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Hämostaseologie
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  This is an Open Access Journal Open Access journal
ISSN (Print) 0720-9355 - ISSN (Online) 2567-5761
Published by Thieme Publishing Group Homepage  [222 journals]
  • Hamostaseologie 2019; 39
    • Hämostaseologie 2019; S 01

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Kongress

      2019-02-13T00:00:00+0100
      Issue No: Vol. 39, No. S 01 (2019)
       
  • Changes and Challenges
    • Hamostaseologie 2019; 39: 001-003
      DOI: 10.1055/s-0039-1678526



      Georg Thieme Verlag KG Stuttgart · New York

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Volltext

      Hamostaseologie 2019; 39: 001-0032019-02-18T00:00:00+0100
      Issue No: Vol. 39, No. 01 (2019)
       
  • Science Meets Clinical Practice
    • Hamostaseologie 2019; 39: 004-005
      DOI: 10.1055/s-0039-1677839



      Georg Thieme Verlag KG Stuttgart · New York

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Volltext

      Hamostaseologie 2019; 39: 004-0052019-02-18T00:00:00+0100
      Issue No: Vol. 39, No. 01 (2019)
       
  • GTH News
    • Hamostaseologie 2019; 39: 101-103
      DOI: 10.1055/s-0039-1683456



      Georg Thieme Verlag KG Stuttgart · New York

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Volltext

      Hamostaseologie 2019; 39: 101-1032019-02-18T00:00:00+0100
      Issue No: Vol. 39, No. 01 (2019)
       
  • Einladung des BDDH e.V.
    • Hamostaseologie 2019; 39: 104-104
      DOI: 10.1055/s-0039-1678609



      Georg Thieme Verlag KG Stuttgart · New York

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Volltext

      Hamostaseologie 2019; 39: 104-1042019-02-18T00:00:00+0100
      Issue No: Vol. 39, No. 01 (2019)
       
  • IWR-1 Inhibits Collagen-Induced Platelet Activation and Protects against
           Thrombogenesis

    • Authors: Wang; Wei, Lai, Songqing, Xiao, ZiJin, Yan, Haiyue, Li, Yongxi, Zhang, Ye, Wu, Yanqing, Wang, Ling
      Abstract: Platelets play a crucial role in haemostasis and several pathophysiological processes. Collagen is a main initiator for platelet activation and aggregation. Given that Wnt signalling negatively regulates platelet function, and IWR-1 (a small molecule inhibitor for Wnt signalling) has the potential of inhibiting collagen synthesis, it is essential to investigate whether IWR-1 regulates collagen-induced platelet activation and protects against thrombogenesis. In the present study we found that IWR-1 pretreatment effectively suppressed collagen-induced platelet aggregation in a dose-dependent manner. In addition, IWR-1 also resulted in a decrease of P-selectin and phosphatidylserine surface exposure using fluorescence-activated cell sorting analysis. In vitro studies further revealed that IWR-1 had a negative effect on integrin a2β1 activation and platelet spreading. More importantly, the results from in vivo studies showed that IWR-1 exhibited a robust bleeding diathesis in the tail-bleeding assay and a prolonged occlusion time in the FeCl3-induced carotid injury model. Taken together, current results demonstrate that IWR-1 could effectively block collagen-induced platelet activity in vitro and in vivo, and suggest its candidacy as a new antiplatelet agent.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-03-08T00:00:00+0100
      DOI: 10.1055/s-0038-1676822
       
  • Pathophysiology of Autoimmune Thrombocytopenia: Current Insight with a
           Focus on Thrombopoiesis

