Bouillet Laurence
CHU de Grenoble, clinique universitaire de médecine interne, centre nationale de référence des angioedèmes (CREAK), boulevard de la Chantourne, 38043 Grenoble cedex 09, France.
Presse Med. 2015 Jan;44(1):52-6. doi: 10.1016/j.lpm.2014.06.027. Epub 2014 Dec 12.
Hereditary angioedema is a rare disease, potentially life-threatening. It requires a specific treatment. Angioedema without wheals associated with abdominal attacks are very specific of this disease. Antigenemy and functional C1Inhibitor assays are necessary for the diagnosis. The hereditary angioedema with normal C1Inh (type III) is a diagnostic challenge. Bradykinin, secondary to kallikrein-kinin system activation is the key mediator of hereditary angioedema. Female are more symptomatic. Attacks can be induced by menstruations, pregnancies or contraceptive pills.
遗传性血管性水肿是一种罕见的、可能危及生命的疾病。它需要特定的治疗方法。与腹部发作相关的无风团血管性水肿是这种疾病非常典型的症状。诊断需要进行抗原检测和功能性C1抑制物检测。C1抑制物正常的遗传性血管性水肿(III型)是一个诊断难题。激肽释放酶 - 激肽系统激活继发产生的缓激肽是遗传性血管性水肿的关键介质。女性症状更明显。发作可由月经、怀孕或避孕药诱发。