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Z Boda

Publications and source records attributed to Z Boda.

At least 19 recordsLinked to original sources
PubMed2003

Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding.

Development of autoantibody against coagulation factor V (FV) is a rare clinical condition with hemorrhagic complications of varying severity. The aim of this study was to establish the pathomechanism of an acquired FV deficiency and characterize the FV inhibitor responsible for the clinical symptoms. A 78-year-old female was admitted to hospital with severe gastrointestinal bleeding. General clotting tests and determination of clotting factors were performed by standard methods. FV antigen and FV containing immune complexes were measured by ELISA. The FV molecule was investigated by Western blotting and by sequencing the f5 gene. The binding of patient's IgG to FV and activated FV (FVa) was demonstrated in an ELISA system and its effect on the procoagulant activity of FVa was tested in clotting tests and in a chromogenic prothrombinase assay. Localization of the epitope for the antibody was performed by blocking ELISA. FV activity was severely suppressed both in plasma and platelets. FV antigen levels were normal by ELISA using polyclonal anti-FV antibody or monoclonal antibody against the connecting region of FV, but depressed when HV1 monoclonal antibody against the C2 domain in the FV light-chain was used as capture antibody. The FV molecule was found intact. An IgG reacting with both FV and FVa was present in the patient's plasma and its binding to FV was inhibited by HV1 antibody. FV-containing immune complexes were detected in the patient's plasma and platelet lysate. The patient's IgG inhibited the procoagulant function of FVa. An anti-FV IgG was present in the patient's plasma and platelets. The autoantibody reacted with an epitope in the C2 domain of FV light chain and neutralized the procoagulant function of FVa.

Aged↗
PubMed2000

[High-shear force-induced platelet aggregation in the screening and diagnosis of von Willebrand disease].

Von Willebrand's disease (vWD) is the most common congenital haemorrhagic diathesis, characterized by the quantitative or qualitative disorder of von Willebrand factor (vWF). A number of methods have been used for the diagnosis of the disease, and the bleeding time determination is widely accepted as a screening test in spite of its low sensitivity. Our aim was to evaluate and compare the performance of two high shear systems (the O'Brien filter test and the PFA-100 device) in the screening and diagnosis of vWD. Thirty patients (n = 13 type 1 with mild symptoms, n = 9 type 1 with severe symptoms, n = 2 type 2A, n = 3 type 2B and n = 3 type 3 vWD) and twenty controls were investigated. In mild vWD the platelet retention in the second phase of the filter test with citrated blood showed the highest sensitivity (91.6%). The sensitivity of the PFA-100 method with collagen-epinephrine cartridges in this group was 76.9%, while the bleeding time was prolonged only in 15.4% of the cases. In severe type 1, in type 2A and type 3 all functional tests reflected the bleeding tendency of the patients. In type 2B disease the bleeding time was prolonged only when the patient was thrombocytopenic, but both high shear systems revealed the disease independently of the presence of thrombocytopenia. The overall sensitivity of the bleeding time determination was 50% compared to the 80-90% sensitivity of the O'Brien filter test and the PFA-100 system. The sensitivity values of the filter test and the PFA-100 device with collagen-epinephrine cartridges were in the same range, but the collagen-ADP cartridges showed a lower (65.5%) sensitivity, though the results were specific and had high positive predictive value. We conclude that both high shear systems are suitable for the screening of vWD, and that they are superior to the traditional bleeding time determination in case of mild disease or type 2B vWD.

Adult↗
PubMed2000

Use of a platelet filter test in patients with thrombocytosis.

