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After the vaccination campaign initiation in Europe and the UK, reports of rare cases of atypical thrombosis, including sinus vein thrombosis and splanchnic venous thrombosis, began to appear in association with the use of vector vaccines AstraZeneca (ChAdOx1) and Johnson & Johnson/Janssen. The syndrome called VITT (vaccine-induced immune thrombotic thrombocytopenia) manifested as thrombosis simultaneously with a decrease in platelet count, a significant increase in D-dimer levels and a detection of factor 4 platelet (PF4) antibodies. We present a detailed review of the epidemiology, pathogenesis, clinical presentation, diagnostics and treatment of VITT, which is by its nature an immune complication, similar to the processes occurring in heparin-induced thrombocytopenia (HIT). All international and national organizations and regulatory authorities, including experts in the field of thrombosis and hemostasis and the VITT expert council recommend continuing the prompt mass vaccination against COVID-19 as the only method that can reduce the incidence of severe cases, stop the spread of COVID-19 infection and the emergence of new dangerous mutations in the viral genome. Failure to vaccinate poses an incomparably greater risk of fatal thrombotic and inflammatory complications associated with infections, compared with the risks of extremely rare adverse events that can occur after vaccination. It should be noted that information on VITT, described as a sporadic phenomenon of an abnormal immune response to some variants of vaccines against COVID-19, cannot be translated to other vaccines (including registered in the Russian Federation) and even more cannot be a reason for refusal to use them.

The rate of thrombosis and disseminated intravascular coagulation (DIC) has been increasing in COVID-19 patients. Key features related to such condition include minimal or no risk of bleeding, moderate thrombocytopenia, high plasma fibrinogen as well as complement components level in the areas of thrombotic microangiopathy. The clinical picture is not typical for classic DIC. This review systematizes the pathogenetic mechanisms of hypercoagulation in sepsis and its extreme forms in patients with COVID-19. The latter consist of the thrombosis-related immune mechanisms, the complement activation, the macrophage activation syndrome, the formation of antiphospholipid antibodies, the hyperferritinemia, and the dysregulation of the renin-angiotensin system. Taking into consideration the pathogenetic mechanisms, the biomarkers had been identified related to the prognosis of the disease development. Patients with pre-existing cardiovascular disease and other risk factors, including obesity, diabetes, hypertension, and aging pose the peak risk of dying from COVID-19. We also summarize new data on platelet and endothelial dysfunction, immunothrombosis, and, as a result, thrombotic storm as essential components of COVID-19 severe features.

Data analysis on the pathogenesis and risk factors of neonatal thrombosis was carried out. The main risk factor of any neonatal thrombosis is central catheter installment, but other maternal, fetal and neonatal factors should be taken into consideration. We discuss the epidemiology of neonatal thrombosis and the main features of the hemostasis system in newborns, the most significant risk factors, including genetic and acquired thrombophilia. We consider the von Willebrand factor activity and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) level in the development of neonatal thrombotic microangiopathy. Finally, we discuss the basic principles of prevented neonatal thrombosis by using low molecular weight heparins.

Antiphospholipid syndrome (APS) is an autoimmune process that increases the risk of arterial and venous thrombosis. The mechanism of damage to the central nervous system (CNS) can be not only due to thrombosis, but also antiphospholipid antibodies (APA) circulating in the peripheral blood. The latter can damage the cerebral vascular endothelium, alter the resistance of the blood-brain barrier and penetrate into the central nervous system, exerting a damaging effect on astroglia and neurons, as evidenced by the release of neurospecific proteins into the peripheral bloodstream. The role of APS in developing cerebral ischemia, migraine, epilepsy, chorea, transverse myelitis, multiple sclerosis, cognitive impairment and mental disorders, as well as the peripheral nervous system is described. It should also be noted about a role of APS for emerging neurological disorders in COVID-19, enabled apart from thrombogenesis due to APA via 2 potential mechanisms - molecular mimicry and neoepitope formation. Further study of the APS pathogenesis and interdisciplinary interaction are necessary to develop effective methods for patient management.

The work is aimed at discussing pregnancy management for the most thrombogenic genetic thrombophilia - antithrombin III (AT-III) deficiency. A detailed analysis of the literature and clinical case of pregnancy management in a patient with AT-III deficiency, pulmonary embolism and habitual history of miscarriage has been performed and presented. Patients with AT-III deficiency are at high risk for developing thrombotic and obstetric complications even despite using therapeutic doses of anticoagulants. Indications for use and modes of administration of AT-III concentrate have not been currently defined clearly. Monitoring therapy with low molecular weight heparin is largely complicated because a test for determining anti-Xa activity is AT-III-dependent. In addition to standard methods for controlling antithrombotic therapy, we used tests characterizing the dynamic blood clot parameters: thromboelastography and thrombin generation test. The peak risk resulting in both thrombotic and hemorrhagic complications in such patients occurs during period of labor and the postpartum period, when a change in the regimen of anticoagulant therapy is required with its temporary withdrawal and additional administration of AT-III concentrate.

Aim: to analyze current literature sources on endometriosis-associated cancer (ЕАС) in gynecology.Materials and Methods. We searched for publications in the international scientific databases: eLibrary, Google Scholar, ScienceDirect, Cochrane Library, PubMed/MEDLINE and clinical research registries. For this systematic analysis, only full-text publications were used. We evaluated the reliability of evidence and the methodological quality of the studies. The data on the modern approach to the therapy and rehabilitation of women with external genital endometriosis (EGE) are presented. Search terms for publications in Russian and English were used: «endometriosis», «endometriosis-associated ovarian neoplasia», «oncology», «rehabilitation».Results. Despite the fact that EGE is considered a benign disease, there is a risk for its malignant transformation. However, the pathogenesis and pathomorphological mechanisms of ЕАС are not yet fully understood.Conclusion. Active management of the rehabilitation period for women with EGE provides an opportunity for early diagnosing recurrences that will provide early diagnostics ovarian ЕАС and improve the main indicators of the quality of life for such patients.

