OBJECTIVE

To describe the clinical observations of pregnancy and delivery management in patients with a rare (orphan) disease — hereditary angioedema (HAE).

MATERIAL AND METHODS

Analysis of modern medical literature on the topic of hereditary angioedema, diagnosis and treatment of this pathology, as well as management and delivery of patients diagnosed with HAE. Weown experience of clinical observation, safe management and delivery of pregnant women with HAE.

RESULTS

Angioedema is an edema of the skin, mucous membranes, and submucosa of various areas of the body. Taking into account the complement-dependent pathogenesis of edema, the definition of «hereditary angioedema» is used. The prevalence of the disease, according to various data, varies from 1:50.000 to 1:100.000. Pregnancy and childbirth serve as trigger factors for the manifestation of HAE, the development of edema of life-threatening localizations, up to fatal outcomes. There is an obvious need to choose the optimal method of delivery in each such clinical observation on an individual basis, as well as to prepare the pregnant woman for childbirth using specific basic HAE therapy.

CONCLUSION

Timely diagnosis, an optimally selected therapy regimen allows patients with HAE to improve their quality of life, minimize the manifestations of the disease, avoiding edema in life-threatening areas, and also allows such patients to successfully plan and carry a pregnancy.