Novel coronavirus infection and Kawasaki disease.

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.

Thrombotic microangiopathy, DIC-syndrome and COVID-19: link with pregnancy prothrombotic state.

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.

Pregnancy and mesenchimal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia).

The objective of this article is to attract the attention of clinical physicians to the rare but extremely relevant clinical pathology of mesenchymal dysplasias (Marfan syndrome, Ehlers-Danlos syndrome, hereditary hemorrhagic telangiectasia) and especially specific characteristics of such diseases during pregnancy. Connective tissue pathology can cover different organs and systems, symptoms of the same disease can vary in different patients thus making diagnostics significantly difficult. Here clinical diagnostic criteria and methods of molecular diagnostics of diseases are described. The pathogenesis of mesenchymal dysplasias is not currently well understood. For the patients with mesenchymal dysplasias pregnancy is fraught with high risk of life-threatening complications. The preferred delivery method for such patients is caesarean section.

[Thrombosis of the cerebral veins in gynecological practice]. / Tromboz tserebral'nykh ven v akushersko-ginekologicheskoi praktike.

The review presents literature data on the risk factors of cerebral venous thrombosis (CVT), a rare life threatening disorder. Currently, the diagnosis of CVT is made more often due to the possibility of using neuroimaging methods of cerebral structures. Pregnancy, postpartum period, use of oral contraceptives are risk factors of CVT. A role of thrombophilia, in particular Factor V Leiden mutations and the prothrombin G20210A polymorphism, in the CVT pathogenesis is confirmed. The development of CVT is an example of the interaction between persistent risk factors increasing the likelihood of thrombotic complications and acquired risk factors. A focal neurological deficit during pregnancy, delivery and postpartum period is the best indicator of CVT diagnosis. Possibilities of the use of anticoagulants in treatment and prevention of CVT are analyzed.

[Pathogenesis and prevention of thromboembolic complications in obstetric practice].

The study was designed to analyse risk factors of venous thromboembolism (VTE), elucidate the role of genetic forms of thrombophilia and antiphospholipid syndrome (APS) in pathogenesis of VTE during pregnancy. 38 pregnant patients with VTE (group I) and 35 healthy pregnant women (group 2, controls) were screened for genetic thrombophilia and antiphospholipid antibodies (APA). Deep vein and ovarian vein thrombosis was diagnosed in 21 and 1 patients of group 1 respectively. VTE occurred in 15 on weeks 20-38 of pregnancy and in 12 in the postpartum period. 38 and 59% of the patients had VTE in their hereditary and obstetric histories respectively. Multigenic thrombophilia was recorded in all patients with VTE (4-6 mutations including homozygous ones), combined defects of fibrinolysis in 86%, and APA in 59%. Multigenic thrombophilia occurred in 20% of the healthy women while APA were not detected. It is concluded that thrombophilia may be an essential pathogenetic mechanism of thromboembolism during pregnancy. Women with pregnancy-associated thromboembolism, thromboembolism in personal or family history, and with the history of obstetric complications should be screened for thrombophilia.

[IVF failures: maternal thrombophilia as a possible cause].

It is studied the association between thrombophilia and failure to achieve pregnancy after IVF. The study group included 55 patients aged 24-45 years, with history of infertility I and II (unexplained genesis). 38 women were with a history of IVF failures and 17 -- without such history. The comparison group consisted of 30 women pregnant after IVF (male factor). The control group was comprised of 50 patients with physiological course of pregnancy. In the study group thrombophilia was verified in 90% of cases; in the group of comparison -- 30%; in control group -- 26% (p<0,05). In the study group the multigenic defects were verified in 90% of cases. The polymorphism of PAI-1 was found in 69% women with a history of IVF failures and in 35% in group without the history of IVF failures. In the group of comparison polymorphism was found in 10% of women and in control group -- 8% (p<0,05). In the study group differential antibiotic prophylaxis was conducted with the result of 36,4% viable pregnancy (20 women of 55). The endpoint for success was a live birth. These data suggest that inherited thrombophilia may play a role in the etiology of IVF failures. The differential antibiotic prophylaxis reduces the abortion rate but also lowers the risk for development retardation, premature birth and preeclampsia.

[Role of endothelial dysfunction and coagulation disorders in the development of pulmonary fibrosis in patients with interstitial lung diseases]. / Rol' éndotelial'noi disfunktsii i koagulologicheskikh narushenii v razvitii legochnogo fibroza u bol'nykh insterstitsial'nymi bolezniami legkikh.

Thirty-two patients with different forms of interstitial lung diseases (ILD), such as idiopathic fibrosing alveolitis (IFA) (n = 17) and fibrosing alveolitis concurrent with diffuse connective tissue diseases (FA-DCTD), were examined. Clinical, echocardiographic, computed tomographic, coagulative, and immunological studies were performed. Enzyme immunoassay was used to determine the levels of a complex of thrombin and antithrombin III (TAT) and platelet factor IV (PF-IV). There were significant increases in the levels of PF-IV (4.36 +/- 0.25 mg/l) and TAT (10.87 +/- 3.8 mg/l) in patients with ILD as compared to the control (2.75 +/- 0.47 and 1.8 +/- 0.2 mg/l, respectively; p < 0.05). In patients with early FA-DCTD with the predominance of the milk glass syndrome during high-resolution CT (HRCT), the level of PF-IV was greater than the normal levels (p < 0.05) and decreased with the progression of the disease and with the formation of the honeycomb lung. If there were HRCT signs of active inflammation, the level of TAT was higher than that in the control; this was also in the development of irreversible fibrous changes.

[Markers of thrombophilia in pulmonary fibrosis complicated by pulmonary hypertension]. / Markery trombofilii pri legochnom fibroze, oslozhnennom legochnoi gipertoniei.

AIM: To determine the role of enhanced blood coagulation in pathogenesis of pulmonary hypertension (PH) at an early stage of fibrosing alveolitis (FA). MATERIAL AND METHODS: Clinical, functional, roentgenological, coagulation and immunological examinations were performed in 17 patients with idiopathic FA (IFA), in 6 patients with exogenic allergic alveolitis (EAA), in 15 FA patients with diffuse diseases of the connective tissue (FA-DDCT). The diagnosis was verified with high resolution computed tomography (HRCT). Lesser circulation was assessed by Doppler echocardiography. Morphological impairment of the lungs was specified in all the patients using analysis of the bronchoalveolar lavage. In 9 FA patients the diagnosis was verified at thoracoscopic biopsy of the lung. The control group consisted of 16 healthy volunteers. Thrombin-antithrombin complex (TAT) and thrombocytic factor 4 (TF-4) were estimated with ELISA as stable, highly sensitive markers of thrombophilia. RESULTS: The TF-4 level was elevated in all IPD patients (p < 0.05), the elevation being highest in FA-DDCT (p < 0.007). With FA progression, TF-4 concentration went down. A weak negative correlation (p < 0.047, r = -0.38) was found with average pressure in the pulmonary artery (PAAP). TAT was higher than control in all the groups (p < 0.05). Maximal TAT values were registered in EAA. If HRCT detected active inflammation and in development of irreversible fibrous changes TAT was higher vs control. A direct correlation between TAT level and PAAP was not found. CONCLUSION: Disorders in thrombocytic and plasmic links of hemostasis are detectable early in IPD. Stable markers of thrombophilia (TAT and TF-4) reflect activity of inflammation in FA. They can be also used as sensitive diagnostic tests for diagnosis of PH and diagnosis of patients with activated coagulation system in IPD.