The impact of mutation position on thrombotic risk in protein S-deficient patients.

Background: Inherited protein S deficiency is a thrombophilic risk factor associated with venous thromboembolism. However, there is not much data on the impact of mutation position on thrombotic risk.

Objectives: The aim of this study was to evaluate the risk of thrombosis due to mutations located in the sex hormone-binding globulin (SHBG)-like region as opposed to the rest of the protein.

Increasing procoagulant activity of circulating microparticles in patients living with HIV.

Objectives: HIV-infected individuals are at higher risk of non-AIDS diseases associated with procoagulant status. Microparticles are elevated in disorders associated with thrombosis (e.g.

[Antithrombotics today].

Over the last 10 years some new drugs affecting blood clotting - antithrombotics - were invented. For more than half a century only warfarin and heparin were available. A better understanding of the pathways involved in activation of blood clotting, the links between these systems, and the impact of thrombosis on inflammation, have led to identification of new targets and development of new drugs, especially new antiplatelet drugs and anticoagulants.

[Chronic disseminated intravascular coagulation (DIC) markers in a patient with multiple angiomatosis during treatment with anti-angiogenics: interferon α, thalidomide and lenalidomide].

Background: Multiple angiomatosis is a rare disease with angiomatous formations in multiple organs and tissues and associated with a risk of fatal bleeding.

Case Description: In this patient, the bones, pleural and peritoneal cavities and digestive tract were involved. The patient had long-term been administered zoledronate that provided relief from bone pain as early as after the second dose.

[Six-year follow-up of a patient with multiple angiomatosis involving skeleton, thoracic and abdominal cavities and the gut wall].

Backgrounds: Multiple angiomatosis is a rare disease causing angiomatous lesions in multiple organs and tissues with a risk of life-threatening haemorrhage.

Observation: A young man was diagnosed with multiple angiomatosis at the age of 28 after two years of back and abdominal pain. Laparotomy revealed multiple spongy lesions mostly within the retroperitoneal space.

[Blood lipid changes at long-term antiretroviral treatment].

Unlabelled: The aim of study was to find the development trend of blood lipid concentration in a group of HIV positive patients treated by combination antiretroviral therapy (cART). We followed changes during the therapy and evaluated their aterogennic nature.

Methods: The group included 118 patients stepwise allocated to the AIDS Centre of the Faculty Hospital Brno, with the monitoring period being up to 1 month as the minimum and up to 17 years as the maximum.

[HIV lipodystrophy].

Combined antiretroviral therapy results in extraordinary decrease of morbidity and mortality of HIV-infected patients and in an essential change of the HIV/AIDS disease prognosis. However, long-term intake of antiretroviral medicaments is related to occurrence of metabolic and morphological abnormalities, of which some have been combined into a new syndrome--the so called HIV lipodystrophy. The HIV lipodystrophy syndrome covers metabolic and morphological changes.

FEIBA in treatment of acute bleeding episodes in patients with haemophilia A and factor VIII inhibitors: a retrospective survey in regional haemophilia centre.

FEIBA (factor eight inhibitor by-passing activity) is used to achieve haemostasis in haemophiliacs with inhibitor. The aim of this study was to evaluate efficacy and consumption of the product in treatment of haemorrhages in haemophiliacs with factor VIII inhibitor, and determine factors that can influence the results of treatment. We used data from our haemophilia centre from years 2000-2008.

[Hemophilia].

Hemophilia is the most spread hereditary bleeding disorder with severe bleeding symptoms. Although the number of hemophiliacs is below 0.1 per thousand in population, the care of these patients consumes a lot of financial expenditures, especially for treatment in appearance of inhibitor.

[Preparation of patients with haemostatic disorder for invasive medical interventions].

The current trend in medicine is to sustain the possibility for necessary procedures to be performed in patients who suffer from haemostatic disorders which complicate eventual surgery. Among such disorders are congenital blood coagulation disorders, haemostatic disorders concomitant with other diseases and also therapies which affect haemostasis either on purpose or as part of adverse effects. Among coagulation disorders are congenital haemorrhagic or thrombotic conditions, acquired blood coagulation disorders--combined in the vast majority of cases-- and associated with pregnancy, severe internal diseases and surgery related diseases, severe injuries, wounds, burns, malignancies, systemic connective tissue diseases, inflammatory bowel disease, and a number of other diseases.