Aim: to assess a rate and range of genetic and acquired thrombophilia in onco-gynecologic patients with ovarian cancer, uterine corpus cancer and cervical cancer.Materials and Мethods. A prospective controlled cohort non-randomized interventional study was conducted: within the years 2014 to 2020, there were examined 546 women with genital malignancies, divided into 2 groups: group I – 155 cancer patients with former thrombosis, group II – 391 women with female genital cancer without former thrombotic complications. Control group consisted of 137 patients with benign female genital tumors. The spectrum of circulating APA was studied: antibodies to â2-glycoprotein I (â2-GPI), annexin V and prothrombin as well as genetic thrombophilia due to mutations genes encoding factor V Leiden, methylenetetrahydrofolate reductase (MTHFR) including polymorphism in genes for prothrombin, platelet glycoproteins and plasminogen activator inhibitor-1 (PAI-1).Results. It was found that frequency of circulating APA as well as incidence rate of genetic thrombophilia between cancer patients from group I vs. group II significantly differed: APA in group I vs. group II was 86 (55.5 %; p < 0.01) vs. 92 (23.5 %) compared to 7 (5.1 %) in control group. Genetic thrombophilia was dominated in group I by mutated MTHFR (92.9 %), polymorphismin PAI-1 (28.4 %) and platelet glycoprotein (44.5 %) that were significantly higher (p < 0.05) compared to group II and control group. Hence, it allows to suggest that such identified thrombophilia markers are largely associated with a risk of developing thrombotic complications.Conclusion. Detected high percentage of patients with circulating APA and genetic thrombophilia among cancer patients with former thromboembolic complications corroborate a role for genetic and acquired thrombophilia in developing pre-thrombotic condition. Detecting a range of circulating APA and genetic thrombophilia allows to identify patients who might be referred to a high risk of thrombogenesis and require to preventive application of anticoagulant therapy.

Our knowledge regarding chemical structure and properties of heparin and its derivatives, including biological properties in blood plasma, on the cell surface and while interacting with receptors, has been progressively growing. New insights are followed by the expansion of therapeutic opportunities and indications for the use of heparins. There are prerequisites for the creation of new generation drugs with modified properties that reduce a bleeding risk while applied for a non-anticoagulant goal. The non-anticoagulant heparin properties allow to consider it as a candidate for pathogenetic treatment of patients with COVID-19. This review focuses on the anticoagulant and non-anticoagulant heparin properties as well as the underlying molecular mechanisms.

Assessment of fetal cardiac function is one of the essential components of fetal echocardiography. Functional impairment is associated with a high risk of adverse perinatal outcomes and even antenatal death. Prenatal detection of hemodynamics changes requires immediate prenatal actions to identify the causes and eliminate the consequences that may require conservative treatment, intrauterine surgical treatment, and early delivery in perinatal centers.

Introduction. Perinatal mortality in multiple pregnancies increases by 8–10 times compared to singletons. Stillbirth is a significant part of all complications of multiple pregnancies. Although the incidence of perinatal mortality in multiple pregnancies has decreased consistently compared to extremely high rates in the past, it remains relatively high, despite significant positive changes in the management of such pregnancies. Aim: to assess the diagnostic potential of the first trimester's biochemical screening in multiple pregnancies for predicting antenatal fetal death. Materials and Methods. As part of a retrospective study, a cohort of twin pregnancies after in vitro fertilization (IVF), intracytoplasmic sperm injection (ICSI), or spontaneous conception underwent screening of the first trimester at 11+0–13+6 weeks of gestation as recommended by the Fetal Medicine Foundation. Determination of pregnancy-associated plasma protein-A (PAPP-A) in blood serum with subsequent calculation of the relative PAPP-A MoM (multiples of median) – a multiple of the median (an indicator of how much the individual test result deviates from the reference values) was performed. Results. Prenatal screening and outcomes of 302 multiple pregnancies showed that with PAPP-A MoM < 0.5, antenatal fetal death occurred with a frequency of 42.86 % (6/14), with PAPP-A MoM within the reference values – In 12.67 % (28/221), with PAPP-A MoM > 2.0 – in 6.7 % (2/30). Differences between patients with PAPP-A MoM < 0.5 and PAPP-A MoM within the reference values, as well as PAPP-A MoM < 0.5 and PAPP-A MoM > 2.0 were statistically significant (p = 0.002 and p = 0.004, respectively). No differences were detected between spontaneous and assisted reproductive technology (ART) pregnancies. Conclusion. In women with multiple pregnancies resulting from ART or spontaneous, PAPP-A MoM values below the reference interval (< 0.5) in the first trimester are associated with an increased risk of antenatal fetal death.

There is a global problem of increment of the number of children with clinical features that mimic Kawasaki Disease (KD) during the ongoing Coronavirus Disease 2019 (COVID-19) pandemic. The disease was first reported by Tomisaku Kawasaki, a Japanese pediatrician, in a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo in January 1961. Now Kawasaki disease is recognized worldwide. The complexity of symptoms was defined as an «acute febrile mucocutaneous lymphnode syndrome". At the moment, it is still unclear whether the coronavirus itself can lead to development of mucocutaneous lymph node syndrome. However, it is believed that COVID-19 virus infection worsens the course of Kawasaki disease, and in some cases, children affected by SARS-V-2 may develop a disease that has a clinical picture similar to Kawasaki disease.

Dear editors of Obstetrics, Gynecology and Reproduction Journal! Due to the particular urgency of the problem of managing patients with a new coronavirus infection (COVID-19), we are sending a letter outlining our position on this issue.

COVID-19 is an infectious disease caused by the beta-coronavirus SARS-CoV-2 that in 2020 has spread worldwide. In most severe patients, the clinical picture begins with respiratory failure further deteriorating up to multiple organ failure. Development of coagulopathy is the most adverse prognostic. Analyzing currently available clinical data revealed that 71.4 % and 0.6 % of survivors and fatal cases, respectively, demonstrated signs of overt disseminated intravascular coagulation (DIC). Monitoring D-dimer level, prothrombin time, platelet count and fibrinogen content is important for determining indications for treatment and hospitalization in COVID-19 patients. In case such parameters deteriorate, a more pro-active “aggressive” intensive care should be applied. Low molecular weight heparin (LMWH) should be administered to all patients with diagnosed COVID-19 infection (including non-critical patients) requiring hospitalization, but having no contraindications to LMWH.

