Hamostaseologie
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One of many challenges in the treatment of persons with haemophilia is the selection and application of appropriate pain-relieving therapies. The current situation of pain management for persons with haemophilia in Germany was evaluated using a survey with the intention of identifying potential areas for improvement. Results of 685 respondents showed that 86% experienced episodes of pain and that pain was already present in 66% of children and adolescents. Joint pain was the most common type of pain (92%), remarkably so even in 80% of young patients. Half of the patients received pharmacological therapy for the pain and 46% of the patients received physiotherapy. Priority and sequence of the contacted physicians and therapists for diagnosis and therapy is described. Satisfaction with pain therapy was expressed by 56% of participants and 18% felt their pain not treated sufficiently. ⋯ The results of the survey will be used to develop measures for improvement of long-term care of haemophilia patients regarding pain therapy.
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The doses of these drugs are barely tested and the potential clinical thromboembolic risk must be taken into account. Despite the widespread use of NOAC (non vitamin-K dependent oral anticoagulants) and recommendations of regulatory agencies and first consensus meeting on handling the bleeding situation under NOAC, especially in hospitals without a large hemostatic focus, uncertainty still exists. In case of mild bleeding from a clinical perspective, the medical care of these patients and the delay of the next dose or discontinuation is advised. A special laboratory analysis is indicated i.e. in case of known higher grade liver and kidney failure, which can cause a prolonged elimination of NOAC. The administration of factor concentrates is not indicated in this situation. In case of moderate to severe bleeding, the primary measures focus on the stabilization of the heart and circulatory function and parallel on the treatment depending on the localization of the bleeding source. According to experience, mostly gastrointestinal bleeding occurs under the NOAC, which should be supplied endoscopically. In life-threatening bleeding in addition to the measures of hemodynamic stabilization usually a special haemostasis management is required, which should be mainly clinically oriented. After the assessment of bleeding predictor, the time of the last dose and the dose of NOAC should be learned, but other causes of bleeding, including Fibrinolysis, should be excluded or treated. Subsequently, routinely promptly rivaroxaban and/or apixaban sensitive thromboplastin time (Quick's value) and a thrombin time (thrombin-poor calibrator) for qualitative assessment can be carried out because only very few hospitals have specific tests (anti-Xa measurements, bovine thrombin), which could be promptly done. If there is a significant deviation from the normal range or to present preliminary value of particular patient, an effect of NOAC most likely exists. In life-threatening bleeding the use of factor concentrates (procoagulants) is indicated. The first-line therapy should be PPSB. Only in exceptional cases, especially when dabigatran is taken, the use of aPPSB (FEIBA®) for prompt haemostasis can be considered. The haemostasis should be always clinically estimated and not according to coagulation tests. The use of rFVIIa (Novo Seven®) shows different results in the bleeding therapy (reversal) under Dabigatran. The doses of these drugs are barely tested and the potential clinical thromboembolic risk must be taken into account. ⋯ The current concepts of the newly developed antidotes are not clinically validated. First prospective, clinical registries have been started.
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Fibrinogen plays an essential role in clot formation and stability. Importantly it seems to be the most vulnerable coagulation factor, reaching critical levels earlier than the others during the course of severe injury. A variety of causes of fibrinogen depletion in major trauma have been identified, such as blood loss, dilution, consumption, hyperfibrinolysis, hypothermia and acidosis. ⋯ Therefore, repeated measurements of plasma fibrinogen concentration are strongly recommended in trauma patients with major bleeding. Recent guidelines recommend maintaining plasma fibrinogen concentration at 1.5-2 g/l in coagulopathic patients. It has been shown that early fibrinogen substitution is associated with improved outcome.
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The plasma circulating zymogenic coagulation factor XIII (FXIII) is a protransglutaminase, which upon activation by thrombin and calcium cross-links preformed fibrin clots/fibrinolytic inhibitors making them mechanically stable and less susceptible to fibrinolysis. The zymogenic plasma FXIII molecule is a heterotetramer composed of two catalytic FXIII-A and two protective FXIII-B subunits. Factor XIII deficiency resulting from inherited or acquired causes can result in pathological bleeding episodes. ⋯ Recently however, with a growing understanding into the pleiotropic roles of FXIII, the fairly frequent milder form of FXIII deficiency caused by heterozygous mutations has become one of the subjects of investigative research. The acquired form of FXIII deficiency is usually caused by generation of autoantibodies or hyperconsumption in other disease states such as disseminated intravascular coagulation. Here, we update the knowledge about the pathophysiology of factor XIII deficiency and its therapeutic options.
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Oral anticoagulants and platelet receptor blockers are widely used in clinical practice with the aim of reducing the risk of thrombotic complications in patients with cardiovascular diseases. Their regular intake and adequate antithrombotic action is vital and this is way numerous assays have been developed for laboratory testing and monitoring of these agents. ⋯ Such assays are increasingly used in clinical routine and their daily use is triggered by the advent of the novel direct oral anticoagulants (DOACs) as an alternative for vitamin K antagonist (VKA) treatment, which are dabigatran, rivaroxaban and apixaban, and by the advent of prasugrel or ticagrelor as an alternative for clopidogrel with regard to platelet P2Y12 receptor inhibition. In this review the most important and most commonly used laboratory assays are summarized as well as their clinical implications with the focus on DOACs as an alternative for VKAs and the different P2Y12 receptor blockers for antiplatelet treatment.