    • Authors: Marini; Irene, Bakchoul, Tamam
      Abstract: Immune thrombocytopenia (ITP) is an autoimmune disease characterized by a significant reduction in the number of circulating platelets which is frequently associated with bleeding. The total count of platelets in the body is finely regulated by the balance between platelet production and destruction. Although the pathogenesis of ITP is still not completely elucidated, it is largely recognized that the low platelet count observed in ITP patients is due to alterations of both mechanisms. An abnormal proliferation of autoreactive T cells leading to the breakdown of immune tolerance to platelet antigens is suggested to be responsible for the up-regulated proliferation of autoantibody producing B cells. Consequently, the immune response induces enhanced T cell-mediated cytotoxicity and antibody-mediated platelet destruction through phagocytosis, complement activation and apoptosis. An additional contribution to the pathophysiology of ITP is given by alterations of thrombopoiesis caused by platelet-reactive autoantibodies or cytotoxic T cells leading to impaired megakaryocyte differentiation and platelet production. All these processes involved in ITP pathophysiology account for the complexity and heterogeneity in the clinical manifestation and therapy responsiveness of this disorder. For this reason, a better understanding of the different underlying mechanisms in ITP is necessary to develop more efficient therapeutic treatments in the future. In this review, we will provide an update on the pathophysiology of ITP with a particular focus on the impact of impaired thrombopoiesis.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-02-25T00:00:00+0100
      DOI: 10.1055/s-0039-1678732
       
  • Diagnosing Immune Thrombocytopenia

    • Authors: Sachs; Ulrich J.
      Abstract: Although the detection of a characteristic autoantibody can prove immune thrombocytopenia (ITP), this diagnosis is often based on the exclusion of other causes of thrombocytopenia. Direct glycoprotein (GP)-specific tests have the property required to demonstrate such a characteristic autoantibody. In contrast, platelet-associated immunoglobulin G or antibody detection in plasma or serum is an insufficient diagnostic test. Moreover, data for commercial capture assays are sparse and their use is currently not recommended. A significant drawback of direct GP-specific tests is their low sensitivity, and a negative test result has no relevance. It is therefore also useful to establish a diagnosis of (primarily) hyperdestructive thrombocytopenia. A full blood count together with the immature platelet fraction has an excellent positive predictive value for ITP. Plasma glycocalicin has no apparent diagnostic value in identifying ITP patients, and conflicting data for TPO preclude its use for diagnostic purposes.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-02-14T00:00:00+0100
      DOI: 10.1055/s-0039-1678739
       
  • Current Trend of Invasive Orthopaedic Interventions for People with
           Haemophilia in Romania: Single Centre Experience

    • Authors: Serban; Margit, Poenaru, Dan Vasile, Patrascu, Jenel Marian, Ritli, Ladislau, Boia, Eugen, Mihailov, Delia Maria, Ursu, Cristina Emilia, Savescu, Delia, Ionita, Ioana, Traila, Adina, Cerbu, Simona, Boeriu, Estera, Jinca, Cristian, Schramm, Wolfgang, Arghirescu, Smaranda
      Abstract: Objective In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. Patients and Methods This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. Results and Discussion The cohort of patients consisted mostly of young adults, aged between 7 and 49 years. The vast majority of them, 91.3% of the patients, had the burden of severe chronic arthropathy. This was the reason for 95 interventions, with programmed solution. In 20 cases the cause of invasive interventions was emergency. Knee and hip replacement represented 28% of the major interventions. The complications we encountered were excessive bleeding (12.2%), infection (13%) and inhibitor development (4.3%). Conclusion Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-02-13T00:00:00+0100
      DOI: 10.1055/s-0039-1677884
       
  • Prevalence of Obesity in Young Patients with Severe Haemophilia and Its
           Potential Impact on Factor VIII Consumption in Germany

