Молодежный инновационный вестникМолодежный инновационный вестник2415-7805Федеральное государственное бюджетное образовательное учреждение высшего образования "Воронежский государственный медицинский университет имени Н.Н. Бурденко" Министерства здравоохранения Российской Федерации9893Conference ProceedingsPathological activity of the hemostasis system as a determining link of the complicated course of pregnancyКазумоваAglayaaglaya.kazumowa@yandex.ruhttps://orcid.org/0009-0003-6481-60172712202413252541603202415062024Copyright © 2024, Казумова A.2024<p>Introduction. In the last decade, more and more studies have reported the presence of congenital thrombotic thrombocytopenic purpura in patients suffering from complications of pregnancy and childbirth such as repeated fetal loss, stillbirth, intrauterine growth retardation and preeclampsia. This pathology requires a special approach in diagnosis and therapy. The aim of the study is to analyze the relevance of the diagnosis of the ADAMTS13 genetic defect in Upshaw-Shulman syndrome, to determine the practicality and productivity of technologies used for the treatment of emerging obstetric complications. Materials and methods. A retrospective analysis of 21 medical records of patients who were treated at the department from 2017 to 2023 was carried out. Results. In total, 21 pregnancy episodes occurred in women from the groups analyzed by the author, of which 15 had obstetric complications, including 46.7% (7/15) premature fetal loss (10 weeks), 26.7% (4/15) late fetal loss (10 weeks) and 26.6% (4/15) premature live births. Conclusions. Monitoring of ADAMTS13 serum levels is carried out when planning a subsequent pregnancy. Long-term complications of TTP should be taken into account: ischemic strokes, secondary hypertension, coronary heart disease, cognitive abnormalities and a decrease in quality of life. Pregnancy with Upshaw-Shulman syndrome will become one of the main areas of obstetric development in the coming years, further associative and high-quality research is needed to form the best therapeutic innovations and opportunities in the future.</p>thrombotic thrombocytopenic purpuraUpshaw-Shulman syndromevon Willebrand factorADAMTS13vascular microthrombosispregnancyplacenta.тромботическая тромбоцитопеническая пурпурасиндром Апшоу-Шульманафактор фон ВиллебрандаADAMTS13сосудистый микротромбозбеременностьплацента1.An open conformation of ADAMTS-13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura / E. Roose, A.S. Schelpe, B.S. Joly [et al.] // Journal of Thrombosis and Haemostasis. – 2018. – Vol. 16, № 2. – P. 378-388.2.Thrombotic microangiopathies of pregnancy: Differential diagnosis / M. Gupta, B.B. Feinberg, R.M. Burwick // Hypertension in Pregnancy. – 2018. - P. 29-34.3.A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy / T. Nonaka, M. Yamaguchi, K. Nishijima [et al.] // Journal of Obstetrics and Gynaecology Research. – 2021. – Vol. 47, № 5. – Р. 1892-1897.4.Hereditary Thrombotic Thrombocytopenic Purpura / J.A. Kremer Hovinga, J.N. George // The New England Journal of Medicine. – 2019. – Vol. 381, № 17. – P. 1653-1662.5.ADAMTS-13 and von Willebrand factor: a dynamic duo / K. South, D.A. Lane // Journal of Thrombosis and Haemostasis. – 2018. - Vol. 16, № 1. – P. 6-18.