Przebieg wrodzonego obrzęku naczynioruchowego z niedoboru C1 inhibitora u koboit podczas ciąż i porodu.

Introduction. The hereditary angioedema (HAE, D 84.1 acc, to ICD-10) is a rare disorder of the incidence of 1 case per 10,000-50,000 in the white population. The most known among this type of angioedemas is the oedema with the background of the C1 inhibitor deficiency. Aim of the study. The aim of the study was to analyse the course of the HAE symptoms, diagnostic and therapeutical problems in HAE women during pregnancy and delivery on the in-house material. Material and methods. We analyzed 84 pregnancies and deliverys among 44 women suffering from hereditary angioedema due to C1 inhibitor deficiency. The increase in the intensity of angieodema symptoms was observed in 15 percent women (18%), and in 5 pregnant women (6%) an attack of angioedema occurred for the first time in their medical history. In the following 32 pregnancies (38%), the course of the disease did not change and 32 patients (38%) had no symtoms during pregnancy. In 10 of 24 women, the course of symptoms was different in consecutive pregnancies. The attacks of angioedema during pregnancy were often the abdominal types and caused diagnostic difficulties. The intravenous administration of C1 inhibitor [Berinert P] solved this problem efficiently alleviating the pain in cases, where it was connected with the attack of angioedema affecting the abdominal organs. Conculusions. The attacks of angioedema were not observed during 82 of 84 deliverys. In 3 cases, the angioedema of perineum occurred immediately after delivery. In 2 cases, facial and laryngeal attack of angioedema occurred during the delivery, thus then necessity to provide Berinert P for a women with angioedema connected with the C1 inhibitor deficiency during the delivery in a must. [ABSTRACT FROM AUTHOR]