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Biomedical subjects

J Hársfalvi

Publications and source records attributed to J Hársfalvi.

At least 19 recordsLinked to original sources
PubMed2004

The most severe forms of Perthes' disease associated with the homozygous Factor V Leiden mutation.

It has recently been postulated that thrombophilia may have a role in the aetiology of Perthes' disease. The published reports, however, remain conflicting. In this study a retrospective analysis of the coagulation parameters was made in 47 patients with Perthes' disease and the results compared with the clinical data. Five patients with Factor V Leiden mutation were found (10.6%) and surprisingly four of them had a homozygous pattern. These four patients showed the most severe form of the disease, Catterall group IV, with flattening of the entire epiphysis, involvement of the metaphysis, shortening and broadening of the femoral neck, trochanteric overgrowth and developed mushroom-shaped aspherical laterally displaced femoral heads in dysplastic acetabula. We would like to suggest that the homozygous form of Factor V Leiden mutation has some role in the clinical course of Perthes' disease and particularly its most severe form.

Activated Protein C Resistance↗
PubMed2001

Organization of the glycoprotein (GP) IIb/IIIa heterodimer on resting human platelets studied by flow cytometric energy transfer.

Glycoprotein IIb/IIIa is a heterodimer of glycoproteins IIb and IIIa which serves as the inducible receptor for fibrinogen and other adhesive proteins at the surface of platelets. Although a model of the quaternary structure of the GPIIb/IIIa molecule has been constructed in solution by Calvete et al. [Biochem. J. 282 (1992) 523], a corresponding model at the surface of intact platelets is still missing. In the present work conformation and lateral distribution of the GPIIb/IIIa heterodimer were studied at a nanometer resolution on the surface of resting human platelets under physiological conditions. The experiments were based on dual wavelength flow cytometric detection of fluorescence resonance energy transfer and application of a panel of monoclonal antibodies raised against well described binding sites. Monodisperse distribution of the GPIIb/IIIa heterodimer has been observed and a detailed three-dimensional proximity map of antibody binding sites was constructed on the platelet membrane, under physiological conditions, for the first time. Our data support the view that the GPIIb subunit is in a bent conformation. A detailed analysis of the K(d)-values and the number of binding sites for a set of monoclonal antibodies was also carried out giving supplementary data for the topology of the binding sites. Our results provide a refinement of the membrane-topology of the GPIIb/IIIa heterodimer.

Animals↗
PubMed1998

Increased platelet activation and decreased fibrinolysis in the pathogenesis of aseptic necrosis of the femoral head.

Thrombotic events, increased tendency toward intravascular thrombosis and decreased fibrinolysis seem to be possible pathologic causes for aseptic necrosis of the femoral head (ANFH) in adults. This project was to study whether either increased platelet activation or decreased fibrinolytic activity and/or any other thrombogenic factor may be implicated in the evolvement of ANFH. The speed of the in vitro lysis was significantly lower in patients (both in primary and in secondary cases) compared with healthy controls. The platelet activation (measuring with beta TG) proved to be significantly higher in the primary group as well as in the secondary group compared with healthy controls. Lp(a) levels were elevated in primary and secondary cases. This alteration was more characteristic in the primary cases. Fibrinogen levels were also elevated in the primary group, but the difference was not significant. The data shown here may further support that hypofibrinolysis and increased thrombogenesis are major causes of ANFH. Early diagnosis of ANFH increases the chances of modifying the course of this disabling disease.

Journal Article↗
PubMed1995

[Changes in fat metabolism in acute carbon tetrachloride intoxication of rats].