The pandemic of a novel coronavirus infection COVID-19 has become a real challenge to the mankind and medical community and has raised a number of medical and social issues. Based on the currently available information on COVID-19 clinical cases, it follows that COVID-19 patients in critical condition exhibit a clinical picture of disseminated intravascular coagulation (DIC), septic shock with developing multiple organ failure, which justifies use of anticoagulant therapy in COVID-19 patients. In addition to isolating virus RNA from biological material and polymerase chain reaction diagnostics, use of simple and easily accessible laboratory blood markers is necessary for management of COVID-19 patients. If the activation of coagulation processes is sufficient enough, consumption of platelets and blood clotting factors can be diagnosed by laboratory methods as prolongation of routine blood clotting tests and increasing thrombocytopenia. Hyperfibrinogenemia, increased D-dimer level, prolonged prothrombin time, thrombocytopenia, lymphopenia, leukocytopenia, increased concentration of interleukin-6 and ferritin are observed in most COVID19 patients. The degree of increase in these changes correlates with severity of the inflammatory process and serves as a prognostically unfavorable sign. Here we discuss value of laboratory monitoring playing an essential role in such pathological crisis that contributes to patient screening, diagnosis as well as further monitoring, treatment and rehabilitation.

Zika virus (ZIKV) is an arthropod-borne virus (arbovirus) in the genus Flavivirus and the Flaviviridae family. In 1947 and 1948 ZIKV was first isolated from a nonhuman primate as well as from mosquitoes in Africa, respectively. For half a century, ZIKV infections in human were sporadic prior to 2015–2016 pandemic spreading. Transmission of ZIKV from mother to fetus can occur in any trimester of pregnancy, even if mother was an asymptomatic carrier. The clinical signs of ZIKV infection are nonspecific and can be misdiagnosed as some other infectious diseases, especially those caused by arboviruses such as Dengue and Chikungunya. ZIKV infection was solely associated with mild illness prior to the large French Polynesian and Brazil outbreaks, when severe neurological complications, Guillain–Barre syndrome and dramatically increased rate of severe congenital malformations (including microcephaly) were reported. The adaptation of ZIKV to an urban cycle in endemic areas suggests that the incidence of ZIKV infections may be underestimated. The pandemic of novel coronavirus infection (COVID-19) demonstrates that lessons from ZIKV pandemic propagation has not been learned properly.

Pregnancies complicated by late-onset fetal growth restriction (FGR) are at increased risk of short- and long-term morbidities. Despite this, identification of cases at higher risk of adverse perinatal outcome, at the time of FGR diagnosis, is challenging. The aims of this study were to elucidate the strength of association between fetoplacental Doppler indices at the time of diagnosis of late-onset FGR and adverse perinatal outcome, and to determine their predictive accuracy.This was a prospective study of consecutive singleton pregnancies complicated by late-onset FGR. Late-onset FGR was defined as estimated fetal weight (EFW) or abdominal circumference (AC) 3In total, 243 consecutive singleton pregnancies complicated by late-onset FGR were included. Composite adverse perinatal outcome occurred in 32.5% (95% CI, 26.7-38.8%) of cases. In pregnancies with composite adverse perinatal outcome, compared with those without, mean uterine artery PI Z-score (2.23 ± 1.34 vs 1.88 ± 0.89, P = 0.02) was higher, while Z-scores of UVBF/AC (-1.93 ± 0.88 vs -0.89 ± 0.94, P ≤ 0.0001), MCA-PI (-1.56 ± 0.93 vs -1.22 ± 0.84, P = 0.004) and CPR (-1.89 ± 1.12 vs -1.44 ± 1.02, P = 0.002) were lower. On multivariable logistic regression analysis, Z-scores of mean uterine artery PI (P = 0.04), CPR (P = 0.002) and UVBF/AC (P = 0.001) were associated independently with composite adverse perinatal outcome. UVBF/AC Z-score had an area under the ROC curve (AUC) of 0.723 (95% CI, 0.64-0.80) for composite adverse perinatal outcome, demonstrating better accuracy than that of mean uterine artery PI Z-score (AUC, 0.593; 95% CI, 0.50-0.69) and CPR Z-score (AUC, 0.615; 95% CI, 0.52-0.71). A multiparametric prediction model including Z-scores of MCA-PI, uterine artery PI and UVBF/AC had an AUC of 0.745 (95% CI, 0.66-0.83) for the prediction of composite adverse perinatal outcome.While CPR and uterine artery PI assessed at the time of diagnosis are associated independently with composite adverse perinatal outcome in pregnancies complicated by late-onset FGR, their diagnostic performance for composite adverse perinatal outcome is low. UVBF/AC showed better accuracy for prediction of composite adverse perinatal outcome, although its usefulness in clinical practice as a standalone predictor of adverse pregnancy outcome requires further research. Copyright © 2019 ISUOG. Published by John WileySons Ltd.Papel de la ecografía Doppler en el momento del diagnóstico de la restricción del crecimiento fetal de aparición tardía para la predicción de resultados perinatales adversos: estudio prospectivo de cohortes OBJETIVO: Los embarazos complicados por la restricción del crecimiento fetal (RCF) de aparición tardía tienen un mayor riesgo de morbilidad a corto y largo plazo. A pesar de ello, es difícil identificar los casos con mayor riesgo de resultados perinatales adversos en el momento del diagnóstico de RCF. Los objetivos de este estudio fueron dilucidar la fortaleza de la asociación entre los índices Doppler fetoplacentarios en el momento del diagnóstico de la RCF de aparición tardía y el resultado perinatal adverso, y determinar su precisión predictiva. MÉTODOS: Este fue un estudio prospectivo de embarazos consecutivos con feto único complicados por una RCF de aparición tardía. La aparición tardía de la RCF se definió como peso estimado del feto (PEF) o circunferencia abdominal (CA)3多普勒超声在诊断迟发性胎儿生长受限时预测不良围产儿结局中的作用:前瞻性队列研究 目标: 妊娠合并迟发性胎儿生长受限(FGR)孕妇的短期长期发病风险增加。即便如此，在FGR诊断中确定高风险围产儿不良结局病例依然很困难。本研究旨在阐明迟发性胎儿生长受限诊断时胎儿胎盘多普勒指数与不良围产儿结局之间的关联强度，并确定其预测准确性。 方法: 这是一项针对连续单胎妊娠合并迟发性胎儿生长受限的前瞻性研究。迟发性FGR定义为32周诊断发现估算胎儿体重(EFW)或腹围(AC)第3百分位数，或EFW或AC第10百分位数且脐动脉(UA)搏动指数(PI)第95百分位数，或脑胎盘比率(CPR)第5百分位数。FGR诊断过程中记录了EFW、子宫动脉PI、UA-PI、胎儿大脑中动脉(MCA)PI、CPR，以及胎儿腹围脐静脉血流正常化(UVBF/AC)。以胎龄的Z评分表示多普勒变量。围产期综合不良结局定义为 发生至少1例胎儿窘迫急诊剖宫产、5分钟Apgar评分7、脐动脉pH7.10、新生儿入院接受特殊护理。通过逻辑回归分析探讨不同超声参数与围产期综合不良结局之间的关联强度，通过受体操作特征(ROC)曲线分析 确定其预测准确性。 结果: 总共包括243例连续单胎妊娠并发迟发性FGR。32.5%(95%CI，26.7-38.8%)的病例出现复合不良围产期结局。相比那些并无复合不良围产期结局的孕妇，复合不良围产期结局孕妇的平均子宫动脉PI Z评分(2.23±1.34对1.88±0.89，P=0.02)较高，而UVBF/AC的Z评分(-1.93±0.88对-0.89±0.94，P≤0.0001)、MCA-PI(-1.56±0.93 对-1.22±0.84，P=0.004)和CPR(-1.89±1.12对-1.44±1.02，P=0.002)较低。在多变量逻辑回归分析中，子宫动脉平均PI(P=0.04)、CPR(P=0.002)和UVBF/AC(P=0.001)的Z评分与复合不良围产期结局独立相关。UVBF/AC Z评分复合不良围产期结局ROC曲线下面积(AUC)为0.723(95%CI，0.64-0.80)，表明其准确性优于平均子宫动脉PI Z评分(AUC，0.593；95%CI，0.50-0.69)和CPR Z评分(AUC，0.615； 95%CI，0.52-0.71)。一个用于预测复合不良围产期结局且包括MCA-PI、子宫动脉PI和UVBF/AC的Z评分在内的多参数预测模型，其AUC为0.745(95%CI，0.66-0.83)。 结论: 虽然在诊断时接受评估的CPR和子宫动脉PI与妊娠并发迟发性FGR复合不良围产期结局独立相关，但它们的复合不良围产期结局诊出性较低。UVBF/AC在复合不良围产期结局的预测上更加准确，虽然它作为不良妊娠结局的独立预测指标在临床实践中的有用性有待进一步研究确定。版权 © 2019 ISUOG。由威利父子公司(John WileySons Ltd)出版。.

