Duke University Medical Center, Durham, North Carolina;
University of California Medical Center, San Diego, California.
Pediatrics. 2016 Nov;138(5). doi: 10.1542/peds.2016-0575.
Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults. Only 1 medication is approved for treatment of children <12 years of age, and there are no reported consensus recommendations for treatment of young children in the United States. The 11-member Medical Advisory Board, with extensive experience in the treatment of children, in concert with the leaders of the HAE Patient's Association, has developed these consensus recommendations to help in recognition, diagnosis, treatment of attacks, and prophylaxis of children with HAE.
遗传性血管性水肿（HAE）是一种潜在危及生命的遗传性疾病，其特征是皮肤肿胀、严重腹痛和上呼吸道肿胀发作。发作通常始于儿童期，但适当的诊断经常被忽视。发作对肾上腺素、抗组胺药或糖皮质激素没有反应。最近，许多有效的药物已被批准用于治疗成人 HAE，HAE 患者协会的医学顾问委员会已制定并报告了成人治疗建议。只有 1 种药物被批准用于治疗<12 岁的儿童，在美国，尚无针对幼儿治疗的共识建议。由在儿童治疗方面具有丰富经验的 11 名医学顾问委员会成员与 HAE 患者协会的领导人共同制定了这些共识建议，以帮助识别、诊断、治疗 HAE 儿童发作和预防。
