From the Department of Medicine, Penn State University, Hershey, Pennsylvania.
Cairo University School of Medicine, Cairo, Egypt; and.
Allergy Asthma Proc. 2022 Sep 1;43(5):388-396. doi: 10.2500/aap.2022.43.220052.
The management of hereditary angioedema has rapidly changed over the past decade. With these changes there has been increased recognition of the unique challenges of diagnosing and managing hereditary angioedema in pediatric populations. The objective of this review was to identify and compare recently published consensus guidelines for the management of hereditary angioedema types 1 and 2 to identify areas of agreement and conflict. A MEDLINE database search was performed to identify guidelines that offered guidance on diagnosing or managing hereditary angioedema in pediatric populations. A limitation was placed on guidelines published in the past 5 years to reflect the most recent literature. Six clinical practice guidelines were included in the analysis. Early detection of disease status, coordination with specialists, and empowering patients with self-administered medications are emphasized, with use of plasma derived C1 esterase inhibitor as first line therapy for aborting attacks. The guidelines are shifting away from attenuated androgens and tranexamic acid for long-term prophylaxis toward medications such as subcutaneous C1 esterase inhibitor, lanadelumab, and berotralstat. Although some differences exist based on geographic region and health system where an included guideline was published, they have very minimal differences on close review.
遗传性血管性水肿的管理在过去十年中发生了迅速变化。随着这些变化，人们越来越认识到儿科人群中诊断和管理遗传性血管性水肿的独特挑战。本综述的目的是确定并比较最近发表的 1 型和 2 型遗传性血管性水肿管理共识指南，以确定共识和冲突领域。进行了 MEDLINE 数据库搜索，以确定针对儿科人群中遗传性血管性水肿的诊断或管理提供指导的指南。将指南的出版时间限制在过去 5 年内，以反映最新的文献。有 6 项临床实践指南被纳入分析。强调早期发现疾病状态、与专家协调以及为患者提供自我管理药物的能力，使用血浆衍生的 C1 酯酶抑制剂作为终止发作的一线治疗。指南正在从减毒雄激素和氨甲环酸的长期预防转向皮下 C1 酯酶抑制剂、拉那芦单抗和贝罗特司他等药物。尽管根据纳入指南发表的地理位置和卫生系统存在一些差异，但仔细审查后差异非常小。