Hormonal contraceptives are effective in preventing an unwanted pregnancy, but have side effects; among them stroke occupies an important place. The development of this serious disease may be associated with the hormonal drug components, but heavily dependent on additional risk factors for vascular and thrombotic complications such as smoking, arterial hypertension, genetically determined and acquired predisposition to thrombotic complications. This review presents an analysis of the most important risk factors for stroke such as genetic predisposition, hyperhomocysteinemia, thrombophilias in women using combined oral contraceptives.

Introduction. Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by intravascular activation of blood clotting without specific localization and arising from various causes. DIC is the most complex and high-priority problem in contemporary clinical medicine including obstetrics and perinatology. DIC significance is related to its peak perinatal mortality, as well as a large rate of complications during childbirth and postpartum period. Aim: to conduct comprehensive data assessment related to DIC pathogenesis and management in pregnant women and newborns. Materials and methods . A search for DIC-related publications within the past 10 years was conducted in international research databases: Scientific Electronic Library Online eLibrary, Google Scholar, ScienceDirect, Cochrane Library, PubMed/Medline. The data regarding DIC diagnostic criteria and markers, as well as current approach to its treatment are presented. Results. DIC may be considered as one of the causes resulting in massive obstetric bleeding. The majority of pregnancy-related complications is manifested as preeclampsia, premature detachment of normally situated placenta and anaphylactoid syndrome of pregnancy. Any massive bleeding should be perceived as hemorrhage resulting from the DIC progression. Conclusion. Knowledge of the DIC pathogenetic mechanisms is necessary for conducting a differential diagnosis and applying timely treatment.

The rapid spread of COVID-19 and the large number of cases put a significant burden on the health systems of any developed country. Specialists in natural disasters and military medicine should be involved in the provision of medical care and observance of anti-epidemic measures. In some countries, including Italy, they were involved only after the situation was dramatically worsening with many clinical units and hospitals overloaded by infected patients. To curb the spread of COVID-19, most countries declared a state of emergency, and unprecedented measures have been taken to strengthen quarantine in suspected or positive symptomatic subjects. Nevertheless, the crisis associated with the unexpectedly global scale and tragedy of the pandemic and the inconsistency of actions of both society and individuals and specialized medical services, lead to insufficient effectiveness of the measures taken in a number of regions. In the present day, it is vital for every person to change its mindset ― relying on personal responsibility to comply with all recommendations of quarantine and anti-epidemic measures, and to reorganize departments and resources of medical institutions at all levels in order to withstand the spread of infection and at the same time provide all those in need with the necessary and appropriate medical care. Particular attention should be paid to the obstetric care service, given that even in normal times, the obstetric hospital is an area of increased responsibility for the life and health of mother and child and future mankind. Fulfillment of existing orders, instructions of national and regional committees, international and national protocols and clinical protocols should undoubtedly lead to a positive result, but this requires additional training of medical personnel at all levels. The purpose of this review is to propose quick key strategies for reassessing the maternity and neonatal wards/ hospitals based on the experience of health systems and organizations which faced the spread of this new coronavirus; this advice may be applied along with binding tight instructions in obstetric hospitals in order to proactively respond to a likely wave of growth in COVID-19.

Neonatal thromboembolism in pediatric patients is a rare but life-threatening condition mainly caused by combinations of at least 2 prothrombotic triggering risk factors such as the central venous lines, septic condition, and prematurity. Other risk factors include asphyxia, dehydration, liver dysfunction, inflammation, and maternal condition. Neonatal hemostatic system is different from one of the older children and adults. Coagulation proteins do not cross the placenta but are synthesized in the fetus from an early stage. In the term neonate, concentrations of several procoagulant proteins, particularly the vitamin K dependent and contact factors are reduced when compared with adults. Conversely, levels of antithrombin, heparin cofactor II and protein C and S are low at birth and fibrinolysis system is characterized by the decreased level of plasminogen and alpha-1-antiplasmin, increased tissue plasminogen activator. These features all tend to be gestational dependent and are more present in the preterm infant. Primarily in this context neonates appear to be at a higher risk of thrombosis than older children. Thrombotic complications reach their peak in the group of children born at 22-27 weeks. The role of inherited thrombophilic risk factors in neonatal VTE development is poorly defined. The presence of inherited and acquired thrombophilia in mother and newborn is also responsible for the development of thrombosis in neonates and should be considered. Thrombophilia in the mother can lead to increased coagulation potential and prethrombotic conditions during pregnancy, causing thrombotic vasculopathy at the placental level. The benefit of identifying thrombophilia in the sick preterm newborns who are in the group of risk for development of thrombotic complications may facilitate the thromboprophylaxis. Further research regarding assessment of risk factors, diagnostics and treatment strategy is required.