In the differential diagnosis of primary and secondary thrombocytosis platelet function tests may play an important role. We examined the applicability of a platelet filter test (shear-dependent platelet aggregation) as a tool, to differentiate primary thrombocytosis (cases with myeloproliferative disorders) from secondary (reactive) thrombocytosis. The test was carried out in 53 patients suffering from myeloproliferative disorders associated with primary thrombocytosis and in 21 patients with other diseases complicated by secondary thrombocytosis. Using citrate as anticoagulant, the sensitivity of the O'Brien's test proved to be 77.1%, and its specificity 94.4%. Using heparin as anticoagulant the sensitivity and specificity of the test were found to be also reliably high. Based on these studies we suggest the use of the O'Brien's filterometer as a screening test in the differential diagnosis in patients with elevated platelet count. In the case of normal results, the causes of reactive thrombocytosis should be clarified first, while with abnormal results, haematological examination of the patients should be performed.

Adenosine Diphosphate↗
PubMed2000

Comparison of the O'Brien filter test and the PFA-100 platelet analyzer in the laboratory diagnosis of von Willebrand's disease.

Von Willebrand's disease (vWD) is the most common congenital haemorrhagic diathesis, characterized by the quantitative or qualitative disorder of von Willebrand factor (vWF). A number of methods have been used for the diagnosis of the disease, and the bleeding time determination is widely accepted as a screening test in spite of its low sensitivity. Our aim was to evaluate and compare the performance of two high shear systems (the O'Brien filter test and the PFA-100 device) in the screening and diagnosis of vWD. Thirty patients (n=13 type 1 with mild symptoms, n = 9 type 1 with severe symptoms, n = 2 type 2A, n = 3 type 2B and n = 3 type 3 vWD) and twenty controls were investigated. In mild vWD the platelet retention in the second phase of the filter test with citrated blood showed the highest sensitivity (91.6%). The sensitivity of the PFA-100 method with collagen-epinephrine cartridges in this group was 76.9%, while the bleeding time was prolonged only in 15.4% of the cases. In severe type 1, in type 2A and type 3 all functional tests reflected the bleeding tendency of the patients. In type 2B disease the bleeding time was prolonged only when the patient was thrombocytopenic, but both high shear systems revealed the disease independently of the presence of thrombocytopenia. The overall sensitivity of the bleeding time determination was 50% compared to the 80-90% sensitivity of the O'Brien filter test and the PFA-100 system. The sensitivity values of the filter test and the PFA-100 device with collagen-epinephrine cartridges were in the same range, but the collagen-ADP cartridges showed a lower (65.5%) sensitivity, though the results were specific and had high positive predictive value. We conclude that both high shear systems are suitable for the screening of vWD, and that they are superior to the traditional bleeding time determination in case of mild disease or type 2B vWD.

Adenosine Diphosphate↗
PubMed1999

[Clinical aspects of heparin induced thrombocytopenia].

Heparin induced thrombocytopenia has remained the major complication of therapy or prophylaxis with heparin. Although low molecular weight heparins seem to confer much lesser chance to induce thrombocytopenia, the danger is still considerable, and the fatal outcome is not rare. A lot of new data have been published about the origin, binding, physicochemical properties of the antibodies, the responsive platelet membrane receptors, and laboratory diagnosis in particular, however, many issues are still unresolved. The anticoagulant treatment of cases, in which heparin induced thrombocytopenia in associated with progressive, frequently arterial thrombosis still needs great skills, experience and the use of new generation antithrombotic agents. This review summarizes briefly the internationally accepted standard diagnostic and therapeutic protocols with heparin induced thrombocytopenia.

Heparin↗
PubMed1999

Acquired Bernard-Soulier syndrome: a case with necrotizing vasculitis and thrombosis.

We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von Willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired Bernard-Soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.

Antibodies, Antinuclear↗
PubMed1999

Safety of low-molecular-weight heparin in pregnancy: a systematic review.

