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Information × Registration Number 0218U000572, 0115U000127 , R & D reports Title To investigate the type inhibitors of coagulation and their pathogenesis in patients with congenital and acquired hemophilia, and antiphospholipid syndrome popup.stage_title Head Loginsky Volodymyr Eustakhovych, Доктор медичних наук Registration Date 02-02-2018 Organization State Institution Institute of Blood Pathology and Transfusion Medicine of National Academy of Medicfl Sciences of Ukraine popup.description2 The subject of the study - 395 patients (aged 6 months to 79 years), of which 61 patients with hemorrhagic syndrome, 101 patients with hemophilia (96 - hemophilia A, 5 - hemophilia B), 233 patients with suspected AFS. All patients were examined and treated in the SI IBPTM NAMS of Ukraine (Lviv). Aim: based on the study of kinetics of pathological inhibitory antibodies in the system of coagulation in response to neutralization of normal plasma, to establish the type of inhibitor and its pathogenetic and prognostic importance in the clinical course of the disease and the effectiveness of treatment of patients with hemophilia, immune coagulopathy and patients with AFS. Clinical and laboratory studies included clinical examination, clinical-genealogical, molecular genetic, cytological, cytogenetic, hormonal, hematological, immunological, biochemical and general coagulation studies. Hematology Analyzer Rybu, No. 36688BG, Biochemical Analyzer Pentra 400, No. 807P4-2012, Semiautomatic Coagulometer Helena-C4, No. C4-2489 were used. As a result of the studies,61 patients with hemorrhagic syndrome and isolated prolonged APTTT in 34.4% were diagnosed with hemophilia A, in 11.5% of hemophilia B, in 13.1% of hemophilia A with the inhibitor of FVIII, in 6.5% of acquired hemophilia A, in 1.6% acquired hemophilia B, in 4.9% Willebrand disease, in 27.9% AFS. An algorithm for laboratory diagnosis of hemostasis disorders in patients with isolated elongated APTT has been developed, which includes two types of correction tests and refinement tests, which allow to diagnose factor deficiencies and differentiate progressive alloimune inhibitor type I with an immediate autoimmune type II inhibitor. In patients with hemophilia with an inhibitor of FVIII (IX), the number of "target" articulations is directly related to the titre of the inhibitor, the proportion of spontaneous bleeding and the consumption of the preparation rFVIIa. Patients with LA have a direct correlation with the titre of the inhibitor and the frequency of all bleeding and reverse with the proportion of spontaneous bleeding and the number of "target" joints. Of the 233 patients with AFS suspected in 104 patients (44.6%), were diagnosed with AFS, of which primary - in 89 (85.6%) patients, and 15 (14.4%) - secondary AFS. LA Diagnosis can be performed in 2 stages, the most informative is achieved in the case of combination of tests. Product Description popup.authors Дзісь Р.П. Красівська В.В. Семерак М.М. Стасишин О.В. Тушницький О.М. popup.nrat_date 2020-04-02 Close