TY - JOUR A1 - Wahn, Volker A1 - Aberer, Werner A1 - Eberl, Wolfgang A1 - Faßhauer, Maria A1 - Kühne, Thomas A1 - Kurnik, Karin A1 - Magerl, Markus A1 - Meyer-Olson, Dirk A1 - Martinez Saguer, Inmaculada A1 - Späth, Peter J. A1 - Staubach-Renz, Petra A1 - Kreuz, Wolfhart T1 - Hereditary angioedema (HAE) in children and adolescents : a consensus on therapeutic strategies T2 - European journal of pediatrics N2 - 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. KW - C1-INH (C1 inhibitor, C1-esterase inhibitor) KW - Hereditary angioedema KW - Consensus KW - Pediatric Y1 - 2012 UR - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/31721 UR - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-317211 SN - 1432-1076 SN - 0340-6199 N1 - 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. VL - 171 SP - 1339 EP - 1348 PB - Springer Science & Business Media B.V. CY - Berlin ; Dordrecht ER -