van der Klooster J M, Schelfhout L J D M, Grootendorst A F, Zweers P G M A
Onze Lieve Vrouwe Gasthuis, afd. Intensivecaregeneeskunde, Postbus 95.500, 1090 HM Amsterdam.
Ned Tijdschr Geneeskd. 2002 Aug 24;146(34):1599-602.
A woman experienced recurrent attacks of angioedema from the age of 17 to 21 years and these appeared to be associated with the use of oestrogens. After stopping the medication her complaints disappeared, but they returned during her first pregnancy. Angioedema is a serious condition, which can lead to acute abdominal symptoms, oedema of the upper respiratory tract and death by asphyxiation. The most well-known cause is hereditary angioedema, an autosomal dominant disorder that is characterized by deficiency of C1 esterase inhibitor (C1-INH). Recently, a new type of hereditary angioedema (type 3) has been reported that occurs exclusively in women and is characterised by oestrogen dependency (both endogenous and exogenous), normal C1-INH concentrations and severe attacks of angioedema, which are clinically indistinguishable from the classic form.
一名女性从17岁至21岁期间反复出现血管性水肿发作，这些发作似乎与雌激素的使用有关。停药后她的症状消失，但在首次怀孕时又复发。血管性水肿是一种严重病症，可导致急性腹部症状、上呼吸道水肿并因窒息而死亡。最广为人知的病因是遗传性血管性水肿，这是一种常染色体显性疾病，其特征为C1酯酶抑制剂（C1-INH）缺乏。最近，已报告一种新型遗传性血管性水肿（3型），仅发生于女性，其特征为雌激素依赖性（内源性和外源性）、C1-INH浓度正常以及血管性水肿严重发作，临床上与经典形式无法区分。
