title: Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies.
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 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.
authors.affiliation: Department of Pediatric Pneumology and Immunology, Campus Virchow Hospital, Charité Medical University, Augustenburger Platz 1, 13353 Berlin, Germany. Volker.Wahn@charite.de
authors.forename: V
authors.initials: V
authors.lastname: Wahn
authors.forename: W
authors.initials: W
authors.lastname: Aberer
authors.forename: W
authors.initials: W
authors.lastname: Eberl
authors.forename: M
authors.initials: M
authors.lastname: Faßhauer
authors.forename: T
authors.initials: T
authors.lastname: Kühne
authors.forename: K
authors.initials: K
authors.lastname: Kurnik
authors.forename: M
authors.initials: M
authors.lastname: Magerl
authors.forename: D
authors.initials: D
authors.lastname: Meyer-Olson
authors.forename: I
authors.initials: I
authors.lastname: Martinez-Saguer
authors.forename: P
authors.initials: P
authors.lastname: Späth
authors.forename: P
authors.initials: P
authors.lastname: Staubach-Renz
authors.forename: W
authors.initials: W
authors.lastname: Kreuz
mesh_terms: D000293:Adolescent; D000328:Adult; D000728:Androgens / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001317:Austria; D001920:Bradykinin / Q000031:analogs & derivatives / Q000627:therapeutic use; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein / Q000627:therapeutic use*; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D018450:Disease Progression; D005858:Germany; D056829:Hereditary Angioedema Types I and II / Q000188:drug therapy*; D006801:Humans; D010455:Peptides / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use; D013557:Switzerland
chemical_list: D000728:Androgens; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000933:Antifibrinolytic Agents; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents; D010455:Peptides; D011994:Recombinant Proteins; C469952:SERPING1 protein, human; C511194:ecallantide; C571093:conestat alfa; C065679:icatibant; D001920:Bradykinin