A5047257964- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Human Health and Disease
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Blood Coagulation and Thrombosis Mechanisms
- Chronic Myeloid Leukemia Treatments
- Hemostasis and retained surgical items
- Antiplatelet Therapy and Cardiovascular Diseases
- Congenital Ear and Nasal Anomalies
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Vascular Malformations Diagnosis and Treatment
- Cancer-related gene regulation
- Biochemical and Molecular Research
- Heparin-Induced Thrombocytopenia and Thrombosis
- Vascular Malformations and Hemangiomas
- Muscle metabolism and nutrition
- Chemistry and Chemical Engineering
- Photopolymerization techniques and applications
Kirov Research Institute of Hematology and Blood Transfusion FMBA
2017-2024
Federal Medical-Biological Agency
2021
Kaluga State University named after K.E.Tsiolkovski
2019
State Research Institute of Highly Pure Biopreparations
2019
Pirogov Russian National Research Medical University
2013
Current hemophilia treatment involves frequent intravenous infusions of clotting factors, which is associated with variable hemostatic protection, a high burden, and risk the development inhibitory alloantibodies. Fitusiran, an investigational RNA interference (RNAi) therapy that targets antithrombin (encoded by SERPINC1), in to address these other limitations.
Fitusiran, an investigational small interfering RNA therapy, reduces antithrombin production to rebalance hemostasis in people with hemophilia A or B, without inhibitors.To evaluate the safety and efficacy of fitusiran treatment for moderate/severe B inhibitors.In this open-label phase 1, part D study, 17 males inhibitors received three once-monthly subcutaneous injections 50 mg (n = 6) 80 11); followed up 112 days. Endpoints included (primary), pharmacokinetics/pharmacodynamics (secondary),...
Essentials Recombinant von Willebrand factor (rVWF) is effective in disease (VWD). A phase 3 study of rVWF, with/without recombinant VIII (rFVIII) before surgery VWD. Overall rVWF's efficacy was rated excellent/good; rVWF administered alone most patients. well-tolerated and hemostasis achieved patients with severe VWD undergoing surgery. SUMMARY: Background has demonstrated for on-demand treatment bleeding (VWD), warranting evaluation the surgical setting. Objectives This (NCT02283268)...
Abstract Long-term prophylaxis with a von Willebrand factor (VWF) concentrate is recommended in patients disease (VWD) who have history of severe and frequent bleeds. However, data from prospective studies are scarce. WIL-31, prospective, noncontrolled, international phase 3 trial, investigated the efficacy safety Wilate VWD. Male female 6 years or older VWD types 1, 2 (except 2N), had completed 6-month, on-demand, run-in study (WIL-29) were eligible to receive for 12 months. At baseline, (n...
Abstract Introduction SHP656 is the first factor VIII (FVIII) product developed using polysialylation (PSA) technology, in which full‐length recombinant (r) FVIII (anti‐haemophilic [recombinant]) conjugated with a 20 kDa PSA polymer. Aim To compare safety, immunogenicity and pharmacokinetics of vs parent rFVIII (octocog alfa) after single infusions 25‐75 IU/kg patients severe haemophilia A (FVIII activity <1%). Methods Multinational, phase 1, prospective, open‐label, two‐period,...
The main method of treatment for hemophilia A is replacement therapy with drugs blood coagulation factors VIII (FVIII). active use biotechnological methods in the production recombinant contributes to development and registration new FVIII drugs. results clinical trials children usually include a limited number patients specific period time. Post-marketing observational studies provide additional information on using drug practice. objectives study were collect analyze data efficacy safety...
Relevance . In accordance with the guidelines on clinical investigation of clotting factor VIII products European Medicines Agency and pharmacovigilance Eurasian Economic Union, after registration a new drug, it is recommended to study its efficacy safety large population patients in standard medical practice clarify identify data. Materials methods prospective, multicenter, open-label, uncontrolled observational study, domestic recombinant B-domain deleted blood FVIII (FVIII) (moroctocog...
The paper presents the study results of dynamics in erythroid indices peripheral blood children early postnatal period ontogenesis. All them were healthy, showed no developmental defects. Based on biometric analysis data obtained, significance genetic and environmental factors identified was determined. It largely caused by influence natural cosmophysical processes during children's development years, such as an increase indicators various types radiation basic indexes solar activity. We...
The aim of the study was to evaluate efficacy, safety and pharmacokinetics nonacog alfa (Innonafactor) in children aged 2 12 years with hemophilia B. Materials methods: 15 patients (7.7±2.5 ) were included an open, multicenter, prospective, non-comparative clinical trial, whom 6 3 years. efficacy drug assessed against background introduction 45±10 IU/kg times a week interval 72–96 hours, by frequency nature adverse reactions. Results: 5 (22%) spontaneous 18 (78%) post-traumatic bleedings...
Currently, the main method of treatment for hemophilia A is replacement therapy with drugs blood coagulation factors VIII (FVIII). As a result development new production technologies, recombinant FVIII are increasingly used A. The justification use in pediatric clinical practice requires careful preparation and research studies their efficacy safety. aim this study was to evaluate efficacy, safety pharmacokinetics (PK) domestic B-domain deleted factor Moroctocog alpha (Octofactor, GENERIUM...
The purpose of the study was to assess efficacy, safety and pharmacokinetics moroctocog alfa (Octofactor) in children aged 2-6 with haemophilia A. Materials methods research : six patients between 2 6 years age (average 4.3±0.8 years) were included open multicenter prospective clinical trial. efficacy drug assessed against background introduction 30±10 IU/kg every 2–3 days, by frequency causality adverse reactions. Results: 7 post-traumatic bleeding registered. average prophylactic dose is...
Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder.Diagnosing VWD challenging as factor levels are influenced by a multitude of variables including ABO blood type, age, stress induced hypothalamic-pituitary-adrenal axis changes, and environmental factors such inflammation hormone exposure.An association between hemoglobin, von Factor (VWF), VIII activity has been described in single institution studies but further study needed.Aims: To investigate...
We studied manifestation peculiarities of 17-hydroxyprogestron in the morning blood children early neonatal period development. An assessment hormone parameters variation depending on time their birth implementation genetic and environmental factors effects was carried out. The intergroup differences 17-OHProgesterone concentration determined by them were not always associated with somatic growth indices variability. presented data indicating that this dependence regulated at level growing...
Чернова Г.В. 1 , Сидоров П.В. Ергольская Н.В. Тимофеева М.А. Петросян В.В. Ширяева Л.В. 2 Сопряженность изменчивости уровня гормонов разной функциональной направленности в крови новорожденных и показателей их физического развития как отражение взаимодействия генетических средовых факторов Научно-образовательный центр биофизических исследований ФГБОУ ВО «Калужский государственный университет им.К.Э.Циолковского» ГБУЗ КО «Детская городская больница» (Россия, Калуга) doi:...