A5020338512- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Blood Coagulation and Thrombosis Mechanisms
- Hemostasis and retained surgical items
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Blood properties and coagulation
- Heparin-Induced Thrombocytopenia and Thrombosis
- Cancer-related gene regulation
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Erythrocyte Function and Pathophysiology
- Chronic Myeloid Leukemia Treatments
- Blood groups and transfusion
- Parathyroid Disorders and Treatments
- Antiplatelet Therapy and Cardiovascular Diseases
- Blood disorders and treatments
- Venous Thromboembolism Diagnosis and Management
- Blood transfusion and management
- Case Reports on Hematomas
- S100 Proteins and Annexins
- Blood donation and transfusion practices
- Intramuscular injections and effects
- Protein Kinase Regulation and GTPase Signaling
- Central Venous Catheters and Hemodialysis
- Trauma, Hemostasis, Coagulopathy, Resuscitation
Ministry of Health of the Russian Federation
2015-2024
National Medical Research Center for Hematology
2010-2024
Russian Academy of Sciences
2012
Academy of Medical Sciences
2010
Concizumab is an anti-tissue factor pathway inhibitor monoclonal antibody designed to achieve hemostasis in all hemophilia types, with subcutaneous administration. A previous trial of concizumab (explorer4) established proof concept patients or B inhibitors.We conducted the explorer7 assess safety and efficacy inhibitors. Patients were randomly assigned a 1:2 ratio receive no prophylaxis for at least 24 weeks (group 1) 32 2) nonrandomly (groups 3 4). After treatment pause due nonfatal...
Development of neutralizing inhibitors against factor VIII (FVIII) is a major complication haemophilia A treatment.The ongoing, international, open-label, uncontrolled, observational immune tolerance induction (ObsITI) study evaluates ITI, the standard care in patients with inhibitors.Forty-eight prospective this interim analysis received single plasma-derived, von Willebrand factor-stabilized, FVIII concentrate (pdFVIII/VWF) for ITI. According to recommended Bonn protocol, 'low responders'...
BackgroundCongenital fibrinogen deficiency is an ultra‐rare disorder in which patients can experience severe and/or frequent bleeding episodes (BEs). Here, we present the largest prospective study to date on treatment of this disorder.MethodsHemostatic efficacy human concentrate (HFC; FIBRYGA®, Octapharma AG) for or surgical prophylaxis was assessed by investigators and adjudicated independent data monitoring endpoint adjudication committee (IDMEAC) according a four‐point scale, using...
We aimed to characterise baseline disease and treatment burden in a large population with haemophilia A/B, both (HAwI/HBwI) without (HA/HB) inhibitors.
Objective. To present the current stock of knowledge about perioperative complications splenectomy for hematological disorders. Key issues. The use minimally invasive technologies in surgery has reduced incidence intra- and postoperative complications. However, both laparoscopic open are associated with technical difficulties patients blood diseases. Splenectomy is significantly complicated by severe splenomegaly, perisplenitis, portal hypertension, hemostatic disorders, severity disease...
Fibrinogen concentrate is the preferred choice for fibrinogen replacement in congenital deficiency. This study investigated hemostatic efficacy of a new plasma-derived, double virus-inactivated (using two dedicated virus inactivation/elimination steps) human on-demand treatment bleeding episodes (BEs) and surgical prophylaxis.In this planned interim analysis prospective, multinational Phase III (NCT02267226), 13 patients with afibrinogenemia (≥12 years) received (FIBRYGA, Octapharma AG)....
Recombinant activated factor VIIa (FVIIa) is a bypassing agent used to treat bleeding episodes in haemophilia patients with inhibitors VIII (FVIII) and IX. The pharmacological effect of FVIIa short-lived therefore the recommended dose 90 μg kg(-1), episode treated multiple injections. A long-acting form that can ensure adequate haemostasis single infusion, without increasing thrombotic risk, would be beneficial. PEGylated liposomes (PEGLip) have been shown bind improve haemostatic efficacy...
The next frontier in hemophilia A management has arrived. However, questions remain regarding the broader applicability of new and emerging therapies, such as long-term safety efficacy non-factor therapies optimal regimens for individual patients. With an ever-evolving clinical landscape, it is imperative physicians to understand how available future could potentially be integrated into real-life practice improve patient outcomes. Against this background, nine experts from Central European...
Introduction Haemophilia A patients are at a high risk of excess bleeding during surgeries. The aim haemostatic therapy the perioperative period is to normalize FVIII level perioperatively and postoperatively maintain normal haemostasis until wound healing complete. Aims/Methods To examine efficacy Nuwiq ® (simoctocog alfa, human‐cl rh ), 4 th generation recombinant produced in human cell line, for surgical prophylaxis with severe haemophilia A. This analysis assessed procedures...
