[Results of treatment of hemophilic synovitis with the application of rifampicin synoviortesis].

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.

[Genetic determinants of hereditary thrombophilia in pathogenesis of venous thrombosis]. / Geneticheskie determinanty nasledstvennoi trombofilii v patogeneze venoznogo tromboza.

AIM: 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. MATERIAL AND METHODS: 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). RESULTS: 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. CONCLUSION: Determination of prothrombotic genotypes is a key factor of treatment efficacy and prevention of life-threatening thromboembolic complications.

[Changes in hemostasis system in patients with hereditary thrombophilia caused by mutation of blood coagulation factor V ( factor V Leiden)]. / Izmeneniia v sisteme gemostaza u bol'nykh s nasledstvennoi trombofiliei, obuslovlennoi mutatsiei faktora V svertyvaniia krovi (faktor V Leiden).

AIM: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Leukocyte and thrombocyte glycosaminoglycans in hemophilia A and von Willebrand's disease]. / Glikozaminoglikany leikotsitov i trombotsitov pri gemofilii A i bolezni Villebranda.

AIM: The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy. MATERIALS AND METHODS: GAG concentration, composition and fraction identification were made in 25 patients with hemophilia A and 10 patients with Willebrand disease. RESULTS: 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. CONCLUSION: Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.

[A method for determining thrombocyte intravascular activation and its significance for clinical practice]. / Metod opredeleniia vnutrisosudistoi aktivatsii trombotsitov i ego znachenie v klinicheskoi praktike.

The authors offer a visual method for assessing the intravascular platelet activation, based on examination under phase-contrast microscope of changes in the shape and relative number of blood platelets in aggregations. Results of examinations of patients with different thrombotic diseases are presented.

[The sonographic characteristics of hemophilic arthropathies]. / Sonograficheskaia kharakteristika gemofilicheskikh atropatii.

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.

[The clinico-instrumental characteristics of the kidneys in hemophilia patients]. / Kliniko-instrumental'naia kharakteristika pochek u bol'nykh gemofiliei.

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 study of the urinary system in hemophiliacs]. / Ul'trazvukovoe issledovanie mochevydelitel'noi sistemy u bol'nykh gemofiliei.

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.

[Pathogenesis and therapy of joint inflammation in patients with hemophilia]. / Osobennosti patogeneza i terapii porazhenii sustavov u bol'nykh gemofiliei.

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.

[Blood rheological disorders in patients with postthrombotic disease and the means for their correction]. / Gemoreologicheskie narusheniia u bol'nykh s posttromboticheskoi bolezn'iu i sposoby ikh korrektsii.

An analysis of results of rheological properties of blood in 45 patients with different forms of postthrombotic disease of lower extremities has shown that 78% of the patients have erythrocytosis and all the patients have hyperviscosity shifts. Correction of the hemorheological changes by the methods of transfusion therapy (plasmapheresis, exfusion of blood, hemodilution) allowed to reduce the microcirculatory disorders in the diseased extremity in this group of patients.

[The treatment of acute venous thromboses with renokinase]. / Lechenie ostrykh venoznykh trombozov renokinazoi.

The article presents results of treatment of 53 patients with acute thrombosis of profound veins of various localizations by means of a new home thrombolytic drug, physiologic activator plasminogen--renokinase. High effectiveness of thrombolytic therapy with good rehabilitation or marked clinical improvement was obtained in 79% of the patients. Renokinase is areactogenic and may be used many times.

[Results of therapeutic use of plasma- and erythrocytapheresis in patients with arterial thromboobliterating diseases of the extremities]. / Nekotorye itogi primeneniia lechebnogo plazma- i éritrotsita- fereza u bo'lnykh s tromboobliteriruiushchimi zabolevaniiami arterii konechnostei.

The article analyzes results of using methods of plasma- and plasmoerythrocytapheresis in 120 patients with occlusive injuries of main arteries of extremities. The efficiency of the methods used both as isolated ones and in combination with surgical procedures in patients with obliterating atherosclerosis, thromboangiitis, and aorto-arteritis of the extremity vessels and the Raynaud disease is estimated.

[Surgical tactics in acute superficial thrombophlebitis]. / Khirurgicheskaia taktika pri ostrom poverkhnostnom tromboflebite.

Results of the surgical treatment of 147 patients for acute thrombophlebitis of the dilated subcutaneous veins are described. Radical vagotomy was performed in most of the patients. The removal of thrombi from the umbilical veins with the help of the Fogarty probe and aspiration with a vacuum suction device was performed in 13 cases. A conclusion is made that the surgical treatment of acute thrombophlebitis of subcutaneous veins of lower extremities is an effective method of prophylactics of thromboses and pulmonary embolism.

[Postoperative recurrence of dilatation of the superficial veins of the legs]. / Posleoperatsionnye retsidivy rasshireniia poverkhnostnykh ven nizhnikh konechnostei.

The article presents an experience with the operative treatment of 476 patients with recurrent dilatation of superficial veins of lower extremities. True and false variants of the recurrences are discussed as well as problems of their diagnosis and treatment. Stress is made on high specific weight (87%) of patients with the development of recurrences due to incomplete examinations and technical defects during the first operation. In 13% of the patients recurrences were caused by progress of the varicose disease and non-eliminated hypertension in profound veins due to their ectasia and incompetence of the valves.

[A method of correction of hemorheological disorders in patients with thrombobliterating diseases of the peripheral arteries of the limbs]. / Metody korrektsii gemoreologicheskikh narushenii u bol'nykh s tromboobliteriruiushchimi zabolevaniiami perifericheskikh arterii konechnostei.

Methods of plasm- and plasmoerythrocytapheresis in combination with hemodilution were used in 28 patients with thrombobliterating diseases of the extremity arteries. A conclusion is made of the necessary detection of hemorheological disturbances in this group of patients. The methods used were shown to be promising. The correlation was established between changes of the rheological properties of blood and clinical course of the disease.