The problem of pregnancy losses and infertility in autoimmune pathology is one of the most urgent problems of modern reproductive medicine. Antiphospholipid antibodies (aPL) are very often connected with reproductive failures such as miscarriage, antenatal fetal death, preeclampsia and even infertility and failure ofTo evaluate the difference in immune status of aPL-positive women with infertility compared to healthy women and explain the possible mechanism of pathological effects of aPL, a correlation analysis between the level of aPL and the lymphocytes subpopulation was performed.We observed 280 women of reproductive age. Of these, 191 who met the inclusion and exclusion criteria were included in the study. All 191 women were tested for lupus anticoagulant (LA), antibodies (isotypes IgG, IgM) to cardiolipin (aCL), ß2-glycoprotein-1 (b2-GpI). Of these, 128 women had high level of aPL. The subpopulation of lymphocyte in aPL-positive women was compared with healthy women without reproductive pathology.In women with aPL, the absolute number of CD3T-regulatory cells play an important role in inducing tolerance to fetal alloantigens and limiting the intensity of the immune response. NK cells play an important role in processes of trophoblast invasion and spiral artery remodeling. Significantly reduced level of T-cells found in women with aPL may be associated with insufficient decidualization of endometrium for embryo invasion, which is clinically manifested by IVF failure.

Failed vacuum-assisted delivery (VD) is associated with increased risk of maternal perineal trauma and neonatal morbidity. Knowledge of the risk factors related to failed VD is essential in the clinical decision-making.To elucidate the strength of association and the predictive accuracy of different ante-partum ultrasound parameters in predicting the risk of failed VD prior to the onset of Labor and to test the diagnostic performance of a multiparametric model including pregnancy and Labor characteristics, ante and intra-partum ultrasound in anticipating failed VD.Prospective study of consecutive singleton pregnancies complicated by VD undergoing a dedicated ultrasound assessment at 36-38 weeks of gestation. Head circumference (HC), estimated fetal weight (EFW) and subpubic angle and (SPA) were recorded before the onset of Labor. At the time of the VD, occiput position, head perineum distance (HPD) and angle of progression (AOP) were also recorded. Multivariate logistic regression and area under the curve (AUC) analyses were used to explore the strength of association and test the diagnostic accuracy of different maternal, Labor and ultrasound characteristics in predicting g failed VD.Four hundred eight pregnancies with successful and 26 with failed VD were included in the analysis. Fetuses experiencing failed VD had a larger HC (1.21 versus 1.07 MoM;Antepartum prediction of failed VD is feasible. HC, SPA but not EFW are independently associated and predictive of failed VD. Adding these variables to a multiparametric model including maternal and intrapartum ultrasound parameters improves the diagnostic accuracy for failed VD.

Due to the increase in life expectancy, today, most women live in a state of estrogen deficiency for more than a third of their life time. According to the recent document on the stages of aging of the female reproductive system (the STRAW+10 working group), there are three main stages that include the reproductive period, the menopausal transition, and postmenopause. One of the most common manifestations of postmenopause is vulvovaginal atrophy or genitourinary syndrome, which leads to a dramatic decrease in the woman’s quality of life. Up to 45 % of postmenopausal women suffer from this disorder, and 80 % of them experience a negative impact on their quality of life. The main symptoms of vulvovaginal atrophy are dryness, itching, burning, and dyspareunia. Caution must be exercised in relation to “silent” atrophy, which is not manifested by subjective complaints of vaginal mucosa atrophy, and can only be visualized by colposcopy. According to the recommendations of the International Menopause Society (IMS), updated in 2016, therapy should be started before the atrophic changes become irreversible; the treatment should continue for a long time to maintain the achieved therapeutic effect. If no symptoms other than vulvovaginal atrophy are noted, local estrogen therapy is indicated. Estriol has advantages over other medications due to its lower affinity for estrogen receptors. In earlier studies as well as those updated here (2014–2018), estriol applied topically as a vaginal cream or vaginal suppositories, has a selective effect on the urogenital tract and practically does not interfere with the proliferation of the uterine endometrium. The confirmed efficacy and safety of estriol allow for its permanent use, beginning with the starting dose and then switching to the maintenance one.

Using illustrative cases of two patients with ovarian cancer who developed unusual thrombosis in pregnancy, to describe the difficulties in diagnosing and managing such patients.We present the analysis of two cases of thrombosis of unusual localization (central artery of the retina and transverse and sigmoid sinus thrombosis) during pregnancy in women with hidden ovarian cancer.In both cases, the symptomatic of unususal thrombosis was interpreted as a manifestation of eclampsia, leading to a choice of non-optimal obstetric tactics on a background of hidden ovarian cancer, which in both cases were diagnosed only postpartum.Pregnancy itself is a risk factor for thrombotic complications and in case malignancy develops in parallel with pregnancy, this risk is multiplied. In patients with ovarian cancer, difficulties arise both in detecting the tumor itself due to the enlarged uterus and in interpreting some of the symptoms, which can be regarded as a manifestation of pregnancy complications.

