Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Wahn, V; Aberer, W; Eberl, W; Fasshauer, M; Kuhne, T; Kurnik, K; Magerl, M; Meyer-Olson, D; Martinez-Saguer, I; Spath, P; Staubach-Renz, P; Kreuz, W; .
Hereditary angioedema (HAE) in children and adolescents-a consensus on therapeutic strategies.
Eur J Pediatr. 2012; 171(9):1339-1348
Doi: 10.1007/s00431-012-1726-4
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- Co-Autor*innen der Med Uni Graz
- Aberer Werner
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Adult -
- Androgens - therapeutic use
- Anti-Inflammatory Agents, Non-Steroidal - therapeutic use
- Antifibrinolytic Agents - therapeutic use
- Antifibrinolytic Agents -
- Bradykinin - analogs and derivatives Bradykinin - therapeutic use
- Child -
- Complement C1 Inactivator Proteins - therapeutic use
- Complement C1 Inhibitor Protein - therapeutic use
- Complement Inactivating Agents - therapeutic use
- Disease Progression -
- Disease Progression -
- Hereditary Angioedema Types I and II - drug therapy
- Humans -
- Peptides - therapeutic use
- Recombinant Proteins - therapeutic use
- Recombinant Proteins -
- Find related publications in this database (Keywords)
- C1-INH (C1 inhibitor, C1-esterase inhibitor)
- Hereditary angioedema
- Consensus
- Pediatric