MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Seminars in Thrombosis and Hemostasis' source. Wed, 08 Feb 2012 17:42:11 +0100 http://www.medworm.com/index.php?rid=5668143&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300948 Semin Thromb Hemost 2012; 38: 23-30DOI: 10.1055/s-0031-1300948Clinicians prescribing new oral anticoagulants (OACs; dabigatran, rivaroxaban, and apixaban) should be aware of the exclusion criteria related to bleeding risks defined in published clinical studies. At least a quarter of patients currently using warfarin have an exclusion criterion that may prevent easy transition to the new OACs. In the summary of product characteristics for dabigatran, as an example, the target populations appear generalized. Due to fixed dosing and predictable pharmacology, routine laboratory monitoring of new OACs is deemed unnecessary. Under special circumstances, however, understanding the extent of thrombin or factor (F) Xa inhibition may aid in evaluating compliance and handling emergency interventions,... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668143 Tue, 07 Feb 2012 05:00:00 +0100 5668143 http://www.medworm.com/index.php?rid=5668142&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300952 Semin Thromb Hemost 2012; 38: 55-63DOI: 10.1055/s-0031-1300952Regulation of binding between von Willebrand factor (VWF) and the platelet receptor glycoprotein (GP) Ibα is one of the key steps in controlling hemostasis and thrombosis. On vascular injury at sites of high shear rates, the GPIbα interaction with subendothelial-bound VWF will initiate the tethering of circulating platelets to the vessel wall. Tethered platelets subsequently roll on the damaged vessel wall, a process that is amplified by the activation of the platelet integrin αΙΙbβ3 (GPIIb/IIIa). The initial tethering to VWF is rapidly followed by platelet binding to collagen through specific receptors (GPVI and α2β1), leading to firm adhesion, activation, and additional stable bonds mediated by αΙΙbβ3. The above de... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668142 Tue, 07 Feb 2012 05:00:00 +0100 5668142 http://www.medworm.com/index.php?rid=5668141&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300944 Semin Thromb Hemost 2012; 38: 01-04DOI: 10.1055/s-0031-1300944Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668141 Tue, 07 Feb 2012 05:00:00 +0100 5668141 http://www.medworm.com/index.php?rid=5668140&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300950 Semin Thromb Hemost 2012; 38: 38-46DOI: 10.1055/s-0031-1300950Ultralarge von Willebrand factor (ULVWF) multimeric strings are rapidly secreted by, and anchored to, stimulated endothelial cells (EC), and are hyperadhesive to platelets until cleavage by ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). In ADAMTS-13-deficient familial and autoantibody-mediated thrombotic thrombocytopenic purpura (TTP), there is severely restricted cleavage of EC-anchored ULVWF-platelet strings. The small amount of active enzyme released from their EC cleaves ULVWF strings minimally just above EC surfaces, thus generating soluble ULVWF multimers that are 2.5 to 50 times longer than plasma von Willebrand factor (VWF) forms. Soluble ULVWF multimers (detected in TTP an... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668140 Tue, 07 Feb 2012 05:00:00 +0100 5668140 http://www.medworm.com/index.php?rid=5668139&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300954 Semin Thromb Hemost 2012; 38: 79-94DOI: 10.1055/s-0031-1300954Recurrent joint bleeding leading to progressive musculoskeletal damage (hemophilic arthropathy), in spite of on-demand replacement with deficient factor concentrates, is the clinical hallmark of severe hemophilia A and B (i.e., the congenital deficiencies of coagulation factors VIII and IX with circulating levels <1 IU/dL). Fifty years of clinical experience, which began in Northern Europe and then initiated in other European countries and in North America, up to the recent randomized clinical trials, have provided definitive evidence that preventing bleeding from an early age through long-term regular prophylactic concentrate infusions limits the adverse clinical consequences of arthropathy and its complications in the quali... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668139 Tue, 07 Feb 2012 05:00:00 +0100 5668139 http://www.medworm.com/index.php?rid=5668138&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300957 Semin Thromb Hemost 2012; 38: 114-124DOI: 10.1055/s-0031-1300957Vitamin D3 deficiency is a highly prevalent condition worldwide. Clinically, vitamin D3 has a key role in calcium homeostasis and bone mineralization and has recently been implicated in the pathogenesis and/or progression of several acute and chronic illnesses, including cardiovascular disease (CVD). Accumulating evidence from observational, prospective studies suggests that low levels of serum 25-hydroxyvitamin D3 are independently associated with an increased risk of CVD events and death. The molecular mechanisms of this association remain incompletely understood. A variety of biologically plausible mechanisms may mediate a cardiovascular role for the active metabolite of vitamin D3. 1-α,25-dihydroxyvitamin D3 regulates the... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668138 Tue, 07 Feb 2012 05:00:00 +0100 5668138 http://www.medworm.com/index.php?rid=5668137&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300946 Semin Thromb Hemost 2012; 38: 07-15DOI: 10.1055/s-0031-1300946Since the quest for a better replacement of warfarin started several decades ago and new compounds were brought forward to clinical trials, the concept of an ideal anticoagulant frequently became presented in lectures and articles. We have here reviewed strengths and weaknesses of the oral thrombin inhibitor dabigatran in terms of pharmacokinetics and clinical data. When strengths clearly exceed the weaknesses for any characteristic, the drug fits into the concept of an ideal anticoagulant in that domain. It is evident that dabigatran does not accomplish that concept for all characteristics but it reaches well above warfarin. We believe it is unlikely that any drug will fulfill all criteria for the ideal anticoagulant. Laborator... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668137 Tue, 07 Feb 2012 05:00:00 +0100 5668137 http://www.medworm.com/index.php?rid=5668136&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300955 Semin Thromb Hemost 2012; 38: 95-101DOI: 10.1055/s-0031-1300955The relationship between cancer and thrombosis has been recognized for nearly 150 years. Although the mechanisms underlying this association are not completely understood, there are increasing evidences suggesting a pivotal role of thrombin in cancer biology. This review will focus on the most important pathways by which thrombin may affect cancer growth and dissemination. In addition, the potential role of congenital (i.e., hemophilia) and pharmaceutical (i.e., antithrombotic agents) anticoagulation in cancer incidence and survival will be investigated through the analysis of the published experimental and clinical studies.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Ta... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668136 Tue, 07 Feb 2012 05:00:00 +0100 5668136 http://www.medworm.com/index.php?rid=5668135&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300949 The objectives of this review are to discuss currently available drugs for the treatment of HIT, highlight the limitations of these agents, and examine future therapies.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668135 Tue, 07 Feb 2012 05:00:00 +0100 5668135 http://www.medworm.com/index.php?rid=5668134&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300956 Semin Thromb Hemost 2012; 38: 102-113DOI: 10.1055/s-0031-1300956Platelets can release a heterogeneous pool of vesicles which include plasma membrane-derived microparticles (PMPs) and multivesicular body-derived exosomes. As both vesicle types are generated upon activation and their distinction is complicated due to an overlap in their molecular properties and sizes, they are best discussed as an entity, the platelet-derived microvesicles (PMVs). PMPs can be formed through several induction pathways, which determine their different molecular profiles and facilitate tailor-made participation in intercellular communication. This dynamic variability may lie behind the multifaceted and sometimes very different observations of the PMPs in physiological and pathological settings. Currently, littl... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668134 Tue, 07 Feb 2012 05:00:00 +0100 5668134 http://www.medworm.com/index.php?rid=5668133&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300945 Semin Thromb Hemost 2012; 38: 05-06DOI: 10.1055/s-0031-1300945Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668133 Tue, 07 Feb 2012 05:00:00 +0100 5668133 http://www.medworm.com/index.php?rid=5668132&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300951 Semin Thromb Hemost 2012; 38: 47-54DOI: 10.1055/s-0031-1300951Thrombotic microangiopathy (TMA) is a term used to describe a group of disorders characterized by hemolytic anemia (with prominent red blood cell fragmentation), thrombocytopenia, and thrombosis in the microvasculature. It may be used when describing patients with thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome, atypical hemolytic uremic syndrome, as well as a myriad of other disorders in which the TMA may be secondary to another disease or disorder. While limited information exists as to the exact cause of microthrombosis in many TMA, recent advances have been made in the understanding of TTP and its pathophysiology. This progress can be attributed to discovery of the von Willebrand factor cleaving protease... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668132 Tue, 07 Feb 2012 05:00:00 +0100 5668132 http://www.medworm.com/index.php?rid=5668131&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300947 This article reports the effects of dabigatran on clinical routine assays and the potential usefulness for determination in special risk groups of patients when overdose or lack of compliance are suspected.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668131 Tue, 07 Feb 2012 05:00:00 +0100 5668131 http://www.medworm.com/index.php?rid=5668130&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1300953 This article reviews the applications of molecular genetics in hemophilia, in general, and how such techniques can be useful for optimizing patient care, in particular.