Introduction.Human papillomavirus infection (HPV-infection) remains one of the most important health problems as it significantly reduces the quality of life and stigmatizes the patients. Also, the prevalence of cervical cancer – the most severe outcome of the HPV-infection is 5 % of the global burden of cancer. Although vaccination against human papillomavirus has been proved efficient, its availability in Russia continues to be limited. Therefore, it is important to review other methods of HPV-infection control. A number of studies have confirmed the efficacy of glucosaminylmuramyl dipeptide (GMDP) in the treatment of diseases associated with HPV-infection, but no systematic evaluation of these studies has been published in the available literature.Aim:to analyze the data on the efficacy and safety of GMDP in the treatment of diseases and conditions associated with HPVinfection.Materials and methods.We used the PRISMA approach. The search for the relevant publications was conducted in international scientific databases: the Scientific Electronic Library, the Google Scholar, the ScienceDirect, the Cochrane Community Library, the Pubmed/MEDLINE, and clinical research registries. For this systematic analysis, only full-text publications were used. We evaluated the reliability of evidence and the methodological quality of the studies.Results.We used the following search queries: "glucosaminyl-muramyl dipeptide", "glucosamine L'muramyl dipeptide", "H-acetylglucosaminyl-H-acetylmuramyl dipeptide", "GMDP", "Licopid" (both in Russian and English transcriptions). Based on the results of the screening, 14 full-text publications were selected. At the final stage, review articles with secondary data were excluded; also excluded were original articles published in doubtful resources and those with an unclear status of peer reviewing. This systematic analysis includes 7 publications of acceptable methodological quality. Here, we summarize the consistent conclusions derived from these reports: the addition of therapy with GMDP to local (surgical) methods increases the efficacy of treatment and the duration of remission; destruction of condylomas is more effective when combined with the course of GMDP as compared to using the local destruction alone; GMDP enhances the production of cytokines that have a direct antiviral and antiproliferative effect in HPV-infection (interleukin-1, tumor necrosis factor alpha, gamma-interferon); GMDP causes normalization of cellular and humoral immunity (T-lymphocytes, T-cytotoxic lymphocytes, B-lymphocytes, CD3+, CD4+, CD8+, CD16+, and CD72+ lymphocytes, as well as the production of serum immunoglobulins IgA, IgG, and IgM). A high safety profile of GMDP is evidenced from the absence of reports on adverse events.Discussion.The recommendation for the inclusion of GMDP into a comprehensive treatment for HPV-infection in addition to local interventions is a strong recommendation. The differences between the Russian and international approaches can be explained by the difference in the available resources and funding. We propose to test whether using GMDP for reducing the risk of recurrent HPV-infection is beneficial in terms of pharmacoeconomics. Conclusion. The high efficacy and safety of GMDP in the combined therapy of HPV-infection has been confirmed. Further carefully designed studies on GMDP are needed.

In this short communication, the editorial board publishes the information on safety of the combined hormonal contraceptive (CHC) Jeanine®, containing the fixed dose combination of dienogest and ethinyl estradiol. This information has been received from the Russian regulatory authority (Roszdravnadzor) and the manufacturer. According to a metaanalysis of four observational studies, women using the dienogest/ethinyl estradiol fixed dose combination had a higher risk of developing venous thromboembolism (VTE) as compared with women taking the CHCs containing the levonorgestrel/ ethinyl estradiol combination. The estimated risk of developing VTE in women taking the dienogest/ethinyl estradiol combination is 8-11 cases of VTE per 10,000 women per year, while in women taking the CHCs containing levonorgestrel, norethisterone or norgestimate the risk is 5-7 cases of VTE per 10,000 women per year. A patient information leaflet amendment is under consideration by the regulatory authorities. For now, however, hormonal contraceptives containing the dienogest/ethinyl estradiol combination accompanied with the previous version of the instruction are still available in pharmacies. Therefore, when prescribing the combined hormonal contraceptives with dienogest and ethinyl estradiol, the obstetrician-gynecologist should discuss these risks with the woman.

Progesterone is a hormone responsible for pregnancy maintenance and the amount of progesterone increases in a woman’s body during pregnancy, as well as the level of female sex hormones, estrogens are also upregulated. Due to these changes the cutaneous sensitivity to external stimuli (meteorological factors, bacteria, etc.) increases. In general, all skin changes during pregnancy can be divided into three groups: physiological changes (hormone-associated), nonspecific or dermatoses that existed before pregnancy or were triggered by it, and specific pregnancy-related dermatoses, which appear during pregnancy and resolve in the postpartum period. In this brief review, we describe the dermatoses commonly seen in pregnancy and present our own clinical examples. We hope the review will be of some practical help for dermatologists and obstetricians.

Background: Antiphospholipid antibodies (aPL) have a multifaceted effect on the hemostatic system, damaging all its protective links.Aim: To study the effect of APA on outcomes of assisted reproductive technologies (ART).Study design: We examined 267 women with infertility, who planned pregnancy using ART. They included 178 women with IVF failure (I group) and 89 women with pregnancy after the IVF program (II group). The comparison group consisted of 80 pregnant women after IVF (male factor); a control group included 80 pregnant women with physiological pregnancy. Results of study demonstrated a high frequency of aPL circulation in a group of women with IVF failures. Overall, the proportion of aPL among all 267 women who planned pregnancy with ART was 32.6%. Elevated levels of aPL in the structure of causes of IVF failures (group I) were observed in 42.1% of them. Among women whose pregnancy occurred with ART (II group) the rate of APA was 19.1%. In the comparison group, in 6.3% of cases, aPL circulation was observed. In the control group, the rate was 3.4%.Conclusion: Considering the high percentage of aPL circulation in the case of IVF failures, authors think that high titers of aPL are a temporary contraindication for IVF. Patients with a history of aPL circulation are required to receive anticoagulant therapy from the first days of the hormonal protocol. The drug of choice is a group of low molecular weight heparins (LMWH). An individual approach is extremely important with the possible identification of causes of IVF failures and selective therapy, which leads to a significant improvement in the outcomes of the IVF program.

ADAMTS-13 is a crucial metalloproteinase involved in liberating fragments of von Willebrand factor (vWF) into the plasma as well as regulating its activity by cleaving "ultra-large" multimers into smaller and less active counterparts. Many pathological conditions, including those emerged during pregnancy are characterized by increased level of vWF and decreased ADAMTS-13 activity. In this regard, it is necessary to monitor the levels of vWF and ADAMTS-13 activity to prevent thrombotic thrombocytopenic purpura (Moschcowitz disease) as one of the most severe forms of thrombotic microangiopathy.

International audience; For last months, humanity has faced a formidable unknown enemy, which is presented as a new coronavirus infection. Despite the fact that the causative agents of new diseases appear at a certain frequency and that the virus SARS-CoV-2 has certain common properties with its predecessors, at the moment we are dealing with a new unknown pathogenesis of the development of severe complications in patients with risk factors. A final understanding of pathological process mechanisms is the goal of the scientific community. Summarizing research data from different countries, it became obvious that in severe cases of viral infection, we are dealing with a combination of the systemic inflammatory response syndrome, disseminated intravascular coagulation and thrombotic microangiopathy (TMA). Thrombotic microangiopathy is represented by a group of different conditions in which thrombocytopenia, hemolytic anemia, and multiple organ failure occur. The article reflects the main types of TMA, pathogenesis and principles of therapy. The main participants in the process are described in detail, including the von Willebrand factor and ADAMTS-13. Based on the knowledge available, as well as new data obtained from patients with COVID-19, we proposed possible models for the implementation of conditions such as sepsis, TMA, and DIC in patients with severe new coronavirus infection. Through a deeper understanding of pathogenesis, it will be possible to develop more effective diagnosis and therapy.

