Registration Number
0216U000345,
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
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Head
Loginsky Volodymyr Eustakhovych, Доктор медичних наук
Registration Date
29-01-2016
Organization
LVIV RESEARCH INSTITUTE OF BLOOD PATHOLOGY AND TRANSFUSIONAL MEDICINE
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Object of study were 101 patients aged 3 to 62 years, including 96 patients with hemophilia A, 5 patients with hemophilia B, who were hospitalized in the clinic and outpatient department of SI "Institute Blood Pathology and Transfusion Medicine of NAMS of Ukraine ". Aim: To set the type of inhibitor and its pathogenetic and prognostic value in the clinical course of the disease and the effectiveness of treatment of patients with hemophilia, immune coagulopathy and patients with antiphospholipid syndrome (APS) by investigation the kinetic of inhibitory antibodies in response to adding normal plasma. Clinical and laboratory studies included clinical examination and tests of coagulation, hematology, biochemical. We used Micros 60 hematology analyzer T number 81473, biochemical analyzer Point + 180, SR 1907.1674, semi-automatic coagulometer Helena-C4 S4-2489 number. Results: screening test as the first stage of diagnosis factor VIII (IX) inhibitors in patients with hemophilia informative and its perform is appropriate. In case of a positive result must perform Bethesda test. Test of correction APTT by normal plasma in increasing concentrations before and after incubation for 1 h in patients with hemophilia allow to distinguish factor VIII (IX) deficiency and identify specific neutralizing activity progressive inhibitor type I and autoimmune immediate inhibitor type II. It was established that the I, II and III stages of sequential algorithm for detecting lupus anticoagulant (LA) in patients with hemophilia tests that characterize the activity of prothrombin complex are informative and suitable for the diagnosis, thanks to their high diagnostic sensitivity and specificity. The amount of "target" joints in inhibitory hemophilia patients positive correlated with inhibitor titer, annual spontaneous bleeding rate (AsBR) and FVIIa consumption. The direct correlation between inhibitor titer and frequency of bleeding was established in patients with LA. AsBR and quantity of "target joints " were decreased in patients with LA.
Product Description
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Дзісь Р.П.
Красівська В.В.
Стасишин О.В.
Тушницький О.М.
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2020-04-02
