Hereditary angioedema (HAE) in children and adolescents : a consensus on therapeutic strategies

  • Hereditary angioedema due to C1 inhibitor (C1 esterase inhibitor) deficiency (types I and II HAE-C1-INH) is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible is important to avoid ineffective therapies and to properly treat swelling attacks. At a consensus meeting in June 2011, pediatricians and dermatologists from Germany, Austria, and Switzerland reviewed the currently available literature, including published international consensus recommendations for HAE therapy across all age groups. Published recommendations cannot be unconditionally adopted for pediatric patients in German-speaking countries given the current approval status of HAE drugs. This article provides an overview and discusses drugs available for HAE therapy, their approval status, and study results obtained in adult and pediatric patients. Recommendations for developing appropriate treatment strategies in the management of HAE in pediatric patients in German-speaking countries are provided.Conclusion Currently, plasma-derived C1 inhibitor concentrate is considered the best available option for the treatment of acute HAE-C1-INH attacks in pediatric patients in German-speaking countries, as well as for short-term and long-term prophylaxis.

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Author:Volker WahnORCiDGND, Werner Aberer, Wolfgang Eberl, Maria Faßhauer, Thomas KühneGND, Karin KurnikGND, Markus Magerl, Dirk Meyer-Olson, Inmaculada Martinez SaguerGND, Peter J. Späth, Petra Staubach-Renz, Wolfhart Kreuz
Pubmed Id:
Parent Title (English):European journal of pediatrics
Publisher:Springer Science & Business Media B.V.
Place of publication:Berlin ; Dordrecht
Document Type:Article
Date of Publication (online):2012/04/29
Date of first Publication:2012/04/29
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2013/10/08
Tag:C1-INH (C1 inhibitor, C1-esterase inhibitor); Consensus; Hereditary angioedema; Pediatric
Page Number:10
First Page:1339
Last Page:1348
Copyright © The Author(s) 2012. Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.
Institutes:Medizin / Medizin
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - Namensnennung 3.0