    • Authors: Olivieri; Martin, Königs, Christoph, Heller, Christine, Horneff, Silvia, Oldenburg, Johannes, Halimeh, Susan, Kentouche, Karim, Knöfler, Ralf, Fischer, Lars, Pfrepper, Christian, Kurnik, Karin, Bidlingmaier, Christoph
      Abstract: Similar to the general population, overweight and obesity have increasingly become a medical and economic burden also in patients with haemophilia in industrialized nations. In this study in seven German haemophilia centres, we identified a prevalence of overweight and obesity of 25.2% among 254 young patients
      Citation: Hamostaseologie ; : -
      PubDate: 2019-02-05T00:00:00+0100
      DOI: 10.1055/s-0039-1677874
       
  • Potential of Multidimensional, Large-scale Biodatabases to Elucidate
           Coagulation and Platelet Pathways as an Approach towards Precision
           Medicine in Thrombotic Disease

    • Authors: Panova-Noeva; Marina, Eggebrecht, Lisa, Prochaska, Jürgen H., Wild, Philipp S.
      Abstract: Cardiovascular and especially thrombotic diseases remain a major cause of morbidity and mortality worldwide. In past years, significant improvements in understanding disease processes, risk assessment, and prediction of clinical outcome in the field of thrombosis and haemostasis have been made by using large-scale biodatabases. These important research resources enable a comprehensive research approach by integrating clinical, environmental, genomic, and molecular information. Cutting edge, high throughput technologies open new data dimensions for clinical large-scale investigations. Joining multiple information levels from several pathophysiological pathways in contrast to a single marker approach might better model the complexity of disease pathogenesis. This review focuses on the possibilities that ultimately allow conducting wide-scale analyses for unravelling the multifaceted interplay between coagulation and cellular (e.g., platelets) elements in the development of thrombotic disease. It further provides examples for the use of biodatabases in the field of venous thromboembolism, explores the links between venous and arterial thrombotic diseases, and finally informs on the current use of platelet biomarkers in the thrombosis and haemostasis field.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-02-05T00:00:00+0100
      DOI: 10.1055/s-0038-1677520
       
  • Impact of Telemedicine Tools on Record Keeping and Compliance in
           Haemophilia Care

    • Authors: Banchev; A., Goldmann, G., Marquardt, N., Klein, C., Horneff, S., Langenkamp, R., Frankenberger, T., Oldenburg, J.
      Abstract: Background Record keeping is integral to home treatment for haemophilia. Issues with paper diaries include questionable compliance, data validity and quality. Implementation of electronic diaries (e-diaries) in haemophilia patients could improve documentation of home treatment. Aim This article evaluates the effects of an e-diary, Haemoassist, on recording and patient compliance with therapy. Patients and Methods An explorative study was used to assess the sequential use of paper diaries and e-diaries by 99 patients with severe haemophilia A or B and 1 with severe factor VII deficiency. Median age was 41 years. Information was obtained from paper records for 3 years preceding the introduction of an electronic record system and the first 6 to 12 months of Haemoassist use. Data from the 3-year period were averaged. Missing data for rounded 12 months of e-diary use were extrapolated to correspond to a full year. Results Enhancement of 23% in record delivery was observed for the period of Haemoassist use (p = 0.013). Twenty-one percent increase in patients’ compliance for data reporting (from 65% 35 to 86% 22, p = 0.003) and 16% increase for documentation of bleedings (from 68 to 84% of patients, p = 0.01) were detected. Compliance to prescribed therapy of patients for the whole studied period improved by 6% (from 82% ± 29 to 88% ± 25, p = 0.05). Major advances were demonstrated predominantly in the age groups of between 13 and 20 and 21 and 40 years. Conclusion e-Diaries' use enables improved recording of information about patients' home treatment and bleeding episodes. Enhanced compliance with therapy may be a further benefit.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-01-17T00:00:00+0100
      DOI: 10.1055/s-0038-1676128
       
