Hereditary angioedema (HAE) in children and adolescents. Consensus on therapeutic strategies

作者：Wahn V^*; Aberer W; Eberl W; Fasshauer M; Kuehne T; Kurnik K; Magerl M; Meyer Olson D; Martinez Saguer I; Spaeth P; Staubach Renz P; Kreuz W

来源：Monatsschrift Kinderheilkunde, 2012, 160(8): 774-781.

DOI：10.1007/s00112-011-2602-4

• C1-inhibitor
• C1 esterase inhibitor
• Hereditary angioedema
• Consensus
• Pediatrics

Hereditary angioedema due to C1-inhibitor deficiency (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 the edema. At a consensus meeting in June 2011, pediatricians and dermatologists from Germany, Austria and Switzerland reviewed the literature, including published international consensus recommendations for HAE therapy across all age groups. These recommendations cannot be unconditionally adopted for pediatric patients in German-speaking countries given the current approval status of drugs. This article provides an overview and discusses the drugs available for HAE therapy, their approval status and study results obtained in adults and pediatric patients. Recommendations for appropriate treatment strategies in the management of HAE in pediatric patients in German-speaking countries are provided. Currently, plasma-derived C1 inhibitor concentrate is the best licensed option for treatment of acute HAE-C1-INH attacks in pediatric patients in German-speaking countries, as well as for short-term and long-term prophylaxis.

• 出版日期2012-8