Unfractionated heparin (UFH) remains the anticoagulant of choice during pregnancy. Low-molecular-weight heparins (LMWH) are an attractive alternative to UFH due to their logistic advantages and their association with a lower incidence of osteoporosis and HIT. We reviewed all published clinical reports concerning the use of LMWH during pregnancy. In addition, participants of an international interest group contributed a cohort of pregnant women treated with LMWH. Pregnancies were divided into two groups; those with and those without maternal comorbid conditions. The number of adverse fetal outcomes and the occurrence of maternal complications were evaluated in the two groups. In the group of women with comorbid conditions (n = 290), 13.4% of the pregnancies were associated with an adverse fetal outcome. In contrast, in the group of women without comorbid conditions (n = 196), 3.1% were associated with an adverse outcome, which is comparable to that seen in the normal population. We conclude that LMWH appear to be a safe alternative to unfractionated heparin as an anticoagulant during pregnancy.

Anticoagulants↗
PubMed1998

[Thrombophilia, anticoagulant therapy and pregnancy].

Thromboembolic complications during pregnancy are the most common causes of maternal death. Here we report on thromboembolic prophylaxis of 60 pregnancies of 32 pregnant women with familial thrombophilia. Long-term Fraxiparine (Sanofi-Chinoin) as thromboprophylaxis was applied in 26 cases throughout pregnancy. UFH (Heparin-Ca inj.) was used in 11 cases, and there were 23 pregnancies without thromboembolic prophylaxis in our patient's case histories. Artificial abortions were not included in this paper. The ratio of successful pregnancies were: with Fraxiparine: 24/26 (92.3%), with UFH (Heparin-Ca): 8/11 (72.7%), without prophylaxis: 4/23 (17.4%). In the patient group treated with Fraxiparine there were no foetopathy, thrombocytopenia or bleeding complication. LMWH is recommended for pregnant women with familial thrombophilia. According to literature data and our own experiences the doses of LMWH in patients with familial thrombophilia, and -antiphospholipid syndrome, and -artificial heart value are suggested.

Adult↗
PubMed1998

[The O'Brien filter test in the differential diagnosis of disorders with high-grade thrombocytosis].

In the differential diagnosis of primary and secondary thrombocytosis, platelet function test can be used. We have examined the possible role of O'Brien's filter test in the differentiation of primary and secondary thrombocytosis in 53 patients with myeloproliferative diseases with primary thrombocytosis and in 21 patients with other disorders complicated by secondary thrombocytosis. By using heparin as an anticoagulant, the sensitivity of O'Brien's filter test proved to be 75%, and it's specificity was 85.7%. In blood samples anticoagulated with citrate, the sensitivity was 100% and specificity 83.3%. Based on these studies we suggest the use of O'Brien's filterometer as a screening test in the differential diagnosis in patients with elevated (> 400 x 10(9)/L) platelet count. In case of normal results, the causes of reactive thrombocytosis should be cleared first, while with pathologic results, haematological examination of the patients should be performed.

Chronic Disease↗
PubMed1997

[Fibrinogen as an independent cardiovascular risk factor and the question of treatment with fibrinogen-lowering drugs].

The atherosclerotic vascular disease is a progressive condition of multifactorial origin. Among cardiovascular risk factors alterations of lipid metabolism are of central role. But the haemostatic system, first of all the plasmatic fibrinogen is also highly implicated. Increased fibrinogen condition can be regarded as a strong and independent predictor of cardiovascular risk. Total cholesterol, LDL-cholesterol and plasma fibrinogen concentration as risk factors are compared. Fibrinogen lowering treatments are also summarized.

Anticoagulants↗
PubMed1997

[Coumarin combined with low-dose acetylsalicylic acid in the prevention of thromboembolic complications in patients with mitral and aortic valve prostheses].