  • Thrombopoietin Receptor Agonist Use for Immune Thrombocytopaenia

    • Authors: Neunert; Cindy E.
      Abstract: Management of patients with corticosteroid-refractory immune thrombocytopaenia (ITP) possesses a significant challenge to practitioners. Until recently, options included splenectomy and immunosuppression. With improved knowledge of both thrombopoiesis and the pathophysiology of ITP, novel drug development with thrombopoietin-receptor agonists (TPO-RAs) was undertaken. Two agents, romiplostim and eltrombopag, are currently approved for use in patients with chronic ITP. Both agents have been shown to increase the platelet count, improve health-related quality of life and reduce bleeding symptoms and concomitant medication use. This review will highlight the discovery of TPO-RA agents, appraise key clinical trials and explore future directions.Behandlung von Patienten mit Kortikosteroid feuerfesten ITP besitzen eine große Herausforderung für Praktiker. Bis vor kurzem waren Optionen Splenektomie und Immunsuppression. Mit verbessert wissen beide Thrombopoeisis und die Pathophysiologie der ITP Roman Medikamentenentwicklung mit Thrombopoetin-Rezeptor-Agonisten (TPO-RAs) wurde durchgeführt. Zwei Agenten, Romiplostim und Eltrombopag, sind derzeit für den Einsatz bei Patienten mit chronischer ITP zugelassen. Beide Agenten haben gezeigt, dass erhöhen die Thrombozytenzahl, gesundheitsbezogene Lebensqualität zu verbessern und verringern Blutungen Symptome und begleitmedikation verwenden. Diese Überprüfung wird Entwicklung von TPO-RA-Agenten zu markieren, wichtige klinische Studien zu beurteilen und zukünftige Richtungen erkunden.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-01-15T00:00:00+0100
      DOI: 10.1055/s-0038-1676129
       
  • Smart Medication ™, an Electronic Diary for Surveillance of Haemophilia
           Home Care and Optimization of Resource Distribution

    • Authors: Mondorf; Wolfgang, Eichler, Hermann, Fischer, Ronald, Holstein, Katharina, Klamroth, Robert, Nimtz-Talaska, Antje, Wermes, Cornelia, Richter, Heinrich, Severin, Kai
      Abstract: Im Folgenden wird das elektronische Substitutionstagebuch smart medication ™ vorgestellt, sowie eine Analyse der damit erhobenen Echtzeitdaten. Seit 2012 wurde smart medication ™ von 663 Patienten in 30 Deutschen Hämophilie Zentren (HZ) genutzt. Daten aus 9 HZ wurden für die nachfolgende Analyse zusammengeführt. Entsprechend der Mittelwerte des jährlichen Faktorenverbrauchs und der Anzahl von Gelenkblutungen wurden vier Gruppen gebildet. Die größte Gruppe (A, 42%) war die mit unterdurchschnittlichem Faktorenverbrauch und weniger als 2,25 Gelenkblutungen pro Jahr. Die zweitgrößte Gruppe (B, 32%) zeigte ähnlich niedrige Blutungsraten bei überdurchschnittlichem Faktorenverbrauch. Eine Reduktion des Faktorenverbrauchs bei einigen dieser Patienten wäre ggf. möglich. Hingegen könnten andere Patienten mit häufigen Gelenkblutungen bei gleichzeitig niedrigem Faktorenverbrauch (D, 13%), von einer Erhöhung der Dosis profitieren. Patienten mit häufigen Gelenkblutungen trotz überdurchschnittlichem Faktorenverbrauch (C, 13%) benötigen eine intensivierte Behandlung jenseits einer Dosisanpassung, wie pharmakokinetisch adaptierte Dosisanpassungen oder orthopädische Maßnahmen. Mit Hilfe von smart medication ™ können Patienten, die eine Änderung ihrer laufenden Behandlung benötigen, lange vor der nächsten Vorstellung im HZ identifiziert werden. Der ständig wachsende Datenpool erlaubt zudem eine kontinuierliche Analyse von Echtzeitdaten und damit eine Optimierung der zur Verfügung stehenden Ressourcen.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-01-08T00:00:00+0100
      DOI: 10.1055/s-0038-1675575
       
