dc.contributor.author
Wahn, Volker
dc.contributor.author
Aberer, Werner
dc.contributor.author
Aygören‐Pürsün, Emel
dc.contributor.author
Bork, Konrad
dc.contributor.author
Eberl, Wolfgang
dc.contributor.author
Faßhauer, Maria
dc.contributor.author
Krüger, Renate
dc.contributor.author
Magerl, Markus
dc.contributor.author
Martinez‐Saguer, Inmaculada
dc.contributor.author
Späth, Peter
dc.contributor.author
Staubach‐Renz, Petra
dc.contributor.author
Weber‐Chrysochoou, Christina
dc.date.accessioned
2022-03-10T12:18:37Z
dc.date.available
2022-03-10T12:18:37Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/34341
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-34058
dc.description.abstract
Background/Methods:
At a consensus meeting in August 2018, pediatricians and dermatologists from German-speaking countries discussed the therapeutic strategy for the treatment of pediatric patients with type I and II hereditary angioedema due to C1 inhibitor deficiency (HAE-C1-INH) for Germany, Austria, and Switzerland, taking into account the current marketing approval status. 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 and an optimal management of the disease are important to avoid ineffective therapies and to properly treat swelling attacks. This article provides recommendations for developing appropriate treatment strategies in the management of HAE-C1-INH in pediatric patients in German-speaking countries. An overview of available drugs in this age-group is provided, together with their approval status, and study results obtained in adults and pediatric patients.
Results/Conclusion:
Currently, plasma-derived C1 inhibitor concentrates have the broadest approval status and are considered the best available option for on-demand treatment of HAE-C1-INH attacks and for short- and long-term prophylaxis across all pediatric age-groups in German-speaking countries. For on-demand treatment of children aged 2 years and older, recombinant C1-INH and bradykinin-receptor antagonist icatibant are alternatives. For long-term prophylaxis in adolescents, the parenteral kallikrein inhibitor lanadelumab has recently been approved and can be recommended due to proven efficacy and safety.
en
dc.rights.uri
https://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subject
C1-INH (C1 inhibitor)
en
dc.subject
hereditary angioedema
en
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit::610 Medizin und Gesundheit
dc.title
Hereditary angioedema in children and adolescents – A consensus update on therapeutic strategies for German‐speaking countries
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation.doi
10.1111/pai.13309
dcterms.bibliographicCitation.journaltitle
Pediatric Allergy and Immunology
dcterms.bibliographicCitation.number
8
dcterms.bibliographicCitation.originalpublishername
Wiley
dcterms.bibliographicCitation.pagestart
974
dcterms.bibliographicCitation.pageend
989
dcterms.bibliographicCitation.volume
31
refubium.affiliation
Charité - Universitätsmedizin Berlin
refubium.funding
DEAL Wiley
refubium.resourceType.isindependentpub
no
dcterms.accessRights.openaire
open access
dcterms.bibliographicCitation.pmid
32524650
dcterms.isPartOf.issn
0905-6157
dcterms.isPartOf.eissn
1399-3038