Safety of biweekly α1-antitrypsin treatment in the RAPID programme. Issue 5 (29th November 2018)

• Record Type: Journal Article
• Authors: Greulich, Timm; Chlumsky, Jan; Wencker, Marion; Vit, Oliver; Fries, Michael; Chung, Thomas; Shebl, Amgad; Vogelmeier, Claus; Chapman, Kenneth R.; McElvaney, Noel G.
• Other Names: author non-byline.; Burdon J.G.W. author non-byline.; Edwards R. author non-byline.; Glanville A. author non-byline.; Holmes M. author non-byline.; Thompson P. author non-byline.; Philipps M. author non-byline.; Wark P.A. author non-byline.; Abboud R.T. author non-byline.; Chapman K.R. author non-byline.; Elwood R.K. author non-byline.; Hernandez P. author non-byline.; Chlumsky J. author non-byline.; Seersholm N. author non-byline.; Skjold T. author non-byline.; Altraja A. author non-byline.; Mäkitaro R. author non-byline.; Ficker J. author non-byline.; Herth J.F. author non-byline.; Schulze K. author non-byline.; Teschler H. author non-byline.; McElvaney N.G. author non-byline.; Chorostowska-Wynimko J. author non-byline.; Sanak M. author non-byline.; Szczeklik A. author non-byline.; Tomkowsk W.Z. author non-byline.; Stoicescu P.I. author non-byline.; Martynenko T. author non-byline.; Piitulainen E. author non-byline.; Campos M. author non-byline.; Craig T.J. author non-byline.; Sandhaus R.A. author non-byline.; Stocks J.M. author non-byline.;
• Is Part Of: European respiratory journal. Volume 52:Issue 5(2018)
• Publication Date: 2018-11-29
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.00897-2018
• Languages: English
• ISSNs: 0903-1936

Abstract

Α1 -antitrypsin (α1 -AT) deficiency is a hereditary disorder characterised by an abnormally low concentration of functional α1 -AT in blood and tissues [1]. The primary role of α1 -AT is to protect elastin-containing tissues, most notably the lung, against the destructive activity of proteolytic enzymes [2]. Patients with severe α1 -AT deficiency present with serum α1 -AT concentrations <11 μM and are prone to destruction of the lung tissue, often developing respiratory symptoms and emphysema in the fourth or fifth decade of life [3, 4]. Administration of 120 mg·kg −1 α1 -antitrypsin on a biweekly basis was safe and well tolerated http://ow.ly/CVbz30lUBum