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Eur J Pediatr (2012) 171:13391348 DOI 10.1007/s00431-012-1726-4
ORIGINAL ARTICLE
Hereditary angioedema (HAE) in childrenand adolescentsa consensus on therapeutic strategies
V. Wahn & W. Aberer & W. Eberl & M. Fahauer &
T. Khne & K. Kurnik & M. Magerl & D. Meyer-Olson &
I. Martinez-Saguer & P. Spth & P. Staubach-Renz &
W. Kreuz
Received: 22 December 2011 /Accepted: 20 March 2012 /Published online: 29 April 2012 # The Author(s) 2012. This article is published with open access at Springerlink.com
Abstract 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
V. Wahn (*)
Department of Pediatric Pneumology and Immunology, Campus Virchow Hospital, Charit Medical University, Augustenburger Platz 1,13353 Berlin, Germanye-mail: [email protected]
W. AbererDepartment of Dermatology and Venereology, Medical University of Graz,Graz, Austria
W. EberlDepartment of Pediatrics, City Hospital, Braunschweig, Germany
M. FahauerDivision of Pediatrics, Department of Pediatric Rheumatology, Immunology and Infectiology, Municipal Hospital St. Georg, Leipzig, Germany
T. KhneUniversity Childrens Hospital, Basel, Switzerland
K. KurnikDr. von Haunersches Childrens Hospital,Inner City Medical Center of the Ludwig Maximilian University, Munich, Germany
M. MagerlAllergy Center, Charit Medical University, Berlin, Germany
D. Meyer-OlsonDepartment of Immunology and Rheumatology, Hannover Medical School,Hannover, Germany
I. Martinez-Saguer : W. KreuzJohann Wolfgang Goethe University Hospital, Frankfurt am Main, Germany
P. SpthInstitute of Pharmacology, University of Bern, Bern, Switzerland
P. Staubach-RenzDepartment of Dermatology, University Hospital Mainz, Mainz, Germany
1340 Eur J Pediatr (2012) 171:13391348
is considered the best available option for the treatment...