  • Role of Endothelial Cells in Acute and Chronic Thrombosis

    • Authors: Bochenek; Magdalena L., Schäfer, Katrin
      Abstract: Haemostasis encompasses a set of strictly regulated actions, such as vasoconstriction, platelet activation and blood coagulation. Endothelial cells play a crucial role in all of these processes and are an integral part of the vascular response to injury resulting in thrombus formation. Healthy endothelium expresses mediators to prevent platelet activation, including prostacyclin and nitric oxide, and to inhibit coagulation, such as thrombomodulin or RNase1. Upon activation, endothelial cells expose von Willebrand factor, integrins and other receptors to interact with activated platelets, erythrocytes and coagulation factors, respectively, resulting in blood clot formation. The endothelial cell response to cytokines and growth factors released from activated platelets and immune cells abundantly present in arterial and venous thrombi also plays an important role for thrombus resolution, whereas failure to completely resolve thrombi may initiate fibrotic remodelling and chronic vascular occlusion both in the arterial and venous tree. Therefore, endothelial cells are increasingly recognized as potential target to prevent thrombotic events and to accelerate thrombus resolution. Here, we discuss recent publications from our group in the context of other studies on the role of the endothelium during acute and chronic thrombotic events.
      Citation: Hamostaseologie ; : -
      PubDate: 2019-01-08T00:00:00+0100
      DOI: 10.1055/s-0038-1675614
       
  • Thromboinflammation and Vascular Dysfunction

    • Authors: Karbach; Susanne, Lagrange, Jérémy, Wenzel, Philip
      Abstract: Thromboinflammation and vascular dysfunction are inseparably combined with the innate and the adaptive immune response. While the role of this interplay has gained considerable attention in the arena of atherosclerosis/atherothrombosis and in deep vein thrombosis, its role in other forms of vascular disease and risk factors is currently emerging. In this brief review, we will focus on thromboinflammation with regard to cytokine signalling as well as on the novel role of a vascular coagulation-inflammatory circuit in arterial hypertension.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-12-04T00:00:00+0100
      DOI: 10.1055/s-0038-1676130
       
  • Functional Classification of Paediatric Patients with Non-syndromic
           Delta-Storage Pool Deficiency

    • Authors: Manukjan; Georgi, Eilenberger, Julia, Andres, Oliver, Schambeck, Christian, Eber, Stefan, Schulze, Harald
      Abstract: Storage pool disease (SPD) covers a group of platelet defects in which α- and/or delta-granules are reduced or cannot be secreted adequately in response to agonists. The detection of delta-granule release defects is hampered by a lack of fast and feasible tests. We aimed to implement a flow cytometry–based kinetic mepacrine assay to better identify and subgroup childhood patients with a mild to moderate bleeding diathesis and compare our method to established laboratory tests. We analysed 50 children with suspected SPD whose initial parameters were re-assessed in a second site visit. Mepacrine uptake and release patterns were correlated with CD63 exposure, platelet ADP/ATP release and content, and the bleeding score ascertained by the ISTH-BAT. Mepacrine release was overall significantly reduced in investigated patients compared with controls. Summarizing, our time-resolved approach proved to be a quick and inexpensive tool that was additionally able to distinguish between mepacrine uptake, mepacrine release, and combined defects. Classification of patients using such a kinetic assay makes it feasible to sensitively detect frequently missed SPD and to group these patients for further analyses and clinical correlations.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-21T00:00:00+0100
      DOI: 10.1055/s-0038-1675574
       
