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

Wahn, V; Aberer, W; Eberl, W; Faßhauer, M; Kühne, T; Kurnik, K; Magerl, M; Meyer-Olson, D; Martinez-Saguer, I; Späth, P; Staubach-Renz, P; Kreuz, W (2012). Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies. European journal of pediatrics, 171(9), pp. 1339-48. Berlin: Springer 10.1007/s00431-012-1726-4

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Pharmacology

UniBE Contributor:

Späth, Peter Julius

ISSN:

0340-6199

Publisher:

Springer

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:35

Last Modified:

17 Mar 2015 21:25

Publisher DOI:

10.1007/s00431-012-1726-4

PubMed ID:

22543566

Web of Science ID:

000307701100007

URI:

https://boris.unibe.ch/id/eprint/14098 (FactScience: 220906)

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