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Allergology Review

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  Authority of Polish Society of Allergology

vol 13. no 2. June 2008  

 Original articles
The course of hereditary angioedema due to C1 inhibitor deficiency in women during pregnancy and delivery
Krystyna Obtułowicz, Grzegorz Porębski, Barbara Bilo, Marcin Stobiecki, Aleksander Obtułowicz

Introduction. The hereditary angioedema – HAE (D84.1) is a rare disorder of the indicidence 1 case on 10 000-50 000 persons of the Caucasian population. The most known among this type of angioedemas is HAE in persons with C1 inhibitor deficiency.
Aim of the study. The aim of the study was to analyse the diagnostic and therapeutical problems as well as the occurence of symptoms HAE during pregnancy and delivery in observed patients.
Material and methods. The analysis was done on the basis of 84 pregnancies and deliveriesin 44 observed women suffering from HAE. The diagnosis of HAE was based on history and typical angioedemas attacks as well as on decreasing minimum 40% the function of C1 inhibitor and C4 serum level.
Results. The exacerbations of HAE symptoms was observed during 15 pregnancies (18%). In following 5 pregnancies (6%) the attack of HAE took place the first time in life of patient. In the following 32 pregnancies (38%) the course of disease did not change and in the next one 32 pregnancies the symptoms disappeared. During consecutive pregnancies among 24 women in 10 the course of illness was different. The attacks of angioedemas during pregnacies were often the abdominal type and caused diagnostic difficulties. The C1 inhibitor infsion (Berinert P) solved this problem efficiently alleviating the symptoms in case of HAE attack. During 84 deliveries the angioedema attack was observed only in 2 cases. In both cases the infussion of Berinert P was necessary because developing of life-treatening facial and larynx angioedemas.
Conclusions. The course of HAE symptoms in pregnant women is different. In 18% there is exacerbation of symptoms. Often there are abdominal type and disappear after infusion of C1 inhibitor. During deliveries HAE attacks are very rare but may be life-treatening and infusion of C1 inhibitor is then necessary.

keywords: obrzęk naczynioruchowy, C1 inhibitor, ciąża, poród , angioedema, C1 inhibitor, pregnancy, delivery

pages: from 91 to 99

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