Orphanet J Rare Dis 2018 Apr 10;13(1):51. Epub 2018 Apr 10.
Allergy Department, Hospital La Paz Institute for Health Research, Madrid, Spain.
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Eur J Dermatol 2017 Apr;27(2):155-159
National Reference Centre for Angioedema, CREAK, France, Department of Internal Medicine, Saint Antoine University Hospital, AP-HP, Paris, France.
Hereditary angioedema (HAE) is a rare genetic disorder characterised by episodes of swelling without urticaria. Berinert® (CSL Behring) is a plasma-derived human C1 inhibitor (C1-INH) concentrate, approved for the treatment of HAE with C1-INH deficiency (C1-INH-HAE), however, it is often used off-label in Europe to treat HAE with normal C1-INH. To report the clinical characteristics of patients with HAE with normal C1-INH (with F12 gene mutation; FXII-HAE) or of unknown origin (U-HAE), and their response to Berinert®. Read More
Allergy 2011 Dec 2;66(12):1604-11. Epub 2011 Sep 2.
Penn State University College of Medicine, Hershey, PA 17033, USA.
Background: The placebo-controlled study International Multicentre Prospective Angioedema C1-INH Trial 1 (I.M.P. Read More
Immunotherapy 2014 17;6(5):533-51. Epub 2014 Mar 17.
Department of Dermatology, University Medical Center, Langenbeckstr 1, 55131 Mainz, Germany.
Hereditary angioedema (HAE) is a relatively rare autosomal dominant disorder that is typically characterized by recurrent episodes of edema in various body locations. It is most commonly caused by an inherited deficiency of functionally active C1 esterase inhibitor (C1-INH). Replacement therapy with a human plasma-derived C1-INH concentrate is recommended for the treatment and prophylaxis of acute attacks of HAE due to C1-INH deficiency (HAE-C1-INH). Read More
Drugs Aging 2016 11;33(11):819-827
CSL Behring, 1020 First Ave, King of Prussia, PA, 19406, USA.
Background: Treatment of hereditary angioedema (HAE) in 'older adults' (those aged ≥65 years) has not been well studied. The international Berinert Patient Registry collected data on the use of intravenous plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH; Berinert/CSL Behring) in patients of any age, including many older adults.
Methods: This observational registry, conducted from 2010 to 2014 at 30 US and seven European sites, gathered prospective (post-enrollment) and retrospective (pre-enrollment) usage and adverse event (AE) data on subjects treated with pnfC1-INH. Read More