[Treatment of haemorrhage with transfusion preparations and blood derivatives].

Appropriate use of blood components and blood products improved both patients care and use of health care resources. In bleeding and/or risk bleeding situations use of these products is often indicated. The authors summarise different guidelines, experiences, indications and problems in hemotherapy concerning bleeding complications.

[Von Willebrand disease].

Von Willebrand disease is the most spread hereditary bleeding disorder with prevalence up to the 1% in the population. Haemorrhagic symptoms are various intensity, mostly not severe. A large part of affected patients do not come to see a doctor.

Hemocoagulation disorders in extensively burned patients: pilot study for scoring of the DIC.

Goal Of The Study: To apply results of the hemocoagulation parameters to the DIC (disseminated intravascular coagulation) score system. Compare parameters of the DIC score in patients with extensive burn trauma (hospitalized at the Intensive Care Unit, ICU) and patients with lesser extent of burn injury (hospitalized at the standard unit). To use these data within the evidence based medicine for the prediction of organ damage and multi-organ failure.

[HIV/AIDS and pathology in blood count].

Background: HIV/AIDS pandemy has hit the entire world. With the use of retroviral therapy the disease became chronic. The life prolongation often leads to complications in various organs.

[Complications of heparin administration].

Heparin as an indirect anticoagulant is used in the clinical practice for more than 60 years. It is an effective drug, but it has limitations. To the side effects belong the heparin resistance, risk of bleeding (haemorrhage), hypersensitivity reactions and heparin-induced osteoporosis.

[The preparation of a patient with long-term anticoagulant cumarin treatment for invasive surgery].

Coumarins belong to drugs widely used and the spectrum of their use is going to grow. From this point of view and/or because the coumarins are adminstrated in patients who are treated for the other diseases--medical or surgical--at the same time, it is necessary to modify, interrupt or replace peroral anticoagulant treatment in the dependence on various aspects. It requires to compound different algorithms for given situations solution.

Haemopoiesis defects in HIV/AIDS-anaemia.

Under physiological conditions haemopoiesis is continuous and maintains a stable number of blood elements. A defect at any stage presents as anaemia, neutropenia, thrombocytopenia or their combinations. The cause of haematological abnormalities with an HIV infection is multifactorial.

[Molecular study of type 2 von Willebrand disease].

Background: von Willebrand disease is an inherited bleeding disorders caused by mutations in the von Willebrand factor gene. We attempted to characterise the phenotype and the genotype in the first five families in Czech Republic affected by this heterogeneous disorder.

Methods And Results: The level of FVIII was measured by the one stage assay, the vWF:Ag by the immunoelectrophoresis, vWF:RiCo by aggregometry.

[ABO/H blood groups and factor V Leiden].

Background: Jick and his co-workers published in 1969 an observation showing the prevalence of blood group 0 being lower in people with thrombosis than in the healthy population. During the next years this finding was several times confirmed. The aim of our work was to answer the question on the distribution of blood groups in individuals with thrombophilia compared to the control group and whether the non-0 blood group increases the thrombosis risk in factor V Leiden carriers.

[Molecular basis of hereditary antithrombin defects in 10 Czech families].

Background: Molecular basis of antithrombin deficiency has not yet been studied in Czech Republic. We looked for the causal mutations throughout the antithrombin gene in 26 patients from 10 unrelated families with antithrombin defect.

Methods And Results: We screened the gene by conformation sensitive gel electrophoresis and sequenced the mismatched regions using fluorescence technology to characterise mutations and polymorphisms.

[The factor V Leiden mutation in users of hormonal contraceptives].

Objective: To assess the frequency of factor V Leiden in oral contraceptive users and to define possible anamnestic data that could predict the presence of factor V Leiden.

Methods: Between 1997 and 1998, 583 users of oral estrogen-progestin contraceptives with no history of thrombotic disease were examined. Factor V Leiden was assessed by PCR after isolating DNA from a peripheral venous blood sample.

[Kinetics of hematopoiesis after high-dose chemotherapy and autologous peripheral stem cell transplantation].

Background: Autologous peripheral blood stem cell transplantation (APBSCT) is gradually replacing autologous bone marrow transplantation in many clinical settings. The key question is how to evaluate the quality of grafts. We analyzed the relationship between hematopoietic reconstitution and characteristics of patients and grafts.