Marfan syndrome is dominantly inherited connective tissue diseases, associated with microfibrillar fiber connective tissue deficiency in vascular walls, heart valves, joints, dural membrane, bones, and other organs. Diagnosis may be confirmed by clinical criteria and molecular tests. Pregnancy bears a risk of fatal complications, including aortic dissection, aortic root dilation, mitral valve insufficiency. Women must be followed and managed in a high risk pregnancy unit by a team from obstetricians, cardiologists, anesthetists, pediatricians, neonatologists and cardiac surgeons who are skilled in high risk pregnancies. This approach allows to avoid the serious maternal and fetal complications. Caesarean section should be offered for such patients, because it minimizes the hemodynamic changes, associated with vaginal delivery. Some authors recommend to perform post-cesarean hysterectomy, because of the high incidence of massive hemorrhage in postpartum.

Background: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called “thrombotic storm” and multiple organ failure, that is, CAPS. Materials and methods: Since 2001–2016, we discovered 17 patients with CAPS development. Conclusion: CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%–40%.

The use of estrogen-containing hormonal drugs facilitates excretion of magnesium ions. It is therefore important to characterize women who use hormonal contraceptives, and to test correlation between the use of hormonal contraception -(HC) and the magnesium deficiency (MgD). Objectives. To create a profile of HC-using women who enrolled in the MAGYN study. Materials and Methods. The study included the disease registry (MgD in women with hormone-dependent diseases) and the data on safety and effectiveness of magnesium supplementation with a combination of magnesium citrate and pyridoxine. The standard questionnaire and biochemical determination of serum magnesium were used to diagnose MgD. The severity of MgD symptoms was evaluated using a 10-point visual analogue scale; the quality of life was assessed using the WHOQOL-BREF questionnaire after four weeks of therapy. Results. Among the total of 9168 women in the MAGYN study, hormonal contraceptives were used by 1528 women (16.7%). Of these women, combined estrogen-progestin single-phase contraceptives were used in 66.2% cases, a vaginal ring with a combination of estrogen and progestogen – 11.7%, and oral combined estrogen-gestagenic two-phase medications – 10.9%. The most common complaints among the women on hormonal contraception were "fatigue", "nervousness", "irritability", "state of chronic stress"; those symptoms were significantly more frequent in women with confirmed MgD. The prevalence of MgD in the group of women receiving hormonal contraception was 67%. Conclusion. The present study demonstrates high prevalence of MgD in women using hormonal contraception. Correcting MgD significantly improves the women’s health and their quality of life. Since the nutrition profile, behavior patterns, quality of life and social preferences of women may depend on urban population size, larger studies are warranted in cities with a population of >250 thousand to obtain the data relevant to the entire country.

The currently accepted role of hemostatic disorders (antiphospholipid syndrome, genetic thrombophilia) in the pathogenesis of frequent obstetric complications (preeclampsia, premature placental abruption, feto-placental insufficiency, recurrent miscarriage) revolutionized the prevention and treatment of these pregnancy-associated abnormalities. An increasing number of direct and indirect anticoagulants have been introduced into clinical practice. The present review addresses the mechanisms of action of dipyridamole, an antiaggregant medication commonly used in obstetrics. In addition to its antiplatelet activity, dipyridamole is also known for other pharmacological effects, including the antioxidant and endothelioprotective activities.

Objectives To describe umbilical vein (UV) hemodynamics at 11 + 0 to 13 + 6 weeks of gestation in pregnancies delivering a large-for-gestational-age (LGA) neonate, and to build a multiparametric model, including pregnancy and ultrasound characteristics in the first trimester, that is able to predict LGA at birth. Methods This was a matched case-control study, of singleton pregnancies that underwent ultrasound examination at 11 + 0 to 13 + 6 weeks for aneuploidy screening, at a single center over a 4-year period. Cases were women who delivered a neonate with birth weight (BW) > 90th centile for gestational age and sex, according to local birth-weight standards, while controls were those who delivered a neonate with BW ranging between the 10th and 90th centiles, matched for maternal and gestational age, at a ratio of 1:3. Each included case underwent Doppler assessment of the uterine arteries and UV, including measurement of its diameter, time-averaged maximum velocity (TAMXV) and UV blood flow (UVBF). UVBF and its components were expressed as Z-scores. Fisher's exact test and Mann-Whitney U-test were used to compare differences in maternal biomarkers and ultrasound characteristics between pregnancies complicated by LGA and controls. Logistic regression and receiver-operating-characteristics (ROC) curve analyses were carried out to identify independent predictors of LGA and to build a multiparametric prediction model integrating different maternal, pregnancy and ultrasound characteristics. Subgroup analysis was also performed, considering women who delivered a neonate with BW > 4000 g. Results In total, 964 pregnancies (241 with LGA at birth and 723 without) were included in the study. In LGA pregnancies compared with controls, UV-TAMXV Z-score (0.8 (interquartile range (IQR), 0.4-1.5) vs 0.0 (IQR, -0.3 to 0.5); P ≤ 0.001) and UVBF Z-score (1.3 (IQR, 0.8-1.9) vs 0.1 (IQR, -0.4 to 0.4); P ≤ 0.001) were higher, while there was no difference in median UV diameter Z-score (P = 0.56). Median uterine artery pulsatility index multiples of the median (MoM; 0.94 (IQR, 0.78-1.12) vs 1.02 (IQR, 0.84-1.19); P = 0.04) was significantly lower in LGA pregnancies. On multivariate logistic regression analysis, maternal body mass index (BMI; adjusted odds ratio (aOR), 1.2 (95% CI, 1.1-1.7); P 4000 g with an AUC of 0.83 (95% CI, 0.75-0.93). Conclusions UVBF measured at the time of the 11-14-week scan is associated independently with, and is predictive of, LGA and BW > 4000 g. Adding measurement of UVBF to a multiparametric model that includes maternal (parity and BMI) and biochemical (PAPP-A) parameters improves the diagnostic accuracy of prenatal screening for LGA at birth. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Membrane sweeping has been shown to potentially reduce the need for formal induction of labor. The primary aim of this study was to elucidate the role of ultrasound assessment of the cervix in predicting successful membrane sweeping in singleton pregnancies at term; the secondary aim was to build a multiparametric prediction model integrating, maternal, pregnancy and ultrasound characteristics, able to anticipate spontaneous delivery at term.Prospective observational study including singleton pregnancies at term undergoing membrane sweeping. Cervical length (CL) and posterior cervical angle (PCA) were assessed on ultrasound immediately before the procedure. Primary outcome was successful membrane sweeping, defined as spontaneous vaginal birth without formal induction within the 24hours. A subgroup analysis was computed considering women experiencing spontaneous vaginal birth within 48 hours from the procedure. The secondary outcome was to explore the diagnostic performance of a multiparametric model including maternal, pregnancy, and ultrasound assessment of the cervix in predicting spontaneous vaginal birth following membrane sweeping. Multivariate logistic regression and area under the curve (ROC) analyses were used to compute the data.One hundred fifty-nine singleton pregnancies undergoing membrane sweeping were included in the analysis. Successful membrane sweeping within 24 hours occurred in 68/159 women (36.5%). Parity (aOR = 1.87, 95% confidence interval [CI] 1.2-2.44), gestational age (aOR = 1.32, 95% CI 1.14-1.76), CL (aOR = 0.47, 95%CI 0.31-0.69) and PCA (aOR = 1.22, 95%CI 1.07-1.41) were independently associated with spontaneous vaginal birth within 24 hours from sweeping. The AUC of the constructed model was 0.796 (95% CI 0.727-0.865). Likewise, CL (aOR = 0.80, 95%CI 0.72-0.89), PCA (aOR = 1.19, 95%CI 1.10-1.28) and gestational age at the procedure (aOR = 1.65, 95%CI 1.09-1.86;Cervical ultrasound assessment of the cervix prior to membrane sweeping is associated with spontaneous vaginal birth within 24 and 48 hours from the procedure. The combination of cervical ultrasonographic parameters with parity and gestational age can predict the chances of delivery within 24 or 48 hours from membrane sweeping. The findings from this study support the use of ultrasound assessment of the cervix prior to membrane sweeping in order to more accurately predict the likelihood of spontaneous vaginal delivery.