  • The Gut Microbiota as an Influencing Factor of Arterial Thrombosis

    • Authors: Reinhardt; Christoph
      Abstract: The mutualistic gut microbiota does not only impact the development and function of various immune cell types, but it also influences the function of the hepatic vascular endothelium and prothrombotic platelet function. With germ-free mouse models, we have demonstrated that gut-derived microbial-associated molecular patterns could stimulate hepatic von Willebrand factor (VWF) synthesis and plasmatic VWF levels through Toll-like receptor-2 (TLR2), thus defining the extent of platelet deposition to the subendothelial matrix of the ligation-injured common carotid artery. In addition to the microbiota-derived choline metabolite trimethylamine N-oxide and the microbiota's regulatory role on the colonic serotonin biosynthesis pathway, affecting prothrombotic platelet function, TLR2-regulated hepatic endothelial VWF synthesis and elevated VWF plasma levels constitute a pivotal mechanism of how the gut microbiota is linked to arterial thrombosis. Conceptually, in addition to the identified functions of the gut microbiota in modulating host nutrition and metabolism, our work places the innate immune functions of the liver sinusoidal endothelium as an actuating variable in arterial thrombus growth.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-20T00:00:00+0100
      DOI: 10.1055/s-0038-1675357
       
  • Apixaban for the Treatment of Chronic Disseminated Intravascular
           Coagulation: A Report of Two Cases

    • Authors: Janjetovic; Snjezana, Holstein, Katharina, Dicke, Christina, Bokemeyer, Carsten, Langer, Florian
      Abstract: Characteristic features of disseminated intravascular coagulation (DIC) are the opposing risks of bleeding (due to consumptive coagulopathy and hyperfibrinolysis) and organ failure (due to widespread microvascular thromboses). The purpose of anticoagulation in DIC is to attenuate excessive thrombin generation and fibrin deposition. While heparins have been shown to be beneficial in this context, the safety and efficacy of direct oral anticoagulants have not yet been sufficiently addressed. Here, we report two patients in whom chronic DIC was stabilized upon administration of apixaban: an elderly male with aortic dissection presenting with significant mucocutaneous bleeding and a younger female with Klippel–Trénaunay–Weber syndrome presenting with multiple superficial vein thromboses (SVTs). In addition to an improvement in DIC parameters, both patients benefited clinically with resolution of bleeding symptoms and prevention of further SVTs, respectively. Oral apixaban thus showed promising safety and efficacy in the management of DIC caused by vascular abnormalities; still further investigations are needed to support these findings.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-19T00:00:00+0100
      DOI: 10.1055/s-0038-1675386
       
  • New Insights into Platelet Signalling Pathways by Functional and Proteomic
           Approaches

    • Authors: Jurk; Kerstin, Walter, Ulrich
      Abstract: As circulating sentinels of vascular integrity, platelets act as crucial haemostatic cells as well as important inflammatory and immune cells, whereas under pathological conditions platelets drive thrombotic as well as non-thrombotic diseases related to chronic inflammation. In addition, platelets serve as an important cellular model to study the biology and pharmacology of signal transduction pathways. Platelet inhibition and activation responses are mediated by multiple signalling networks, which are tightly regulated by balanced catalysis of protein phosphorylation and dephosphorylation through protein kinases and protein phosphatases, respectively. However, we are only at the beginning of understanding the complexity of interacting signalling pathways and their impact on platelet function. Here, we review current functional and proteomic approaches that lead to novel concepts of understanding the proteome, kinome and phosphatome of human platelets. A more in-depth understanding of both protein kinases and protein phosphatases using human platelets will contribute to evaluate their further diagnostic and therapeutic potential in inflammation- and immune-mediated diseases.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-19T00:00:00+0100
      DOI: 10.1055/s-0038-1675356
       
  • Epidemiology and Treatment of Patients with Haemophilia in
           Austria—Update from the Austrian Haemophilia Registry

