722
Views
32
CrossRef citations to date
0
Altmetric
ORIGINAL RESEARCH

The Clinical Profile of Subjects Included in the Swedish National Register on Individuals with Severe Alpha 1-Antitrypsin deficiency

&

References

  • 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.
  • Sveger T. Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med 1976; 294:1316–1321.
  • 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; 52:244–248.
  • WHO. α1-Antitrypsin deficiency: Memorandum from a WHO meeting. Bull World Health Organ 1997; 75:397–415.
  • Stockley RA, Luisetti M, Miravitlles M, Piitulainen E, Fernandez P. 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; 29:582–586.
  • Jeppsson JO, Franzen B. Typing of genetic variants of alpha 1-antitrypsin by electrofocusing. Clin Chem 1982; 28:219–225.
  • Quanjer P. Standardized lung function testing. Report working party. Bull Eur Physiopathol Respir 1983; 19(Suppl 5):1–95.
  • 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; 49:695–698.
  • McElvaney NG, Stoller JK, Buist AS, Prakash UB, Brantly ML, Schluchter MD, Crystal RD. Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Alpha 1-Antitrypsin Deficiency Registry Study Group. Chest 1997; 111:394–403.
  • Hutchison DCS. α1-antitrypsin deficiency in Europe: geographical distribution of Pi types S and Z. Respir Med 1998; 92:367–377.
  • Stoller JK. Clinical features and natural history of severe α1-antitrypsin deficiency. Roger S Mitchell Lecture. Chest 1997; 111:123–128.
  • Elzouki A-N, Segelmark M, Wieslander J, Eriksson S. Strong link between the alpha1-antitrypsin pizza allele and Wegener's granulomatosis. J Intern Med 1994; 236:543–548.
  • Segelmark M, Elzouki A-N, Wieslander J, Eriksson S. The PiZZ gene of α1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis. Kidney International 1995; 48:844–850.
  • Tanash HA, Nilsson PM, Nilsson JA, Piitulainen E. Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ). Thorax 2008; 63:
1091–1095.
  • Tanash HA, Nilsson PM, Nilsson JA, Piitulainen E. Survival in severe alpha-1-antitrypsin deficiency (PiZZ). Respir Res 2010; 11:44.
  • Dirksen A, Dijkman JH, Madsen F, Stoel B, Hutchison DCS, Ulrik CS, Skovgaard LT, Kok-Jensen A, Rudolphus A, Seersholm N, Vrooman HA, Reiber JHC, Hansen NC, Heckscher T, Viskum K, Stolk J. A randomized clinical trial of alpha-1 antitrypsin augmentation therapy. Am J Respir Crit Care Med 1999; 160:1468–1472.
  • Dirksen A, Piitulainen E, Parr DG, Deng C, Wencker M, Shaker SB, Stockley RA. Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha-1 antitrypsin deficiency. Eur Respir J 2009; 33:1345–1353.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.