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The article presents ultrasonic characterization of hemophilic synovitis in 73 hemophilia patients. The technology of performing rifampicin synoviortesis under ultrasonic control has been developed, ensuring the exact fulfilling intraarticular punctures taking into account the alterations in the intraarticular structures and optimization of filling the articular cavity with sclerosant. The effectiveness of the developed method was 93.7% in the 3 years of follow-up observations.

To study the role of genetic determinants of hereditary thrombophilia in pathogenesis of various clinical manifestations of venous thrombosis in the citizens of the North-West Region of Russia.Mutations of the genes of factor V (FV Leiden), prothrombin (G20210-A) and polymorphism C677-T in the gene of methylentetrahydrofolate reductase (MTHFR) were detected using polymerase chain reaction (PCR) with a following restriction analysis of PCR product in 183 patients with venous thrombosis (115 with isolated thrombosis of the deep veins and 68 with thromboembolism of the pulmonary artery).It was established that mutation FV Leiden is a significant risk factor of deep vein thrombosis in the legs and postthrombotic disease, but this mutation is weakly associated with pulmonary artery thromboembolism (PAT). An essential PAT risk factor is carriage of the variant prothrombin G20210A.Determination of prothrombotic genotypes is a key factor of treatment efficacy and prevention of life-threatening thromboembolic complications.

To study the incidence of mutation of Leyden's factor V in patients with venous thrombosis and the hemostatic system in carrier of this genetic defect.A hundred and one patients aged 15-69 years who had venous thrombosis and 10 individuals with mutation of Leyden's factor V without manifestations in the history of thrombosis were examined. Factor V gene mutations and the thrombocyte and plasma links of hemostasis were determined by routine methods.The Leyden's factor V genotype Arg506--Gln was detected in 17 of the 101 patients with venous thrombosis. Patients and asymptomatic individuals with this factor were found to have significant hypercoagulation, as evidenced by lower activated protein C-resistance index, higher factor VIII (von Willebrand's factor) activity, elevated von Willebrand's factor antigen levels, and enhanced intravascular platelet activation. In the presence of lupoid anticoagulant, hypercoagulation increased and protein C activity decreased.Detection of signs of hypercoagulation in patients with inherited thrombophilia at recovery in carriers of Leyden's factor V without clinical manifestations of thrombosis shows it necessary to make a particularly careful monitoring of the hemostatic system in these subjects. This is especially important for hypercoagulation-predisposing situations, such as pregnancy, surgical interventions, long-term immobilization, use of contraceptives, etc. when preventive measures may be used to prevent thrombotic events.

The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy.GAG concentration, composition and fraction identification were made in 25 patients with hemophilia A and 10 patients with Willebrand disease.In hemophiliacs, leukocytes contained low concentrations of GAG. In those with bleeding and synovitis GAG levels were lower than the average, in those with extensive hematomas in the absence of locomotor disorders the above levels were close to normal. Chondroitinsulphate dominated in GAG composition though it was less polydisperse. Heparin sulphate levels were elevated. Platelet GAG characteristics were close to normal. In Willebrand disease leukocyte GAG content and composition was similar to those in hemophilia A except some differences in electrophoretic properties of small GAG components.Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.

The work presents findings of an ultrasonic examination of joints in 30 patients with hemophilia and in 15 healthy persons. Of 30 hemophilic patients 15 had hemarthroses and 15 had chronic hemorrhagic-destructive arthropathies. On the basis of the examinations carried out a conclusion was made about high informative value of sonography as a non-invasive diagnostic method and as estimation of the efficiency of treatment of hemophilic arthropathies.

Urinary system was examined in 35 hemophiliacs. 25 of them had in the past 2 to 6 episodes of hematuria. The examinees had also depressed cellular immunity. Their humoral immunity, on the contrary, was activated. In the absence of clinical and x-ray evidence of urinary abnormalities, renal affections were registered in 88.6% of the examinees. According to the data obtained all hemophiliacs suffer from unbalance of cellular and humoral immunity, abnormal clearance of immune complexes, probable renal damage. Therefore, it is desirable to make ultrasonic investigation of the kidneys in all hemophiliacs to check latent renal affection and take therapeutic measures.

Ultrasonic investigation of the urinary system and the kidneys has been performed in 40 hemophilia A patients. The size of the kidneys was reduced in the majority of patients, the area of the calices in relation to relevant parenchyma was relatively enlarged. Parenchyma to caliceal-pelvic system ratio made up 1.5:1. The caliculi and pelves were dilated. The resultant ultrasonic picture was characteristic of chronic pyelonephritis. Concrements of the right and left kidney were detected in 45% and 62.5% of the patients, respectively. The bladder looked asymmetric with thick walls and echogenic particles in the cavity. Control laboratory findings failed to reveal any renal pathology. It is clear that diagnostic ultrasound is important in hemophiliacs who may develop latent renal disorders. The risks of replacement therapy with blood preparations and sequela of renal hemorrhages are considered.

This study was aimed at investigation of immune status in patients with hemophilic arthropathies and design of optimal therapeutic programs for them. 103 hemophiliacs were examined. Of them 56 patients had chronic productive synovitis and 47 osteoarthrosis. All the patients displayed higher levels of CIC and immunoglobulins than normal subjects, the highest values being registered in synovitis. Synovial fluid in them contained CIC and IgG, IgA, IgM. The findings suggest that immune disorders are actively involved in pathogenesis of hemophilic arthropathies. Programs of immunocorrecting therapy of synovitides and osteoarthroses including plasmapheresis and intraarticular administration of hormonal and cytostatic drugs produced a clinical response in 80% of the cases. The remission lasted up to 1 year.