García Robledo P, García Ortiz J C
Servicio de Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario 12 de Octubre, Madrid, Spain.
Unidad de Alergología, Hospital Universitario Río Hortega, Valladolid, Spain.
Rev Esp Anestesiol Reanim (Engl Ed). 2025 May;72(5):501732. doi: 10.1016/j.redare.2025.501732. Epub 2025 Feb 15.
Hereditary angioedema (HAE) is of interest to anaesthesiologists due to its impact on perioperative management. HAE manifests as episodic, self-limiting oedema in the deep subcutaneous or submucosal tissue that can be fatal if it involves the airway. It is usually caused by C1 esterase inhibitor deficiency. HAE episodes can be triggered by trauma, including surgical and medical procedures associated with mechanical trauma. Our understanding of HAE has evolved in recent years following the development of new genetic sequencing technologies that have made it possible to differentiate variants with normal C1 esterase inhibitor levels. In Spain, the most frequent of these is HAE due to a mutation in FXII, previously known as HAE type III. There are as yet no good quality studies on perioperative management and treatment of these patients. The admission of a patient with FXII-HAE for labour induction has given us the opportunity to review the perioperative management of these cases.
遗传性血管性水肿（HAE）因其对围手术期管理的影响而受到麻醉医生的关注。HAE表现为深部皮下或黏膜下组织的发作性、自限性水肿，如果累及气道可能会致命。它通常由C1酯酶抑制剂缺乏引起。HAE发作可由创伤触发，包括与机械创伤相关的外科手术和医疗操作。近年来，随着新的基因测序技术的发展，我们对HAE的认识有所进展，这些技术使区分C1酯酶抑制剂水平正常的变异体成为可能。在西班牙，最常见的是由于FXII基因突变导致的HAE，以前称为III型HAE。目前尚无关于这些患者围手术期管理和治疗的高质量研究。一名FXII-HAE患者因引产入院，这使我们有机会回顾这些病例的围手术期管理。