CCl4 is an organic solvent and a well known hepatotoxic agent. Injury is mediated by reactive free radicals, mainly-CCl3 (trichloromethyl). Liver lesion develops within one-two hours, however, late toxic effects may appear after a delay of several hours or two to three days. Some drugs and liver toxicants cause disturbances in synthesis and metabolism of triglycerides, cholesterol and lipoproteins, thus damaging the basic resource for living cells. In our experiments a single 1.25 ml/kg CCl4 dose was administered s.c. to male Wistar rats. 24 hours later triglyceride level increased in the plasma by 223%. Cholesterol content suffered no changes. HDL-C level decreased by 40%. LDL-C concentration was higher by 235%. Cholesterol/HDL-C ratio increased by 0.75% on account of the practically unchanged cholesterol amount in the blood. The most often used calculation (Friedewald equation) LDL-C/HDL-C ratio was higher by 305%. The increased triglyceride content in the blood is in correlation with the fatty degeneration of the liver. The high LDL-C/HDL-C ratio may point to incipient atherosclerotic complications. The pathological lipid levels measured 24 hours after intoxication claim for delayed toxic effects to be taken into consideration. It may be suggested to determine the main lipid parameters after carbon tetrachloride exposition.

Animals↗
PubMed1994

[Correlation between adhesive factors of primary hemostasis and liver fibrogenesis in alcoholic live cirrhosis].

Colchicine administration is able to reduce collagen production and the fibrogenetic process in alcoholic liver diseases in the same time. The decrease of the serum lipid peroxidation capacity along with type III procollagen propeptide level was associated with the simultaneous reduction of plasmatic von Willebrand factor, fibronectin and beta-thromboglobulin levels. This observation suggest a connection between the regulation of primary haemostasis and liver fibrogenesis.

Colchicine↗
PubMed1993

Endothelium releases more von Willebrand factor and tissue-type plasminogen activator upon venous occlusion in patients with liver cirrhosis than in normals.

Venous occlusion was used in 8 patients with liver cirrhosis and in 10 normals to investigate the pathomechanism of long-term elevation of plasma von Willebrand factor antigen (vWFAg) in liver cirrhosis. The following parameters were determined at baseline, and immediately, 60 min and 24 h after 10 min venous occlusion: vWFAg, ristocetin cofactor (RiCoF), in vitro platelet retention (Adeplat T), and tissue-type plasminogen activator (t-PA). Every baseline value in the liver cirrhosis group was significantly higher than in the controls. In both groups the 10-min values were significantly higher than their corresponding baseline results. Hence, comparing the two groups, in liver cirrhosis a significantly higher release of vWFAg and t-PA could be observed. These findings suggest on the one hand that the increased release contributes substantially to the sustained elevation of plasma vWF level in liver cirrhosis. On the other hand, the results indicate that not only the vascular surface of the diseased liver but most probably the total endothelium plays an important role in this phenomenon.

Adult↗
PubMed1992

[Primary hemostasis studies in Conn syndrome].

The various components of haemostasis seems to be affected by the high cortisol level in Cushing's disease. Increase in coagulation factor levels, decreased fibrinolysis, high von Willebrand factor plasmatic levels along with easy bruising due to vascular damage have been described. In this report a similar but less accentuated elevation of von Willebrand factor levels are documented in aldosterone producing adrenocortical adenoma, and in the same time enhanced platelet activation was also found. The primary haemostatic alterations correlated well with aldosterone levels and subsided significantly after the removal of the ademonas. Further studies seem to be desirable to analyse and elucidate haemostatic functions in Conn's syndrome.

Adult↗
PubMed1991

[Primary hemostasis in mixed connective tissue disease].

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease. High levels of Willebrand factor antigen and activity were detected in the presence of thrombocytopenia, previous thrombotic events, pulmonary vascular lesions and in the presence of circulating antiendothelial antibodies. Increased platelet activation was documented also in antibody positive cases and in thrombocytopenia. The alterations of endothelial and platelet functions may play important role in the development of vascular complications of mixed connective tissue disease.

Adult↗
PubMed1991

[Successful treatment of severe hemorrhage by using thrombocyte suspension with cryoprecipitate in type III Willebrand disease].