Authors studied the effect of coumarin, and its combination with low-dose (125 mg/day) acetylsalicylic acid in the prevention of thromboembolic complication during a 10-year period (average 4.7 years) in a randomized trial of 296 patients aged 18-60 year with tilting disc type prosthetic heart valve (159 mitral and 137 aortic) in sinus rhythm. In the group treated with coumarin (152 patients, 743.4 patient-years) 4 cases (2 of them fatal) of valve thrombosis, 12 cases of peripheral embolism and 9 cases (3 intracranial, 3 among them fatal) of major bleeding were observed; in the group treated with coumarin plus acetylsalicylic acid (144 patients, 638.7 patient-years) 2 cases (1 of them fatal) of valve thrombosis, 4 cases of peripheral embolism and 14 cases (3 of them fatal) of major bleeding were observed. In the case of valve thromboses the difference between the two groups was non-significant but still clinically remarkable; peripheral embolism occurred in significantly higher number (p < 0.05). There was no statistically significant difference of bleeding complications between the two groups. The results suggest that the combination of coumarin plus low-dose acetylsalicylic acid is more effective in the prevention of thromboembolic complications in patients with mitral and aortic prosthetic heart valve than coumarin alone; the danger of bleeding complications seems to be acceptable with adequate control.

Adolescent↗
PubMed1996

[Fibrinolysis and vascular diseases].

A wide array of in vitro fibrinolysis tests had been performed for a long period, suggesting defective fibrinolysis, mainly impaired tPA response and reserve as major, frequent abnormality, predecessor or causative factor of venous thromboembolism. However, these abnormalities were troublesome to reproduce, and more recently fibrinolytic activators and inhibitors received growing attention as rather atherogenic and less thrombogenic risk factors. Even if it is still not settled, lipoprotein(a) may interfere with fibrinolysis, and seems to carry atherogenic risk, too. The genetic polymorphism of fibrinogen, plasminogen, PAI-1 and some other compounds modifying circulating fibrinogen levels are also discussed in this review.

Arteriosclerosis↗
PubMed1996

[Low-molecular-weight heparin in the prevention of thromboembolism in pregnant thrombophilic patients].

Prophylaxis of thromboembolism with low molecular weight heparin (LMWH) may offer some advantages over unfractionated heparin during pregnancy. Controlled studies with LMWH for thromboprophylaxis in pregnancy are failing, although according to some recent studies LMWH did not cross the placental barrier. LMWH as thromboprophylactic agent was used in three young pregnant women with familial thrombophilia (two with PC one with AT-III deficiency). According to the bodyweight of patients the applied doses of LMWH were 5,000-10,000 ICU once daily. Laboratory control (determination of anti-FXa activity in plasma samples) was made monthly. The three pregnancies were uneventful, thromboembolic or haemorrhagic complications did not develop. Newborns were healthy, with no coincide of disturbances of haemostasis. The LMWH-demand is certainly increased at the late period of pregnancy. LMWH as thromboprophylaxis is recommended for pregnants with familial thrombophilia. The necessary dose of Fraxiparine may be 70 ICU/kg/day in the first and 100 ICU/kg/day in the second half of pregnancy.

Adult↗
PubMed1996

Studies of the platelet filter test (shear dependent platelet aggregation) in patients with uncommon haemorrhagic disorders.

Platelets of anticoagulated whole blood forced at 40 mmHg through a fine filter are activated, aggregated and retained, so block the filter (platelet filter test, O'Brien JR, Salmon GP. Blood 1987; 1354-1361). Our clinical experiences with this simple and quick haemostasis test are summarized. Patients were investigated with different types of vWD (type-1 = 35, type-2A = 7, type-2B = 7, type-3 = 1), Glanzmann's thrombasthenia, congenital deficiency of cyclo-oxygenase, acquired Bernard-Soulier syndrome, FXII-, FXIII-deficiency and a control group. The cumulative drop count and the platelet retention were carefully measured during two phases of the filter test. Platelet count, bleeding time, vWF:Ag and vWF:Rcof activity were measured along with the platelet filter test. The filter was not blocked and the platelet retention was abnormally low in all patients with thrombasthenia, type-2a, type-2B, type-3 vWD. Treatment with 1-desamino-8-D-arginine-vasopressin (DDAVP) caused enhanced platelet retention in 16 patients with type-1 vWD. The test is simple, quick and cheap, has good reproducibility, and may be useful in clinical haemostasis laboratories for examination of high shear induced platelet functions.

Adolescent↗