    • Authors: Rejtő; Judit, Reitter-Pfoertner, Sylvia, Kepa, Sylvia, Feistritzer, Clemens, Grundbichler, Michael, Hörbst, Alexander, Jones, Neil, Muntean, Wolfgang, Neumeister, Peter, Oberbichler, Stefan, Schuster, Gerhard, Schwarz, Rudolf, Thom, Katharina, Zwiauer, Karl, Streif, Werner, Male, Christoph, Pabinger, Ingrid
      Abstract: The Austrian Haemophilia Registry collects epidemiological data on patients with haemophilia, on treatment modalities and potential side effects. The Registry covers more than 85% of the assumed total number of haemophilia patients in Austria. This report summarizes data on 753 patients: 84.3% (635) have haemophilia A and 15.7% (118) have haemophilia B. Patients' median age is 34 years (range: 1–93 years). Of the total cohort, 39.0% (294) patients have severe haemophilia, 11.3% (85) moderate haemophilia, and 49.4% (372) mild haemophilia. Of the patients with severe haemophilia, 38.4% (113) have been infected with hepatitis C virus (HCV) and 12.6% (37) are human immunodeficiency virus (HIV) positive. Overall, 10.6% (67) of patients with haemophilia A and 1.7% (2) of those with haemophilia B have had an inhibitor in their history. Among patients with severe haemophilia, 68.4% (201) receive prophylaxis and 28.6% (84) receive on-demand therapy. There are 65.0% (191) patients with severe haemophilia who are treated with recombinant products. In conclusion, most patients with severe haemophilia receive prophylactic treatment. HCV and HIV infections are still important issues in the Austrian haemophilia population.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-12T00:00:00+0100
      DOI: 10.1055/s-0038-1675354
       
  • Cofactor-Independent Antiphospholipid Antibodies: Implications for
           Pathogenesis, Diagnosis, and Treatment of Antiphospholipid Syndrome

    • Authors: Lackner; Karl J., Müller-Calleja, Nadine
      Abstract: The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has been intensively studied after the early discovery that it was possible to induce the clinical manifestations in animals by transfer of antiphospholipid antibodies (aPL). In recent years, it has become clear that aPL are not only structurally heterogeneous but also have different pathogenic properties. This review will focus on the relevance of antigenic specificity of aPL in terms of pathogenesis, diagnosis, and perhaps treatment of APS.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-11-12T00:00:00+0100
      DOI: 10.1055/s-0038-1675355
       
  • Optimizing the Personalized, Risk-Adjusted Management of Pulmonary
           Embolism: An Integrated Clinical Trial Programme

    • Authors: Barco; Stefano, Konstantinides, Stavros V.
      Abstract: Acute pulmonary embolism (PE) contributes significantly to the global burden of cardiovascular disease. The severity of the acute PE event determines the expected estimated risk of early death. This risk is influenced by the degree of dysfunction of the right ventricle (RV), as assessed by the presence of acute RV pressure overload on imaging and/or elevated cardiac biomarkers, and by demographic and clinical factors, including relevant comorbidities. Haemodynamic instability and cardiogenic shock is at the top of the PE severity spectrum, as it represents the most extreme manifestation of RV failure and a key determinant of poor prognosis. Ideally, risk-adjusted treatment should implement: (1) optimized timing and regimens of reperfusion therapy for unstable patients; (2) early discharge and continuation of anticoagulation treatment at home (low-risk PE); or (3) hospital admission and clinical/haemodynamic monitoring in patients at intermediate risk. The challenge is now to provide the basis for a comprehensive personalized, risk-adjusted care for patients with acute PE. The aim of the integrated academic clinical trial programme of the Center for Thrombosis and Hemostasis at the University of Mainz is to develop and prospectively validate, in multinational studies, strategies for reperfusion and anticoagulant treatment of acute PE across the entire spectrum of early risk as well as clinical pathways for post-PE patient care and follow-up.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-10-18T00:00:00+01:00
      DOI: 10.1055/s-0038-1673413
       
  • Antiphospholipid Antibodies in a General Obstetric Population: Clinical
           Impact on Pregnancy Outcome and Relationship with the M2 Haplotype in the
           Annexin A5 (ANXA5) Gene

