Sabbah A, Marsollier I, Trouillard V
Laboratoire d'Immuno-Allergologie CHRU Angers.
Allerg Immunol (Paris). 1989 Oct;21(8):297-300.
Hereditary angio-oedema (AOH) is a familial affliction that is most often hereditary, but sometimes acquired and linked to a deficiency of C1 esterase inhibitor. 8 cases have been examined, amongst which there was one attack in a young girl, without any clinical signs. Amongst the other 7, one had acquired AOH. The diagnosis was confirmed by the deficiency of C1 esterase inhibitor, associated with a significant reduction of the other fraction of complement c3-c4 and also total haemolytic complement. Strong doses of corticosteroids were used in 4 patients for urgent treatment. Basic treatment with DANAZOL was commenced in 3 patients.
遗传性血管性水肿（AOH）是一种家族性疾病，大多具有遗传性，但有时也可后天获得，且与C1酯酶抑制剂缺乏有关。共检查了8例患者，其中一名年轻女孩发作时无任何临床症状。在其他7例中，有1例为后天性AOH。通过C1酯酶抑制剂缺乏得以确诊，同时伴有补体c3 - c4的其他组分以及总溶血补体显著降低。4例患者使用大剂量皮质类固醇进行紧急治疗。3例患者开始使用达那唑进行基础治疗。