Introduction . Haemophilia and von Willebrand disease constitute the most common hereditary coagulopathies. However, such rare coagulopathies as congenital factor V defi ciency can mistakingly be referred to these diseases. Aim To describe clinical manifestations treatment of ciency. General findings The article presents a literature review, well three case studies patients with deficiency. Given that choice haemostatic therapy depends on accurate diagnosis, issues associated differential...
Abstract Aim To test a novel method of assessment platelet adhesion to fibrinogen‐coated surface in whole blood under flow conditions. Methods We developed fluidic device that mimics vessels. The detection is based on recording scattered laser light signal from fibrinogen‐covered surface. Testing was performed platelet‐rich plasma (PRP) and healthy volunteers. Control measurements were performed, followed by tests with inhibition GPIIa/IIIb GPIb receptors. Then, the same testing sequence...
Heparin-induced thrombocytopenia (HIT) is a serious and potentially life-threatening side effect of heparinotherapy. It an antibody-mediated process that causes platelet activation, increases the procoagulant characteristics blood and, as result, endangering limbs thrombosis. Venous thrombosis more common than arterial thrombosis, especially deep vein lower pulmonary artery Mortality from complications heparinotherapy occurs with frequency 20–30 % cases. Diagnosis HIT difficult. Such basic...
Abstract Background Immune tolerance induction (ITI) with repeated factor VIII (FVIII) administration is the only strategy proven to eradicate inhibitors. The observational ITI study evaluating a range of FVIII products. Methods This subgroup analysis reports prospective interim data for patients treated plasma-derived, von Willebrand factor-stabilized concentrate (pdFVIII/VWF, octanate). Complete success (CS) required achievement three criteria: inhibitor titer < 0.6 BU/mL; recovery ≥...
Plasma fibronectin is a high molecular weight adhesive glycoprotein. There are two types of fibronectin: plasma (soluble) and cellular derived (insoluble). Electron microscopy revealed structural organization compact expanded. In solution, has conformation, after binding to certain substrates (collagen, fibrin, heparin), it one the main opsonins blood in relation “targets” phagocytosis predominantly non-bacterial nature, as well some bacteria. For treatment septic processes, respiratory...
Hemophilia A (HA) is one of the most widespread, X-linked, inherited bleeding disorders, which results from defects in F8 gene. Nowadays, more than 3500 different pathogenic variants leading to HA have been described. Mutation analysis essential for accurate genetic counseling patients and their relatives. We analyzed 273 unrelated families with forms HA. The consisted testing intron inversion (inv22 inv1), then sequencing all functionally important gene fragments. identified 101 267...
Background . Internationally published data about the course of COVID-19 in patients with congenital bleeding disorders (CBDs) are limited. There questions how affects CBDs and, conversely, affect coronavirus infection. Aim — to analyze Russia. Materials and methods A cross-sectional survey was conducted at National Medical Research Center for Hematology (Moscow) period from June 25 July 31, 2022. cluster 187 different regions cities Russia were interviewed a questionnaire based on...
Coagulation factor VII (proconvertin) is one of the proteins starting blood coagulation cascade. Plasma FVII concentration regulated by different factors. A low level could also be a result deficiency (MIM# 227500), rare autosomal recessive inherited disease caused pathogenic variants in F7 gene. The aim this study was to describe mutation spectrum gene and genotype–phenotype relationship patients with Russia for first time. We studied primary structure 54 unrelated direct Sanger sequencing....
The implementation of the state program “7 highcost nosologies” and active work Russian hematologists have significantly improved specialized care for children adults with Hemophilia. hemophilia patient registry as 10.25.2018 contained information about 7433 patients, whom A – 6525 people. About 400 people were diagnosed inhibitors. inhibitor predominantly appeared at child young age (up to 20 years). There is a high supply coagulation factors concentrates treatment in Federation 8.1 IU...
Introduction. Cryosupernatant is blood component. the supernatant plasma removed during preparation of cryoprecipitate. Aim. To provide information on composition and methods production, storage, transportation clinical use Cryosupernatant. General fi ndings. In comparison with fresh frozen (FFP) cryoprecipitate, depleted in factor VIII, brinogen von Willebrand (VWF). defi cient high molecular weight multimers VWF, but contains VWF metalloproteinase. The concentrations V, antithrombin III,...
To determine the features of preoperative hemostatic therapy and laboratory control in patients with severe inhibitory forms hemophilia A under preventive treatment by FVIII.Four underwent surgery between 2021 2022. All received Emicizumab (the first monoclonal drug for «non-factor» hemophilia) prevention specific hemorrhagic signs hemophilia.Surgical intervention was essential. Additional not carried out or performed reduced mode. There were no hemorrhagic, thrombotic other complications....