    • Authors: Villani; Michela, Colaizzo, Donatella, Martinelli, Pasquale, Cappucci, Filomena, Fischetti, Lucia, Maruotti, Giuseppe M., Intrieri, Mariano, Greco, Luigi, Chinni, Elena, Tiscia, Giovanni L., Margaglione, Maurizio, Grandone, Elvira
      Abstract: Antiphospholipid (aPL) antibodies are recognised risk factors for adverse obstetric outcomes. Recently, carriers of the M2 haplotype in the Annexin A5 gene have been shown to have a higher susceptibility to develop aPL antibodies. In a general obstetric population, we prospectively evaluated the possible relationship between: (1) aPL antibodies and M2 haplotype; and (2) aPL antibodies and/or M2 haplotype and obstetric outcomes. From a cohort of 3,097 consecutive pregnant women, 1,286 samples were analysed for the presence of both anti-cardiolipin and anti-human β2-glycoprotein I antibodies; samples with available DNA (n = 606) were also investigated for the M2 haplotype. Overall, 41/1,286 (3.2%) women showed the presence of aPL antibodies. Among them, 2 (4.8%) experienced a pregnancy loss and 38 (92.7%) gave birth to live-born babies (p-value = non-significant vs. those without aPL antibodies). M2 haplotype was identified in 140 (23.1%) out of 606 women with DNA available: 3/140 (2.1%) M2 carriers and 17/466 (3.6%) non-carriers tested positive for aPL antibodies, respectively (p-value = non-significant). In total, 15/150 (10%) M2 and/or aPL antibody carriers, and 38/445 (8.5%) non-aPL antibody and/or M2 carriers suffered from obstetric complications, respectively (p-value = non-significant). No relationship between aPL antibodies and M2 haplotype was found. Furthermore, neither aPL antibodies nor the M2 haplotype is associated with obstetric complications.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-10-10T00:00:00+01:00
      DOI: 10.1055/s-0038-1673414
       
  • Endothelial Function in Patients with Severe and Moderate Haemophilia A
           and B

    • Authors: Böhmert; Stephanie, Schubert, Ralf, Fichtlscherer, Stephan, Alesci, Sonja, Miesbach, Wolfgang
      Abstract: The life expectancy of patients with haemophilia has increased and therefore the interest in age-related comorbidities has grown. The aim of this study was to determine whether haemophilia patients have a different endothelial function compared with the general population. A total of 26 patients with severe or moderate haemophilia A or B, 14 controls and 36 patients with coronary artery disease (CAD) were included in this study. Five markers of endothelial dysfunction (MOEDs) were determined. Moreover, the endothelial function was examined using the Itamar Endo-PAT, and the reactive hyperemia index (RHI) was calculated from the results. The MOEDs soluble intercellular adhesion molecule-1 (p = 0.0095) and interleukin-6 (p = 0.010) were significantly higher for patients with haemophilia compared with the control group. The presence of increased adhesion molecule levels and low-grade inflammation is suggestive of a decreased endothelial function. RHI is impaired in CAD patients (1.862), whereas haemophilia patients have an RHI of 1.958 in comparison with 2.112 in controls (p = 0.127). Therefore, laboratory and functional measurements imply a possible higher risk for CAD in haemophilia patients.
      Citation: Hamostaseologie ; : -
      PubDate: 2018-10-09T00:00:00+01:00
      DOI: 10.1055/s-0038-1673415
       
  • First Case of Laparoscopic Mini-Gastric Bypass for the Treatment of Morbid
           Obesity in Severe Haemophilia A
    • Hamostaseologie
      DOI: 10.1055/s-0038-1668571



      Georg Thieme Verlag KG Stuttgart · New York

      Artikel in Thieme eJournals:
      Inhaltsverzeichnis     Volltext

      Hamostaseologie ; : -2018-09-24T00:00:00+01:00
       
 
 
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