):208-13.
21. Wewers MD, Casolaro MA, Sellers SE, Swayze SC, McPhaul KM, Wittes JT, et al. Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema. N Engl J Med. 1987;316:1055-62.
22. Seersholm N, Wencker M, Banik N, Viskum K, Dirksen A, Kok-Jensen A, Konietzko N. Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group. Eur Respir J. 1997;10:2260-3.
23. 1- Antitrypsin. Am J Respir Crit Care Med. 1998;158:49-59.
24. Lieberman J. Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency. A new hypothesis with supporting data. Chest. 2000;118:1480-5.
25. Wencker M, Fuhrmann B, Banik N, Konietzko N. Longitudinal Follow-up of Patients With 1-Protease Inhibitor Deficiency Before and During Therapy With IV 1-Protease Inhibitor Chest. 2001;119:737-44.
26. Dirksen A, Dijkman JH, Madsen F, Stoel B, Hutchison DCS, Ulrik CS, et al. A Randomized Clinical Trial of 1-Antitrypsin Augmentation Therapy Am J Respir Crit Care Med. 1999;160:1468-72.