A 24-year-old woman with severe haemorrhagic complication due to type III von Willebrand's disease is reported, as the first such case in Hungary. Analysis of seven family members affected by the type III mutant gene was compatible with an autosomal recessive mode of inheritance. Severe, iatrogenic epistaxis of the patient was not improved by cryoprecipitate and DDAVP. Platelet suspension together with cryoprecipitate immediately stopped bleeding. Simultaneous application of cellulare (platelet suspension) and plasmatic (cryoprecipitate) Willebrand protein is suggested for patients with severe (type III) von Willebrand's disease.

Adult↗
PubMed1991

Alterations of primary haemostasis in mixed connective tissue disease (MCTD).

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease to evaluate the incidence and the possible role of haemostatic alterations in the complications occurring during the course of the disease. High levels of Willebrand-factor antigen and ristomycin-cofactor activity were detected in patients with thrombocytopenia, previous thrombotic event, pulmonary vascular lesions and usually in the presence of circulating anti-endothelial antibodies. Increased platelet activation could have been found in antibody positive cases and in patients with thrombocytopenia as well. The documented alterations of endothelial and platelet functions may play important role in the vascular complications of mixed connective tissue disease.

Adult↗
PubMed1991

[Successful cyclosporin therapy of acquired hemophilia caused by factor VIII (VIII:C) inhibiting antibody].

A 47-year-old woman is reported who had a life-threatening, subarachnoideal bleeding due to the spontaneous development of factor VIII:C inhibitor. Cyclosporin combined with prednisone allowed a full recovery and the elimination of antibody even when other therapeutic facilities failed to be effective. Actual problems of treatment of factor VIII inhibitors are briefly surveyed.

Antibodies↗
PubMed1991

Treatment of the severe bleeding episode in type III von Willebrand's disease by simultaneous administration of cryoprecipitate and platelet concentrate.

A severe, life-threatening bleeding episode in a 24-year-old woman suffering from type III von Willebrand's disease was treated by large doses of cryoprecipitate with unsatisfactory results. Bleeding ceased and the bleeding time normalized only after concomitant administration of platelet concentrates. In the treatment of von Willebrand's disease patients possessing platelets with absent or insufficient von Willebrand factor activity the administration of plasma concentrates together with platelets appears to be justified.

Adult↗
PubMed1990

[Thrombotic changes in hemostasis following streptokinase therapy in myocardial infarct].

Thrombolytic treatment of acute myocardial infarction proved to be able to restore infarct artery patency and to decrease hospital mortality. The number of bleeding complications have remained at an acceptably low level, however some thromboembolic complications occurring during the first week following thrombolytic therapy have been recently observed. Signs of increased in vivo platelet activation (by measuring beta-thromboglobulin and thromboxane metabolite levels) and endothelial damage (Willebrand-factor estimations) could have been detected in our patients treated with brief high dose intravenous streptokinase, altogether with diminished antithrombin III and protein C antigen levels and activity. Intravenous streptokinase treatment of acute myocardial infarction might be able to cause thrombotic haemostatic alterations, which require meticulous haemostasis monitoring and early, correct antithrombiotic therapy.

Blood Coagulation↗
PubMed1989

[Thrombotic microangiography (thrombotic thrombocytopenic purpura and hemolytic uremia syndrome)].

Thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome have quite uniform or identical histopathological features and share very similar clinical characteristics, so thrombotic microangiopathy may be used as a common descriptive term of the two syndromes. During the past one and a half year five patients with thrombotic microangiopathy, receiving plasma therapy were observed by the authors. Owing to the early, correct diagnosis and plasma administration four patients had been recovered from the disease. The evaluation of clinical signs and symptoms, altogether with some simple laboratory data (megakaryocytic thrombocytopenia, nonimmune acquired haemolytic anaemia, renal insufficiency, neurologic alterations, etc.) remained essential in the recognition and treatment of thrombotic microangiopathy. After giving a short review of the literature the authors deal with the results of some more specific examinations (prostanoids, beta-thromboglobulin, the quantitative and qualitative analysis of Willebrand factor, etc.) which may give further data to the complex aetiopathogenesis of thrombotic microangiopathy.

Adolescent↗