The article considers the reasons and the role of the folate deficiency in development of the complicated pregnancy and congenital malformations. The biological functions of folate in an organism are described, as well as the role of folate in an exchange of methionine and a hyperhomocysteinemia as one of the reasons of malformations and complications of pregnancy. The role of a genetic mutation of MTHFR as the hyperhomocysteinemia reasons at patients with deficiency of folate and the treatment of the state of the deficiency of folate are discussed.

Heart rhythm disorders during pregnancy sifnificantly increase the risk of the cardioembolism in terms of the hypercoaguability state of pregnant women. The presence of atrial fibrillation during pregnancy in conditions of additional risk factors, inculding history of stroke and thromboembolic attacks, artifial heart valves, arterial hypertension, diabetes mellitus, ischemic disease or heart failure require anticoagulant therapy during all pregnancy. Acquired or genetic forms of thrombophilia also can increase the risk of thromboembolism in women with cardiac arrhythmias during pregnancy.

The prevalence of HPV in Russia and the world is quite high, although epidemiological data are limited because morbidity rate of papillomatosis, particularly genital warts, are not clear. The most effective method of preventing infection is vaccination against HPV infection. One of the most effective preventive measure to reduce the risk of death from HPV-related cancer diseases is a regular screening. None of the treatments of HPV infection will not totally effective and will not prevent disease transmission. In most cases, treatment is aimed at getting rid of symptoms but not cure infection. Immunomodulators for management of HPV-associated diseases could not have evidence base. Clinically proven treatment with inosine pranobex eliminates HPV to 98% cases and 3 times reduce frequency of relapses in combined therapy. Correct and complete counseling, aimed at informing the patient about the connection of HPV infection and cancer, as well as the possibility of preventing infection by timely vaccination and proper compliance are issue at present time. Isoprinosine saves a woman from the HPV, reducing the possibility of recurrence and malignancy, only when patient has complete course of therapy.

Thrombotic microangiopathy is one of the most serious thrombotic complications characterized by microvascular thrombosis in various organs and accompanied by thrombocytopenia and hemolytic anemia. The term thrombotic microangiopathy has incorporated several nosology, which are characterized by different mechanisms of microvascular thrombosis. Currently thrombotic microangiopathy include thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), heparin-induced thrombocytopenia, HELLP-syndrome. Pregnancy presents one of the key triggers to the development of thrombotic microangiopathy. This fact gives us a significant opportunity to study the pathogenesis of thrombotic microangiopathy in the context of the physiological changes of hemostasis during pregnancy. At the same time the discovery of molecular mechanisms of thrombotic microangiopathy allows for a new research on the field of pathogenesis of thrombotic complications associated with pregnancy, as well as the pathogenesis of so-called placental obstetric complications, including severe preeclampsia, premature detachment of normally situated placenta, septic shock.

Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders ...

We have observed the development of a catastrophic antiphospholipid syndrome (CAPS) in a pregnant woman hospitalized at 28 weeks of gestation with a severe preeclampsia. On the same day, an eclampsia attack developed, and an emergency surgical delivery was performed. On the third day, multiorgan failure developed. Examination showed a persistent circulation of lupus anticoagulant, high level of antibodies to cardiolipin, b2-glycoprotein I, and prothrombin. The usual diagnosis of the severe preeclampsia masked a catastrophic antiphospholipid syndrome, exacerbated by the coincident presence of several types of antiphospholipid antibodies. The first pregnancy resulted in a premature birth at 25 weeks, possibly also due to the circulation of antiphospholipid antibodies. The trigger of the catastrophic form development was the pregnancy itself, surgical intervention, and hyperhomocysteinemia. CAPS is the most severe form of antiphospholipid syndrome, manifested in multiple microthrombosis of microcirculation of vital organs and in the development of multiorgan failure against the background of the high level of antiphospholipid antibodies. CAPS is characterized by renal, cerebral, gastrointestinal, adrenal, ovarian, skin, and other forms of microthrombosis. Thrombosis recurrence is typical. Thrombotic microvasculopathy lies at the heart of multiorgan failure and manifests clinically in central nervous system lesions, adrenal insufficiency, and ARDS development. CAPS is a life-threatening condition, therefore, requires an urgent treatment. Optimal treatment of CAPS is not developed. CAPS represent a general medical multidisciplinary problem.

Сatastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome that results in a widespread coagulopathy and high titres os antiphospholipid antibodies and affects predominantly small vessels supplying organs with the development of multiorgan failure. International approaches on terminology, risk factors, pathogenesis, criteria of diagnostics, clinical symptoms and management of patients with CAPS are reviewed. Own clinical experience of management 17 patients with CAPS development also presented. CAPS is life-threatening condition but optimal treatment for CAPS is not developed yet. CAPS presents a multidisciplinary problem.