[...]Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.Article in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5668130 Tue, 07 Feb 2012 05:00:00 +0100 5668130 http://www.medworm.com/index.php?rid=5538794&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297371 Semin Thromb Hemost 2011; 37: 918-928DOI: 10.1055/s-0031-1297371ABSTRACTHistorically, humans have long sought to enhance their “athletic” performance to increase body weight, aggressiveness, mental concentration and physical strength, contextually reducing fatigue, pain, and improving recovery. Although regular training is the mainstay for achieving these targets, the ancillary use of ergogenic aids has become commonplace in all sports. The demarcation between ergogenic aids and doping substances or practices is continuously challenging and mostly based on perceptions regarding the corruption of the fairness of competition and the potential side effects or adverse events arising from the use of otherwise unnecessary ergogenic substances. A kaleidoscope of side effects has been associat... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538794 Fri, 23 Dec 2011 05:00:00 +0100 5538794 http://www.medworm.com/index.php?rid=5538793&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297364 Semin Thromb Hemost 2011; 37: 863-868DOI: 10.1055/s-0031-1297364© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538793 Fri, 23 Dec 2011 05:00:00 +0100 5538793 http://www.medworm.com/index.php?rid=5538792&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297372 Semin Thromb Hemost 2011; 37: 929-945DOI: 10.1055/s-0031-1297372ABSTRACTA variety of disease- and treatment-related factors affect the coagulation system and the risk of bleeding and thrombotic complications in patients with multiple myeloma (MM) and related plasma cell disorders (PCD). As commonly observed in other cancer settings, the malignant clone induces a cytokine environment responsible for a hypercoagulable state. The increase of blood viscosity and impairment of platelet and coagulation function due to circulating monoclonal proteins are considered key mechanisms in the hemostatic abnormalities frequently detected in patients with PCD. However, clinically significant bleeding is relatively rare and poorly correlated with these abnormalities. Management is often challenging becaus... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538792 Fri, 23 Dec 2011 05:00:00 +0100 5538792 http://www.medworm.com/index.php?rid=5538791&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297370 Semin Thromb Hemost 2011; 37: 908-917DOI: 10.1055/s-0031-1297370ABSTRACTThe prompt and accurate diagnosis of pulmonary embolism (PE) greatly influences patient outcomes. However, diagnosing PE is one of the most difficult challenges confronting physicians, even more so when the clinical suspicion is addressed in children, during pregnancy, or in elderly patients. In these patient groups, symptoms and signs from concomitant conditions or diseases may mimic PE and make difficult defining clinical probability categories for PE as usually applied to general adult patients. Moreover, the diagnostic techniques show wider, specific limitations in these settings. PE is considered rare in children. The diagnostic management of a child with suspected PE is largely extrapolated from the knowledge ach... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538791 Fri, 23 Dec 2011 05:00:00 +0100 5538791 http://www.medworm.com/index.php?rid=5538790&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297369 Semin Thromb Hemost 2011; 37: 903-907DOI: 10.1055/s-0031-1297369ABSTRACTThe world is experiencing an obesity pandemic, with rates of obesity rising for more than two decades. Obesity is defined as a body mass index (BMI) of 30 kg/m2 or greater. Of particular concern are the risks that millions of obese people are likely to develop chronic diseases and at earlier ages than their parents might have. The risk of venous thrombosis increases with obesity, so that the incidence of this pathology is also expected to rise significantly. There is remarkable and consistent evidence from a systematic review, as well as cohort and case–control studies that obesity might predispose to venous thromboembolism (VTE). The risk appears to be at least double that for normal weight subjects (BMI 20 to 24.9 ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538790 Fri, 23 Dec 2011 05:00:00 +0100 5538790 http://www.medworm.com/index.php?rid=5538789&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297368 Semin Thromb Hemost 2011; 37: 897-902DOI: 10.1055/s-0031-1297368ABSTRACTVenous thromboembolism (VTE) is one of the most frequent and serious vascular diseases. Although the major risk factors of VTE are well recognized, the pathology often develops in subjects without any obvious precipitating factor. Recent evidence suggests a link between arterial and venous thrombosis, particularly in patients with idiopathic venous thrombosis. Therefore, similar or identical risk factors may play a role in the development of both diseases. A positive association between classical risk factors of atherosclerosis, including dyslipidemia, and VTE has been reported. Recent studies demonstrated an association between hypercholesterolemia and objectively verified VTE. Circulating lipids have been shown to ha... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538789 Fri, 23 Dec 2011 05:00:00 +0100 5538789 http://www.medworm.com/index.php?rid=5538788&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297365 Semin Thromb Hemost 2011; 37: 869-874DOI: 10.1055/s-0031-1297365ABSTRACTThe pathogenesis of acute thrombosis, either arterial or venous, is typically multifactorial and involves a variety of factors that may be considered relatively “innocuous” when present alone. When someone is unlucky enough to accumulate several risk factors, compounded in many cases by one or more acute triggers, that person may be propelled over a threshold that precipitates the development of an acute episode of thrombosis. There is now reliable evidence that acute thromboses (both venous thromboembolism and acute coronary syndrome) follow a typical seasonal pattern and particularly display a characteristic spike during holiday periods. Overindulgence and abrupt changes of several lifestyle habits have been desc... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538788 Fri, 23 Dec 2011 05:00:00 +0100 5538788 http://www.medworm.com/index.php?rid=5538787&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297363 Semin Thromb Hemost 2011; 37: 859-862DOI: 10.1055/s-0031-1297363© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538787 Fri, 23 Dec 2011 05:00:00 +0100 5538787 http://www.medworm.com/index.php?rid=5538786&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297373 Semin Thromb Hemost 2011; 37: 946-954DOI: 10.1055/s-0031-1297373ABSTRACTPatients undergoing chronic hemodialysis have a high risk of arterial thrombotic events as well as vascular access thrombosis (VAT). The latter complication has been consistently associated with inherited (i.e., the prothrombin 20210 polymorphism, and polymorphisms in the genes encoding for transforming growth factor-β1, nitric oxide synthase, plasminogen activator inhibitor-1, angiotensin converting enzyme, and methylene tetrahydrofolate reductase), and acquired thrombotic risk factors (i.e., diabetes, obesity, atrial fibrillation, hypertension, hyperhomocysteinemia, hyperlipoproteinemia(a), low serum albumin, antiphospholipid antibodies, autoantibodies against protein C and S, erythropoietin administration, malnutri... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538786 Fri, 23 Dec 2011 05:00:00 +0100 5538786 http://www.medworm.com/index.php?rid=5538785&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297366 Semin Thromb Hemost 2011; 37: 875-884DOI: 10.1055/s-0031-1297366ABSTRACTThe consequences of heavy or irregular alcohol drinking have long been known. Recently, consistent information has been provided in support of an association between light/moderate alcohol consumption and protection from vascular and all-cause mortality, ischemic stroke, peripheral arterial disease, congestive heart failure, and recurrence of ischemic events. After reviewing the information with respect to major aspects of cardiovascular pathophysiology, to potential confounders and to underlying mechanisms, several concepts emerge. First, the recommended amounts of “safe alcohol drinking” in healthy individuals are up to two standard drinks (∼20 g/d) for a man and up to one drink (10 g/d) for a nonpregnant woman... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538785 Fri, 23 Dec 2011 05:00:00 +0100 5538785 http://www.medworm.com/index.php?rid=5538784&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297375 This article provides an overview of microthrombosis in small blood vessels, with discussion of the progressive stages of its development. The initial event is triggered by a variety of diseases, followed by a second phase when multiple contributory factors amplify the process with the final phase of microvascular occlusion and microvascular thrombosis. The outcome is either recovery or injury to the affected organ. If the process is generalized, it is often associated with catastrophic or fatal outcomes. Our current knowledge of the major role of contributory factors leads to a new paradigm. A therapeutic approach limited to a single target of the underlying pathogenic factor, such as the use of anticoagulants, is insufficient and too often unsuccessful. Simultaneous management of all the... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538784 Fri, 23 Dec 2011 05:00:00 +0100 5538784 http://www.medworm.com/index.php?rid=5538783&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297367 Semin Thromb Hemost 2011; 37: 885-896DOI: 10.1055/s-0031-1297367ABSTRACTVenous thrombosis and arterial thrombosis are traditionally regarded as two different diseases with respect to pathophysiology, epidemiology, and treatment strategies. Research findings of the past few years suggest that this categorical distinction may be too strict. However, whether the described relationship between venous and arterial thrombosis is real or a result of other factors such as confounding, chance, or bias is still unclear. In this review, we discuss the current literature while using causal diagrams to better understand possible causal relations between cardiovascular risk factors, atherosclerosis, arterial thrombosis, and venous thrombosis. Furthermore, we propose study designs to investigate the caus... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538783 Fri, 23 Dec 2011 05:00:00 +0100 5538783 http://www.medworm.com/index.php?rid=5538782&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297374 Semin Thromb Hemost 2011; 37: 955-960DOI: 10.1055/s-0031-1297374ABSTRACTIn severe infection and sepsis, activation of coagulation frequently occurs, which contributes to the development of multiple organ dysfunction. Factor V Leiden is a relatively common mutation resulting in a mild prohemostatic state and consequently with an increased tendency to develop thrombosis. Hypothetically, patients with factor V Leiden may suffer from more severe coagulopathy in cases of severe infection or sepsis. Aggravation of the procoagulant state in sepsis may subsequently result in more severe organ dysfunction and an increased risk of death. In this article we review the experimental and clinical evidence regarding the relationship between the presence of a factor V Leiden mutation and the incidence and... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5538782 Fri, 23 Dec 2011 05:00:00 +0100 5538782 http://www.medworm.com/index.php?rid=5526280&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1295635 Semin Thromb Hemost 2011; 37: 856-858DOI: 10.1055/s-0031-1295635© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526280 Wed, 21 Dec 2011 05:00:00 +0100 5526280 http://www.medworm.com/index.php?rid=5526279&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297174 This article will review surgical procedures, such as BTS, Glenn shunt, and Fontan shunt, prosthetic mechanical valves, and CPB, and their risk of thrombotic complications. There is insufficient evidence and no consensus for optimal anticoagulant prophylaxis or treatment in children with CHD. Current recommendations are mostly based on adult data.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526279 Tue, 20 Dec 2011 05:00:00 +0100 5526279 http://www.medworm.com/index.php?rid=5526278&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297173 Semin Thromb Hemost 2011; 37: 814-825DOI: 10.1055/s-0031-1297173ABSTRACTWithin the last two decades low molecular weight heparins (LMWH) have gained increasing widespread use as anticoagulants in children. The use of LMWH has been implemented into clinical care even though there is a lack of firm evidence on the efficacy and safety of LMWH in this population due to the absence of sufficiently powered randomized controlled trials. In the absence of clinical trials, we performed a meta-analysis of available single-arm studies using LMWH in children. A systematic search of electronic databases (Medline, EMBASE, OVID, Web of Science, The Cochrane Library) for studies published from 1980 to 2010 was conducted using keywords in combination both as MeSH terms and text words. Two authors independe... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526278 Tue, 20 Dec 2011 05:00:00 +0100 5526278 http://www.medworm.com/index.php?rid=5526277&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297165 Semin Thromb Hemost 2011; 37: 745-755DOI: 10.1055/s-0031-1297165ABSTRACTChildhood immune thrombocytopenia (ITP) is generally a benign self-limiting disorder of young children with <10% of cases requiring regular platelet enhancing therapy at 1 year following diagnosis. Increasingly, children with newly diagnosed ITP, who have isolated thrombocytopenia and no atypical features in the history or physical examination, are managed with minimal investigation and observation alone. The role of up-front, short-course corticosteroid therapy without bone marrow aspiration in this subgroup of cases merits further investigation. For children with clinically significant chronic ITP, the timing of elective splenectomy and the role of splenectomy-sparing strategies such as rituximab continues to be d... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526277 Tue, 20 Dec 2011 05:00:00 +0100 5526277 http://www.medworm.com/index.php?rid=5526276&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297177 Semin Thromb Hemost 2011; 37: 848-855DOI: 10.1055/s-0031-1297177ABSTRACTAs a result of technological advances in the field of high-throughput genomics, there has been a remarkable transition in studying the nature of complex genetic disorders. The genetic analysis of prothrombotic risk factors has shifted from candidate gene to genome-wide association studies (GWAS) in adults. GWAS established a framework in which up to 90% of common genetic variation can be analyzed in a single experiment. Given the ubiquity of the GWAS approach in the adult population, it will become essential for clinicians and researchers in the field of pediatrics to interpret results derived from genetic high-throughput studies. Here, we review the current knowledge regarding genetic factors affecting prothrombotic r... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526276 Tue, 20 Dec 2011 05:00:00 +0100 5526276 http://www.medworm.com/index.php?rid=5526275&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297166 Semin Thromb Hemost 2011; 37: 756-762DOI: 10.1055/s-0031-1297166ABSTRACTChildren presenting with bleeding symptoms pose a diagnostic challenge. The evaluation should include a comprehensive medical and bleeding history, detailed family history, a physical examination, and selected laboratory tests. Evaluation of the bleeding history should include an objective quantitation of the bleeding symptoms, followed by objective quantitation of the family members' bleeding symptoms. If a bleeding disorder is suspected, based on personal and/or family bleeding history, specific laboratory testing is needed for attaining a diagnosis. The screening tests for coagulation factor deficiencies are considered reliable, routinely available, and easy to perform. However, for primary hemostatic defects, the v... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526275 Tue, 20 Dec 2011 05:00:00 +0100 5526275 http://www.medworm.com/index.php?rid=5526274&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297170 This article reviews the benefits and limitations of testing for thrombophilic disorders, for example, factor V Leiden, prothrombin mutation, and deficiencies of antithrombin, protein C, or protein S in childhood.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526274 Tue, 20 Dec 2011 05:00:00 +0100 5526274 http://www.medworm.com/index.php?rid=5526273&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297163 Semin Thromb Hemost 2011; 37: 730-736DOI: 10.1055/s-0031-1297163ABSTRACTIt has long been considered that a severe coagulation deficiency in premature newborns could be a major contributing factor in the occurrence of intraventricular hemorrhage (IVH). High-grade IVH has also been shown to coincide with severe derangement of coagulation in extremely low birth weight infants. This review focuses on the relevance of the physiologically developing immature hemostatic system to IVH, and the potential benefit of agents affecting hemostasis for IVH therapy or prevention in preterm infants. The findings of small, open-label interventional studies on the effect of ethamsylate, vitamin K, fresh frozen plasma, recombinant activated factor VII, and prothrombin complex concentrate on the premature coag... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526273 Tue, 20 Dec 2011 05:00:00 +0100 5526273 http://www.medworm.com/index.php?rid=5526272&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297168 This article reviews the progresses that occurred to PE in children placing them in historical perspective with the key events relevant to PE in adults. A brief summary of the initial reports encountered in the pediatric literature followed by key conclusions drawn from national database reports characterizing its epidemiology in children is highlighted. Additionally, a section with the diagnostic tools pertaining to children is included. Closing remarks encompass commentaries related to therapy and outcomes, reflecting on current knowledge gaps related to PE in children.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526272 Tue, 20 Dec 2011 05:00:00 +0100 5526272 http://www.medworm.com/index.php?rid=5526271&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297176 This article expands on these recommendations by summarizing and reviewing the current literature in pediatric anticoagulation studies; discussing the relationship of choice of outcomes to specific clinical trial design; providing a more detailed discussion on the rationale for the defined outcomes in pediatric anticoagulation trials, as well as a detailed examination of secondary and tertiary outcomes. With a growing interest in multicenter, multinational pediatric anticoagulation clinical trials, establishment of standardized outcome measures that are applied uniformly over all studies is critically important. Agreement from the international academic community on these outcomes is an important step in assuring well-designed clinical trials, which are implemented to optimize the preventi... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526271 Tue, 20 Dec 2011 05:00:00 +0100 5526271 http://www.medworm.com/index.php?rid=5526270&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297175 Semin Thromb Hemost 2011; 37: 834-839DOI: 10.1055/s-0031-1297175ABSTRACTMeasurement of quality of life (QOL) has been accepted as an important outcome measure in therapeutic clinical trials. Long-term antithrombotic therapy is hypothesized to induce treatment dissatisfaction and influence QOL. Health-related quality of life (HRQOL) can be measured by an inventory developed specific to the patient condition. Pediatric QOL inventory for children on long-term antithrombotic therapy should assess constructs salient for this population. Creation of an HRQOL measurement inventory requires rigor and methodological adherence. Identification and evaluation of QOL constructs is critical to improve care and is accepted as the “gold standard” measurement for patient-centered outcomes in clinical r... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526270 Tue, 20 Dec 2011 05:00:00 +0100 5526270 http://www.medworm.com/index.php?rid=5526269&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297167 Semin Thromb Hemost 2011; 37: 763-761DOI: 10.1055/s-0031-1297167ABSTRACTThrombosis in children is becoming more prevalent due to increased awareness of these issues in the pediatric population and advances in medicine. Management of affected children are challenging due to differences in their hemostatic system compared with adults. Prospective, controlled trials for management/treatment of children with thrombosis are lacking. Many of the available guidelines for treatment of thrombosis in children are extrapolated from adult data and do not account for the uniqueness of the pediatric hemostatic system, although more research and data are becoming available. This review will focus on children over 1 year of age, including adolescents, looking at the etiology of thrombosis, diagnosis, mana... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526269 Tue, 20 Dec 2011 05:00:00 +0100 5526269 http://www.medworm.com/index.php?rid=5526268&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297172 Semin Thromb Hemost 2011; 37: 810-813DOI: 10.1055/s-0031-1297172ABSTRACTIncreased levels of lipoprotein(a) (Lp[a])are known independent risk factor for atherosclerosis, heart disease, and stroke in adults. Even in children it could be shown that elevated levels of Lp(a) are an independent risk factor for symptomatic thromboembolism. The aim of this work was to describe the methods used for evaluating Lp(a) phenotypes, to link them to Lp(a) plasma concentrations, and to establish age-dependent reference values in children. Lp(a) plasma concentrations were measured with enzyme-linked immunosorbent assay technique in parallel to agarose gel electrophoresis and subsequent anti-apolipoprotein(a) immunoblotting. We included 184 pediatric patients with stroke or venous thromboembolism, and 150 he... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526268 Tue, 20 Dec 2011 05:00:00 +0100 5526268 http://www.medworm.com/index.php?rid=5526267&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297171 Semin Thromb Hemost 2011; 37: 802-809DOI: 10.1055/s-0031-1297171ABSTRACTThe aim of this study was to estimate the impact of antiphospholipid (aPL) antibodies on the risk of incident thromboembolism (TE; arterial and venous) in children via meta-analysis of published observational studies. A systematic search of electronic databases (Medline, EMBASE, OVID, Web of Science, The Cochrane Library) for studies published from 1966 to 2010 was conducted using keywords in combination both as MeSH terms and text words. Two authors independently screened citations and those meeting the a priori defined inclusion criteria were retained. Data on year of publication, study design, country of origin, number of patients/controls, ethnicity, TE type, and frequency of recurrence were abstracted. Heterogenei... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526267 Tue, 20 Dec 2011 05:00:00 +0100 5526267 http://www.medworm.com/index.php?rid=5526266&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297161 Semin Thromb Hemost 2011; 37: 721-722DOI: 10.1055/s-0031-1297161© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526266 Tue, 20 Dec 2011 05:00:00 +0100 5526266 http://www.medworm.com/index.php?rid=5526265&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297169 Semin Thromb Hemost 2011; 37: 786-793DOI: 10.1055/s-0031-1297169ABSTRACTChildhood arterial ischemic stroke (AIS) is a rare, but serious, medical condition, which is fatal in approximately 3% and associated with both acute and long-term neurologic impairment in over 70% of cases. Common etiologies include sickle cell disease, congenital heart disease, arterial dissection, prothrombotic conditions, and preceding viral infections; however, one in four cases is considered idiopathic. To date, no randomized controlled clinical trials (RCTs) have been conducted to establish evidence for current therapeutic strategies outside of sickle cell disease, thus, treatment strategies are largely shaped by consensus-based guidelines, in which, beyond the acute period, aspirin is the mainstay of therapy an... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526265 Tue, 20 Dec 2011 05:00:00 +0100 5526265 http://www.medworm.com/index.php?rid=5526264&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297162 This article will initially discuss the most recent evidence with respect to qualitative age-related changes in the coagulation system. Subsequently the article will discuss the coagulation system during childhood in light of the three aforementioned areas of clinical impact and suggest possible strategies to further understand this complex and exciting field of study.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526264 Tue, 20 Dec 2011 05:00:00 +0100 5526264 http://www.medworm.com/index.php?rid=5526263&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1297164 Semin Thromb Hemost 2011; 37: 737-744DOI: 10.1055/s-0031-1297164ABSTRACTThe hemophilias are the most common X-linked inherited bleeding disorders, which if not properly managed can lead to chronic disease and lifelong disabilities. The challenges and issues in newborns are different from that in older children and adults. Bleeding events still predominate as the diagnostic trigger in children, however, the sites of bleeding vary with age. While delivery-associated intracranial hemorrhage (ICH), circumcision, and venipuncture bleeding are common in the newborn period, joint disease and head trauma occur in the older child and adolescent. Awareness of clinical manifestations and treatment complications are crucial in instituting appropriate management and implementing preventive strategies. ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5526263 Tue, 20 Dec 2011 05:00:00 +0100 5526263 http://www.medworm.com/index.php?rid=5423240&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281042 Semin Thromb Hemost 2011; 37: 555-559DOI: 10.1055/s-0031-1281042ABSTRACTNew Zealand is a small country of two islands and 4 million people, of which 1 million reside in the South Island. Canterbury Health Laboratories provides laboratory services to the whole of South Island and lower parts of North Island. There are 155 von Willebrand disease (VWD) patients in our South Island database, of which 17 have type 2 and 3 have type 3 VWD. A brief overview of diagnostic services for VWD being followed in our region is detailed in this article. We strive continually to advance the repertoire of diagnostic tests. We also present an analysis of our experience with a flow-based functional von Willebrand factor assay. The VWD patients are managed by hemostasis team members, who also provide screening... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423240 Fri, 18 Nov 2011 05:00:00 +0100 5423240 http://www.medworm.com/index.php?rid=5423239&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281036 Semin Thromb Hemost 2011; 37: 503-510DOI: 10.1055/s-0031-1281036ABSTRACTThe correct diagnosis and classification of von Willebrand disease (VWD) is important for therapy and genetic counseling but is made difficult due to the variability of its clinical expression and limitations of laboratory methods. A national registry of VWD patients has been initiated in Spain. The results of a concise survey on the diagnosis of VWD show the frequency of VWD is fivefold greater in Spain than that expected from epidemiological studies in other European countries; this may result from overdiagnosis and/or a higher prevalence of VWD. These results clearly reinforce the need for the Spanish VWD registry. A consensus guideline for optimal treatment of VWD is being elaborated in Spain. Desmopressin (DDAVP) ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423239 Fri, 18 Nov 2011 05:00:00 +0100 5423239 http://www.medworm.com/index.php?rid=5423238&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1280567 Semin Thromb Hemost 2011; 37: 425-426DOI: 10.1055/s-0031-1280567© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423238 Fri, 18 Nov 2011 05:00:00 +0100 5423238 http://www.medworm.com/index.php?rid=5423237&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291382 Semin Thromb Hemost 2011; 37: 713-720DOI: 10.1055/s-0031-1291382ABSTRACTQuebec platelet disorder (QPD) is an autosomal dominant bleeding disorder associated with reduced platelet counts and a unique gain-of-function defect in fibrinolysis due to increased expression and storage of urokinase plasminogen activator (uPA) by megakaryocytes. QPD increases risks for bleeding and its key clinical feature is delayed-onset bleeding, following surgery, dental procedures or trauma, which responds only to treatment with fibrinolytic inhibitors. The genetic cause of the disorder is a tandem duplication mutation of the uPA gene, PLAU, which upregulates uPA expression in megakaryocytes by an unknown mechanism. The increased platelet stores of uPA trigger plasmin-mediated degradation of QPD α-granule pro... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423237 Fri, 18 Nov 2011 05:00:00 +0100 5423237 http://www.medworm.com/index.php?rid=5423236&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281048 Semin Thromb Hemost 2011; 37: 595-601DOI: 10.1055/s-0031-1281048ABSTRACTvon Willebrand disease (VWD) in all developing countries including India is considered a rare coagulation disorder, contrary to many reports from Western countries. Prevalence data based on hospital referrals identifies type 3 VWD as the most common subtype followed by type 1 and type 2. Approximately 60 to 70% cases of type 3 VWD are reportedly born of consanguineous marriages. The discriminatory diagnostic tests mainly include assays for factor (F)VIII:C and ristocetin-induced platelet agglutination and von Willebrand factor (VWF) antigen either by immunoelectrophoresis or by enzyme-linked immunosorbent assay. VWD-type assisting tests like VWF collagen binding, VWF ristocetin cofactor assay, VWF-FVIII binding assay, ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423236 Fri, 18 Nov 2011 05:00:00 +0100 5423236 http://www.medworm.com/index.php?rid=5423235&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281047 Semin Thromb Hemost 2011; 37: 587-594DOI: 10.1055/s-0031-1281047ABSTRACTSpecial challenges exist in the management of patients with von Willebrand disease (VWD) because of limitations in diagnostic facilities and therapeutic options. However, even within these limitations, it is possible to establish comprehensive services for this condition. Our data show that among 202 patients with VWD, 107 were type 3, 62 were type 1, and the others different categories of type 2. Basic tests such as bleeding time and activated partial thromboplastin time with factor (F)VIII coagulant are able to diagnose most of those with severe disease. We have been able to adapt the specific tests such as von Willebrand factor (VWF) ristocetin cofactor and VWF antigen from the tedious batched manual methods to cost... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423235 Fri, 18 Nov 2011 05:00:00 +0100 5423235 http://www.medworm.com/index.php?rid=5423234&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281043 This article reports on the clinical and laboratory data of a large pediatric cohort in one single Argentinian center. The biological and clinical responses to desmopressin and replacement therapies are also described. Over a 15-year period, 194 of 1150 children (16.9%) were diagnosed as having type 1 VWD (80%), type 2 VWD (19%), and type 3 VWD (1%). The distribution of the different type 2 VWD subtypes was type 2A VWD, 43%; type 2B VWD, 32%; type 2M VWD, 19%; and type 2N VWD, 6%. Eighty patients with type 1 VWD and 12 patients with type 2 VWD were prospectively evaluated to desmopressin (DDAVP) response. A complete response was observed in all children with type 1 VWD, whereas 40% of the children with severe type 1 VWD and with type 2 VWD achieved a complete response. All the children who... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423234 Fri, 18 Nov 2011 05:00:00 +0100 5423234 http://www.medworm.com/index.php?rid=5423233&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281029 This article demonstrates how measuring VWFpp and calculating the VWFpp-to-VWF:antigen ratio (VWFpp ratio) are good alternatives to DDAVP for investigating VWF survival. In type 1 VWD, the VWFpp ratio has been found normal in patients with pure quantitative VWF defects, markedly increased in cases with an isolated decline in VWF survival, and more or less increased in patients with both quantitative defects and a shorter VWF survival. The same applies to type 2B VWD, which is characterized by an increased VWFpp ratio and a shorter VWF survival, with values that appear inversely related. Exploring VWF half-life by assaying VWFpp is useful not only for the more precise characterization of VWD but also for defining its most appropriate treatment.[...]© Thieme Medical PublishersArticle in Thi... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423233 Fri, 18 Nov 2011 05:00:00 +0100 5423233 http://www.medworm.com/index.php?rid=5423232&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291378 Semin Thromb Hemost 2011; 37: 682-689DOI: 10.1055/s-0031-1291378ABSTRACTThe GATA family of transcription factors, including the founding member, GATA-1, have an important role in gene regulation. GATA-1 is integral to successful hematopoiesis. A wide variety of mutations in GATA-1 affect its function, as well as its interaction with its cofactors (especially Friend of GATA) and the genes upon which GATA-1 acts. Here we review the known mutations, focusing on the specific alterations within the amino acid sequence, the resulting effect on hematopoietic development, and the clinical manifestations that result. Attention is also paid to the relationship between Trisomy 21, also known as Down syndrome, and the phenomenon of a truncated GATA-1, named GATA-1s. The evidence for specific interacti... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423232 Fri, 18 Nov 2011 05:00:00 +0100 5423232 http://www.medworm.com/index.php?rid=5423231&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1280568 Semin Thromb Hemost 2011; 37: 429-430DOI: 10.1055/s-0031-1280568© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423231 Fri, 18 Nov 2011 05:00:00 +0100 5423231 http://www.medworm.com/index.php?rid=5423230&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281035 Semin Thromb Hemost 2011; 37: 431-439DOI: 10.1055/s-0031-1281035© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423230 Fri, 18 Nov 2011 05:00:00 +0100 5423230 http://www.medworm.com/index.php?rid=5423229&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291370 Semin Thromb Hemost 2011; 37: 615-616DOI: 10.1055/s-0031-1291370© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423229 Fri, 18 Nov 2011 05:00:00 +0100 5423229 http://www.medworm.com/index.php?rid=5423228&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291379 Semin Thromb Hemost 2011; 37: 690-697DOI: 10.1055/s-0031-1291379ABSTRACTOver the last two decades the genetic causes of several Mendelian platelet disorders have been elucidated, while the genetics of many other thrombocytopenic conditions are still unresolved. Among those are the gray platelet syndrome (GPS) and the thrombocytopenia linked to the THC2 locus on human chromosome 10p11–12. GPS is an α-granule defect associated with the development of myelofibrosis and mild to moderate thrombocytopenia. Most forms of GPS are autosomal recessive, and recently, the recessive form of the disease was mapped to chromosome 3p21. THC2-linked thrombocytopenia is an autosomal dominant disorder in which affected family members have a mild reduction in platelet counts and occasional bleeding. Platele... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423228 Fri, 18 Nov 2011 05:00:00 +0100 5423228 http://www.medworm.com/index.php?rid=5423227&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281045 Semin Thromb Hemost 2011; 37: 576-580DOI: 10.1055/s-0031-1281045ABSTRACTPatients with von Willebrand disease (VWD) in South Africa are cared for in 17 Hemophilia Treatment Centers. The exact prevalence of the disease is uncertain, but 539 patients are annotated in registries. VWD patients are mostly diagnosed in the five largest academic centers, and the classification of the subtypes is performed by one of these, the VWD testing facility. An algorithm is used for the diagnosis of VWD. The distribution of subtypes diagnosed by the VWD reference center is 38%, 58%, and 4% for type 1, 2, and 3, respectively, and ~15% of plasma samples received are rejected due to poor storage and transport conditions. A novel single nucleotide polymorphism has been found in an African patient with type 2B VW... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423227 Fri, 18 Nov 2011 05:00:00 +0100 5423227 http://www.medworm.com/index.php?rid=5423226&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1280566 Semin Thromb Hemost 2011; 37: 427-428DOI: 10.1055/s-0031-1280566© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423226 Fri, 18 Nov 2011 05:00:00 +0100 5423226 http://www.medworm.com/index.php?rid=5423225&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291374 Semin Thromb Hemost 2011; 37: 640-652DOI: 10.1055/s-0031-1291374ABSTRACTPregnancy is associated with physiological and pathological changes in platelet numbers and function, which can be of clinical concern because of risks for maternal and fetal or neonatal bleeding. Thrombocytopenia in pregnancy is frequently encountered and may be due to increased platelet turnover and plasma dilution, immune-mediated mechanisms, or a complication of a more severe underlying pregnancy-related disorder such as preeclampsia. Inherited defects in platelet function and number may also manifest during pregnancy with the risk of bleeding dependent on the underlying problem. In some women, the diagnosis of thrombocytopenia will precede pregnancy but in others, the problem is first identified when routine pregn... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423225 Fri, 18 Nov 2011 05:00:00 +0100 5423225 http://www.medworm.com/index.php?rid=5423224&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291377 Semin Thromb Hemost 2011; 37: 673-681DOI: 10.1055/s-0031-1291377ABSTRACTCongenital amegakaryocytic thrombocytopenia (CAMT, MIM #604498) is a rare inherited bone marrow failure syndrome presenting as isolated hypomegakaryocytic thrombocytopenia at birth without other characteristic physical anomalies. Most of the patients develop a severe aplastic anemia and trilineage cytopenia during the first years of life and hematopoietic stem cell transplantation is the only curative treatment. In most of the cases the disease is caused by homozygous or compound heterozygous mutations in the gene MPL encoding the receptor for the hematopoietic growth factor thrombopoietin. The present review summarizes clinical and laboratory data for 96 patients with CAMT, reported since 1990.[...]© Thieme Medical P... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423224 Fri, 18 Nov 2011 05:00:00 +0100 5423224 http://www.medworm.com/index.php?rid=5423223&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281037 Semin Thromb Hemost 2011; 37: 511-521DOI: 10.1055/s-0031-1281037ABSTRACTvon Willebrand disease (VWD) is the most common inherited bleeding disorder and is due to quantitative and/or qualitative defects of von Willebrand factor (VWF). Despite the improved knowledge of the disease, detailed data on VWD types requiring specific treatments have not been reported thus far. To determine the number and types of VWD requiring therapy with desmopressin (DDAVP) and/or VWF/FVIII concentrates in Italy, a national registry on VWD (RENAWI) was organized. Only 16 of 48 centers included VWD in the RENAWI with diagnoses performed locally. Patients with uncertain results were retested by two expert laboratories using multimeric analysis and mutations of the VWF gene. A total of 1234 of 1529 (81%) cases sati... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423223 Fri, 18 Nov 2011 05:00:00 +0100 5423223 http://www.medworm.com/index.php?rid=5423222&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281041 Semin Thromb Hemost 2011; 37: 542-554DOI: 10.1055/s-0031-1281041ABSTRACTvon Willebrand disease (VWD) is the most common inherited bleeding disorder and arises from deficiencies and/or defects in the plasma protein von Willebrand factor (VWF). VWD is classified into six different types, with type 1 identifying a (partial) quantitative deficiency of VWF, type 3 defining a (virtual) total deficiency of VWF, and type 2 identifying four separate types (2A, 2B, 2M, and 2N) characterized by qualitative defects. The classification is based on phenotypic assays including factor VIII coagulant, VWF antigen, and VWF activity, primarily by ristocetin cofactor and collagen binding, as supplemented by additional testing. In Australia, >30 pathology-based laboratories perform VWD testing, and tests an... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423222 Fri, 18 Nov 2011 05:00:00 +0100 5423222 http://www.medworm.com/index.php?rid=5423221&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281046 Semin Thromb Hemost 2011; 37: 581-586DOI: 10.1055/s-0031-1281046ABSTRACTvon Willebrand disease (VWD) is caused by a deficiency or dysfunction of von Willebrand factor (VWF). The pathophysiology, classification, diagnosis, and management of VWD are relatively complex, but their understanding is important for proper diagnosis and management of patients with VWD. There are inherent difficulties in both the identification and classification of VWD because of clinical uncertainty and the limitations in the test processes and test panels typically used by laboratories. The most common test panel employed by laboratories, particularly in the geographic regions covered by the mutational studies, would comprise factor VIII coagulant (FVIII:C), VWF protein (antigen; VWF:Ag), and ristocetin cofactor ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423221 Fri, 18 Nov 2011 05:00:00 +0100 5423221 http://www.medworm.com/index.php?rid=5423220&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291380 Semin Thromb Hemost 2011; 37: 698-706DOI: 10.1055/s-0031-1291380ABSTRACTGlanzmann thrombasthenia (GT) is the most widely studied inherited disorder of platelets; it is caused by the absence of platelet aggregation due to quantitative and/or qualitative deficiencies of the αIIbβ3 integrin coded by the ITGA2B and ITGB3 genes located at 17q21–23. Although platelet count and platelet volume (and morphology) are normal in classic GT, some reports have inferred a role for αIIbβ3 in megakaryocytopoiesis and some novel but rare point mutations in either of the ITGA2B and ITGB3 genes have been associated with an altered platelet production and selective deficiencies in platelet function. This was brought to light by the discovery of mutations at Arg995 in αIIb and Asp723 in β3 that lead to ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423220 Fri, 18 Nov 2011 05:00:00 +0100 5423220 http://www.medworm.com/index.php?rid=5423219&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281049 Semin Thromb Hemost 2011; 37: 602-606DOI: 10.1055/s-0031-1281049ABSTRACTLittle information is available on von Willebrand disease (VWD) in Iran. More than 900 patients with VWD are registered, but the real number of patients with VWD is likely to be larger. Fifteen hemophilia treatment centers in addition to 31 other academic centers are the main sites for the clinical management and treatment of VWD in Iran. All centers are staffed by specialists in the medical care of VWD. Epistaxis and menorrhagia are the most frequent clinical manifestations in Iranian patients with VWD. von Willebrand factor/factor VIII concentrates, desmopressin, cryoprecipitates, and tranexamic acid are the main medications used routinely in Iranian patients, and they are administered based on the type of disease, t... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423219 Fri, 18 Nov 2011 05:00:00 +0100 5423219 http://www.medworm.com/index.php?rid=5423218&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291373 Semin Thromb Hemost 2011; 37: 631-639DOI: 10.1055/s-0031-1291373AbstractThe precise mechanisms leading to platelet-targeted autoimmunity in immune thrombocytopenia (ITP) are not known. Cellular checkpoints normally regulate immunological self-reactivity during the development of B and T cells through cell deletion, receptor editing, induction of anergy, and extrinsic cellular suppression. When these checkpoints fail, tolerance to self-antigens may be lost. In this review, we summarize the various immune mechanisms contributing to the development of ITP and relate them back to the checkpoint model of autoimmunity. These mechanisms, including increased levels of lymphocyte growth factors, resistance to death signals, and loss of T-regulatory function, result in an environment permissive to t... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423218 Fri, 18 Nov 2011 05:00:00 +0100 5423218 http://www.medworm.com/index.php?rid=5423217&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281034 This article summarizes the guidelines of the Nordic Haemophilia Council (NHC), which are intended to serve as a practical tool and provide the standards for diagnosing and treating VWD patients. The complete Nordic Guidelines on VWD are available at the NHC Web site (http://nordhemophilia.org).[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423217 Fri, 18 Nov 2011 05:00:00 +0100 5423217 http://www.medworm.com/index.php?rid=5423216&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281039 Semin Thromb Hemost 2011; 37: 528-534DOI: 10.1055/s-0031-1281039ABSTRACTvon Willebrand disease (VWD) is a common bleeding disorder with prevalence in the United States of 0.01 to 1% and a prevalence in the region around Milwaukee, Wisconsin, of at least 0.025%. Care of local patients with VWD primarily occurs through our comprehensive treatment centers, although some patients are managed solely by their primary care physician or community hematologist. Type 1 VWD is the most common subtype, with more females carrying this diagnosis than males. Diagnosis and treatment in general follows guidelines outlined by the National Institutes of Health. An ongoing study, the Zimmerman Program for the Molecular and Clinical Biology of VWD, is currently enrolling patients with all VWD subtypes across t... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423216 Fri, 18 Nov 2011 05:00:00 +0100 5423216 http://www.medworm.com/index.php?rid=5423215&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281044 Semin Thromb Hemost 2011; 37: 568-575DOI: 10.1055/s-0031-1281044ABSTRACTvon Willebrand disease (VWD) is a bleeding disorder with variable clinical expression. In this article we describe types, clinical features, genetic testing when needed, genotype/phenotype relationships, and the response to desmopressin (DDAVP) testing, according to our experience. Our findings are possible type 1, 69.6%; type 1, 13.5%; severe type 1, 0 .35%; type 3, 0.55%; type 2A, 9.5%; probable 2B, 0.6%; type 2M, 2.5%; and probable type 2N, 3.4%. The most frequent symptoms are ecchymoses-hematomas and epistaxis, and, in females >over 13 years also menorrhagia. In pregnant patients, assessment of laboratory parameters in months 7 and 8 is recommended to plan the need for prophylaxis at term. DDAVP merits to be con... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423215 Fri, 18 Nov 2011 05:00:00 +0100 5423215 http://www.medworm.com/index.php?rid=5423214&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281038 Semin Thromb Hemost 2011; 37: 522-527DOI: 10.1055/s-0031-1281038ABSTRACTIn Canada, care for individuals with inherited bleeding disorders, including von Willebrand disease, is provided by 26 tertiary care multidisciplinary Inherited Bleeding Disorders clinics geographically spread across the country. The Association of Hemophilia Clinic Directors of Canada, the Canadian Association of Nurses in Hemophilia Care, the Canadian Physiotherapists in Hemophilia Care, the Canadian Social Workers in Hemophilia Care, and the Canadian Hemophilia Society all collaborate to provide optimal management for patients with inherited bleeding disorders. The standards of care for these patients were explicitly laid out in a 2007 document published by the Canadian Hemophilia Standards Group (with representatio... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423214 Fri, 18 Nov 2011 05:00:00 +0100 5423214 http://www.medworm.com/index.php?rid=5423213&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281031 Semin Thromb Hemost 2011; 37: 470-479DOI: 10.1055/s-0031-1281031ABSTRACTThe online locus-specific database for von Willebrand disease (VWFdb) acts as a repository for sequence variant data and associated resources for those with an interest in the disorder. It currently holds details of 561 mutations and 217 polymorphisms in the von Willebrand factor (VWF) gene. Lists can be queried and displayed by VWF region or disease type. A total of 42% of the mutations are located in the large exon 28, the most heavily studied VWF region, and mutations have been reported in all but 4 of the 51 protein-coding exons. Polymorphisms are reported in the 5′ and 3′ untranslated regions and in 33 exons and 35 introns. Additional resources include references linked to sequence variation entries, descripto... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423213 Fri, 18 Nov 2011 05:00:00 +0100 5423213 http://www.medworm.com/index.php?rid=5423212&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291372 Semin Thromb Hemost 2011; 37: 621-630DOI: 10.1055/s-0031-1291372ABSTRACTImmune thrombocytopenia (ITP) has long been characterized as an autoimmune disease that exhibits antibody-mediated destruction of platelets. Many of the therapies have targeted reducing the antibody production and/or the platelet destruction process within the reticuloendothelial system, including steroids, immunoglobulin, anti-RhD immunoglobulin, splenectomy, and rituximab. Relatively new insights into the pathophysiology of this disorder have led to the introduction of new therapies, such as the use of thrombopoietic agents to enhance platelet production. This review outlines many of the most commonly used therapeutic agents for the treatment of severe thrombocytopenia associated with both newly diagnosed and chronic... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423212 Fri, 18 Nov 2011 05:00:00 +0100 5423212 http://www.medworm.com/index.php?rid=5423211&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291376 Semin Thromb Hemost 2011; 37: 664-672DOI: 10.1055/s-0031-1291376AbstractThrombocytopenia (TP) Cargeeg is a unique autosomal dominant disorder, affecting a seven-generation family, caused by cytochrome c (CYCS) mutation that dysregulates platelet formation. The CYCS mutation in this disorder is a glycine 41 replacement by serine, which yields a cytochrome c variant with enhanced apoptotic pathway activity in vitro. The deregulated apoptosis in this disorder affects megakaryocytes (MK) during platelet formation, leading to early and ectopic platelet release in the bone marrow (BM). Notably, the family has no other phenotypic indication of abnormal apoptosis, implying that cytochrome c activity is not a critical regulator of physiological apoptosis in most cells. The pathophysiology of this u... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423211 Fri, 18 Nov 2011 05:00:00 +0100 5423211 http://www.medworm.com/index.php?rid=5423210&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291371 Semin Thromb Hemost 2011; 37: 617-620DOI: 10.1055/s-0031-1291371© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423210 Fri, 18 Nov 2011 05:00:00 +0100 5423210 http://www.medworm.com/index.php?rid=5423209&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291375 Semin Thromb Hemost 2011; 37: 653-663DOI: 10.1055/s-0031-1291375ABSTRACTHeparin-induced thrombocytopenia (HIT) is a prothrombotic drug reaction caused by platelet-activating antibodies. HIT sera often activate platelets without needing heparin—such heparin-“independent” platelet activation can be associated with HIT beginning or worsening despite stopping heparin (“delayed-onset HIT”). We address important issues in HIT diagnosis and therapy, using a recent cohort of HIT patients to illustrate influences of heparin type; triggers for HIT investigation; serological features of heparin-independent platelet activation; and treatment. In our cohort of recent HIT cases (n = 13), low-molecular-weight heparin (dalteparin) was a common causative agent (n = 8, 62%); most patients ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423209 Fri, 18 Nov 2011 05:00:00 +0100 5423209 http://www.medworm.com/index.php?rid=5423208&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1291381 Semin Thromb Hemost 2011; 37: 707-712DOI: 10.1055/s-0031-1291381ABSTRACTThrombocytopenia-absent radius (TAR) syndrome is a relatively uncommon condition characterized by absent radii with the presence of thumbs and congenital or early-onset thrombocytopenia that tends to resolve in childhood. The precise cause of this condition is unknown, although recently a microdeletion of chromosome 1q21.1 has been found in all investigated individuals. However, this microdeletion alone is not sufficient to cause TAR syndrome, and another, uncharacterized genetic alteration is thought to be involved in the pathogenesis.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423208 Fri, 18 Nov 2011 05:00:00 +0100 5423208 http://www.medworm.com/index.php?rid=5423207&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281032 Semin Thromb Hemost 2011; 37: 480-487DOI: 10.1055/s-0031-1281032ABSTRACTIn the Netherlands, specialized care for patients with a bleeding disorder, including hemophilia, von Willebrand disease (VWD), and allied disorders, is concentrated in 13 Hemophilia Treatment Centers. The Dutch Hemophilia Treaters Society, the Dutch Hemophilia Nurses' Society, and the Netherlands Hemophilia Patients Society collaborate to optimize management of patients with a bleeding disorder. A recently updated consensus guideline of hemophilia and allied bleeding disorders provide guidance on the current optimal diagnostic strategy and treatment of VWD. Genetic testing is not routinely performed in the Netherlands. Desmopressin (DDAVP) is the choice of treatment in VWD patients responsive to DDAVP, as determined b... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423207 Fri, 18 Nov 2011 05:00:00 +0100 5423207 http://www.medworm.com/index.php?rid=5423206&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281050 Semin Thromb Hemost 2011; 37: 607-614DOI: 10.1055/s-0031-1281050ABSTRACTIn China, the care of patients with a bleeding disorder is not organized into designated centers with a national protocol. However, currently developed organizations of hematologists and hemophilia patients are beginning to ensure better diagnosis, treatment, and care of affected patients. The diagnosis of von Willebrand disease (VWD) in China is still at an early stage. Misdiagnosis of the disease is difficult to avoid, and there is a need to improve our current diagnostic strategy. Our data show that assessment of von Willebrand factor (VWF) antigen levels is pivotal in ranking VWD clinical severity for replacement therapy. Treatment choices for VWD in China are currently limited to replacement therapy and antifibrin... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423206 Fri, 18 Nov 2011 05:00:00 +0100 5423206 http://www.medworm.com/index.php?rid=5423205&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281030 Semin Thromb Hemost 2011; 37: 464-469DOI: 10.1055/s-0031-1281030ABSTRACTPlatelet-type von Willebrand disease (PT-VWD) is an autosomal dominant rare bleeding disorder characterized by hyperresponsive platelets. This inherent platelet function defect is due to a gain-of-function mutation within the GP1BA gene coding for the platelet surface glycoprotein Ib alpha protein, the receptor for the adhesive protein von Willebrand factor (VWF). The defect results in excessive and unnecessary platelet–VWF interaction with subsequent removal of the hemostatically efficient high molecular weight VWF as well as platelets from the circulation, leading to thrombocytopenia and bleeding diathesis. Patients with PT-VWD present with mild to moderate mucocutaneous bleeding, which becomes more pronounced duri... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423205 Fri, 18 Nov 2011 05:00:00 +0100 5423205 http://www.medworm.com/index.php?rid=5423204&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281033 Semin Thromb Hemost 2011; 37: 488-494DOI: 10.1055/s-0031-1281033ABSTRACTThe UK treatment strategy for von Willebrand disease (VWD) is based on consensus guidelines produced by the United Kingdom Haemophilia Centre Doctors' Organization (UKHCDO) relating to the diagnosis and management of VWD. Selection of therapeutic products suitable for treatment of this complex inherited bleeding disorder is based on the observed response. Desmopressin (DDAVP), an analog of vasopressin, is the recommended treatment in individuals who respond to this drug on trial infusion. DDAVP clearly has no effect in type 3 VWD but may have variable clinical effect in individuals with other subtypes or may be contraindicated in some cases. In patients where DDAVP treatment is unsuitable, replacement factor concentrat... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423204 Fri, 18 Nov 2011 05:00:00 +0100 5423204 http://www.medworm.com/index.php?rid=5423203&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281040 Semin Thromb Hemost 2011; 37: 535-541DOI: 10.1055/s-0031-1281040ABSTRACTWe have analyzed the databases for von Willebrand disease (VWD) from the hemophilia center for adult patients with bleeding disorders in South Australia. We define the prevalence of types of VWD to determine the proportion of who would be treated with factor (F) VIII/von Willebrand factor (VWF) concentrate to prevent or control hemorrhage. In severe or moderately severe patients, we use plasma-derived FVIII/VWF concentrate, and for mild to severe cases, we use desmopressin plus tranexamic acid. There are 103 patients with VWF ristocetin (RCo) ≤50 IU/dL: 38 (37%) severe (VWF:RCo <10 IU/dL), 28 (27%) moderate (VWF:RCo 10 to 29 IU/dL), and 37 (36%) mild (VWF:RCo 30 to 50 IU/dL). Hence in 66 (64%), FVIII/VWF concentra... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423203 Fri, 18 Nov 2011 05:00:00 +0100 5423203 http://www.medworm.com/index.php?rid=5423202&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1281028 Semin Thromb Hemost 2011; 37: 440-455DOI: 10.1055/s-0031-1281028ABSTRACTvon Willebrand disease (VWD) is the most common inherited bleeding disorder and arises from deficiencies and/or defects in the plasma protein von Willebrand factor (VWF). The current report overviews the diagnosis and management of VWD as reflected by differential processes applied within centers around the world. The prevalence of VWD, as well as the frequency of different VWD types, is also reported. VWD prevalence data varies according to methodology used, with epidemiological/population screening estimates approximating 1% of the population (or 10,000 cases per million population), several orders of magnitude higher than estimates from bleeding disorders registry data or regional/center analysis (which instead rang... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5423202 Fri, 18 Nov 2011 05:00:00 +0100 5423202 http://www.medworm.com/index.php?rid=5080893&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276590 Semin Thromb Hemost 2011; 37: 408-424DOI: 10.1055/s-0031-1276590ABSTRACTVitronectin is an abundant adhesive glycoprotein in blood plasma and is found associated with different extracellular matrix sites, the vessel wall, and tumor cells, particularly upon tissue remodeling, injury/repair, or under disease conditions. Plasma vitronectin is a structurally labile molecule that may be converted into a multimeric/multivalent form by interaction with various (hemostatic) factors or through surface binding. Several distinct binding domains along the vitronectin sequence for integrin-type cell adhesion receptors, for urokinase receptor or proteoglycans as well as for growth factors, endow vascular matrix- or fibrin-associated vitronectin with differentiated cell attachment and aggregatory properti... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080893 Fri, 29 Jul 2011 23:00:00 +0100 5080893 http://www.medworm.com/index.php?rid=5080892&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276589 Semin Thromb Hemost 2011; 37: 395-407DOI: 10.1055/s-0031-1276589ABSTRACTPlasminogen activator inhibitor (PAI)-2 expression is acutely upregulated in pregnancy, inflammation, infection, and other pathophysiological conditions. Circumstances that prevent PAI-2 upregulation are associated with chronic pathology. Altogether this strongly suggests that PAI-2 is one of the many proteins that maintain homeostasis during damage or stress. However, several functions ranging from a classical serpin to various intracellular roles have been ascribed to PAI-2 and, because none of these have been definitively proven in vivo, to this day its precise role or roles remains an enigma. This review readdresses the evidence supporting a role for PAI-2 in fibrinolysis and proteolysis within extracellular enviro... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080892 Fri, 29 Jul 2011 23:00:00 +0100 5080892 http://www.medworm.com/index.php?rid=5080891&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276588 Semin Thromb Hemost 2011; 37: 389-394DOI: 10.1055/s-0031-1276588ABSTRACTHistidine-rich glycoprotein (HRG) is one of the major plasma proteins and thought to function in blood coagulation, fibrinolysis, and innate immune systems. The amino acid sequence of HRG revealed a multidomain structure consisting of cystatin-like domains 1 and 2, a Pro-rich domain 1, a His-rich domain, a Pro-rich domain 2, and a C-terminal domain. Broad ligand-binding properties of HRG are involved in the multivalent functions of HRG. Among various functions of HRG, its interactions with heparin/heparan sulfate, fibrinogen, and plasminogen are thought to be intimately related to its roles in the coagulation and fibrinolytic systems. Recent studies on these topics are mainly reviewed in this article. The newly disclos... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080891 Fri, 29 Jul 2011 23:00:00 +0100 5080891 http://www.medworm.com/index.php?rid=5080890&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276587 Semin Thromb Hemost 2011; 37: 382-388DOI: 10.1055/s-0031-1276587ABSTRACTCoagulation factor XIII (F13) circulates in blood as a heterotetramer composed of an A subunit dimer and a B subunit dimer. It is activated by thrombin and crosslinks fibrin monomers. Congenital F13 deficiency demonstrates a lifelong bleeding tendency, abnormal wound healing, and recurrent miscarriages, and it first manifests as umbilical bleeding after birth. In contrast, secondary F13 deficiencies due to its overconsumption and/or hypobiosynthesis by disseminated intravascular coagulation, major surgery, liver diseases, and other disorders are rather common but rarely complicated with bleeding symptoms. Recently, consultations with physicians who have patients with hemorrhagic-acquired F13 deficiency with anti-F13 in... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080890 Fri, 29 Jul 2011 23:00:00 +0100 5080890 http://www.medworm.com/index.php?rid=5080889&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276586 Semin Thromb Hemost 2011; 37: 375-381DOI: 10.1055/s-0031-1276586ABSTRACTThe contact system is a protease cascade that is initiated by factor XII activation on cardiovascular cells. The system starts procoagulant and proinflammatory reactions, via the intrinsic pathway of coagulation and the kallikrein-kinin system, respectively. The biochemistry of the contact system in vitro is well understood. However, activators of the system in vivo and their contributions to disease states have remained enigmatic. Recent experimental and clinical data have identified misfolded proteins, collagens, and polyphosphates as the long-sought activators of the contact system in vivo. Here we present an overview about contact system activators and their contributions to health and pathology.[...]© Thieme Medi... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080889 Fri, 29 Jul 2011 23:00:00 +0100 5080889 http://www.medworm.com/index.php?rid=5080888&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276585 Semin Thromb Hemost 2011; 37: 362-374DOI: 10.1055/s-0031-1276585ABSTRACTC1-inhibitor (C1-inh) is a crucial regulator of the activation of plasmatic cascade systems involved in inflammation contributing to the homeostasis in the generation of proinflammatory mediators. The importance of C1-inh is illustrated by patients with hereditary angioedema where decreased levels of C1-inh lead to an uncontrolled generation of vasoactive peptides resulting in potential life-threatening subcutaneous edema. Recent publications, however, suggest that the anti-inflammatory properties of C1-inh do not strictly depend on its capacity to regulate the complement and contact phase system. This review summarizes the biochemical characteristics of C1-inh and its role in the regulation of plasmatic cascade system... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080888 Fri, 29 Jul 2011 23:00:00 +0100 5080888 http://www.medworm.com/index.php?rid=5080887&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276584 Semin Thromb Hemost 2011; 37: 355-361DOI: 10.1055/s-0031-1276584ABSTRACTBlood coagulation and complement pathways are two important natural defense systems. The high affinity interaction between the anticoagulant vitamin K–dependent protein S and the complement regulator C4b-binding protein (C4BP) is a direct physical link between the two systems. In human plasma, ~70% of total protein S circulates in complex with C4BP; the remaining is free. The anticoagulant activity of protein S is mainly expressed by the free form, although the protein S-C4BP complex has recently been shown to have some anticoagulant activity. The high affinity binding of protein S to C4BP provides C4BP with the ability to bind to negatively charged phospholipid membranes, which serves the purpose of localizing c... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080887 Fri, 29 Jul 2011 23:00:00 +0100 5080887 http://www.medworm.com/index.php?rid=5080886&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276583 Semin Thromb Hemost 2011; 37: 349-354DOI: 10.1055/s-0031-1276583ABSTRACTProtein C inhibitor (PCI) is a serine protease inhibitor and was originally identified as an inhibitor of activated protein C (APC). However, PCI is not specific for APC and also inhibits several proteases involved in coagulation, fibrinolysis, cancer, wound healing, and fertility. The biological function of PCI is unknown due to broad enzyme specificity, its wide tissue distribution, and the lack of a suitable animal model. This review highlights the specific roles of PCI in the areas of hemostasis and thrombosis and fertilization, and it also describes the latest information on the fascinating participation of the protein in intracellular processes, phospholipid binding, and killing of bacteria.[...]© Thieme Medical... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080886 Fri, 29 Jul 2011 23:00:00 +0100 5080886 http://www.medworm.com/index.php?rid=5080885&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276582 This article reviews the laboratory history, protein characteristics, structure–activity relationships, protease inhibition, physiological function, and medical relevance of HCII in hopes of regenerating interest in this sometimes forgotten serpin.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080885 Fri, 29 Jul 2011 23:00:00 +0100 5080885 http://www.medworm.com/index.php?rid=5080884&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1276581 Semin Thromb Hemost 2011; 37: 337-338DOI: 10.1055/s-0031-1276581© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=5080884 Fri, 29 Jul 2011 23:00:00 +0100 5080884 http://www.medworm.com/index.php?rid=4665720&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1274516 Semin Thromb Hemost 2011; 37: 328-336DOI: 10.1055/s-0031-1274516ABSTRACTHeparin-induced thrombocytopenia (HIT, type II) is an immune-mediated disorder due to antibodies formed against heparin-platelet factor 4 complexes, usually appearing at days 5 to 14 after initiation of heparin. It is important to recognize HIT because heparin prophylaxis or treatment paradoxically associates with new venous and/or arterial thrombosis. Early clinical suspicion and diagnosis together with proper pharmacotherapy and close laboratory monitoring are the cornerstones for successful management. This includes monitoring of Thrombocytopenia, its Timing to heparin administration, appearance of new Thrombosis or resistance to treatment, and differential diagnosis by exclusion of oTher causes (the 4T's). Specific... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665720 Wed, 30 Mar 2011 23:00:00 +0100 4665720 http://www.medworm.com/index.php?rid=4665719&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1274515 This article discusses the implications of these differences for the production of biosimilar LMWHs and provides recommendations to address the limitations in the pending U.S. Congress legislation, a well-intentioned undertaking but one that must preserve the health and welfare of citizens who require these critical care medications.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665719 Wed, 30 Mar 2011 23:00:00 +0100 4665719 http://www.medworm.com/index.php?rid=4665718&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273095 Semin Thromb Hemost 2011; 37: 315-321DOI: 10.1055/s-0031-1273095ABSTRACTParoxysmal nocturnal hemoglobinuria is a disorder associated with hemolysis, pancytopenia, and thrombosis due to the loss of the glycosylphosphatidylinositol (GPI) anchored complement regulatory proteins. The mechanism of thrombosis is multifactorial. Although intravascular hemolysis has been implicated as the etiology, the effect of complement on GPI anchor-deficient platelets, granulocytes, monocytes, and endothelial cells contributes significantly to the risk of thrombosis. Moreover, there appears to be an underlying inflammatory state that is linked to hemostatic activation that may induce thrombosis through a pathway independent of hemolysis.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of con... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665718 Wed, 30 Mar 2011 23:00:00 +0100 4665718 http://www.medworm.com/index.php?rid=4665717&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273094 This article offers a general overview of the relationship between FXIII and venous thromboembolism (VTE), to collect individual publications on this topic, present conclusions, and examine limitations of published studies. Special attention is given to the association of FXIII-A polymorphism with the risk of VTE, which has provoked considerable interest over the last decade.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665717 Wed, 30 Mar 2011 23:00:00 +0100 4665717 http://www.medworm.com/index.php?rid=4665716&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273093 This article reviews current knowledge regarding this topic and in light of recent data from a large prospective study. This is the first report from the prospective National Research Institute of Tuberculosis and Lung Disease DVT registry. Patients with ultrasound-confirmed symptomatic DVT were enrolled, and thrombus sidedness was investigated in each case. Computed tomography pulmonary angiography was used to diagnose coexisting pulmonary embolism (PE) in DVT patients with suggestive symptoms. Embolic burden score was calculated for those with PE. From the total of 100 patients, 45 had left-sided DVT, 41 had right-sided DVT, and 14 had bilateral DVT. Presenting symptoms and comorbidities were comparable, except for cancer, which was more common in those with right-sided involvement (eith... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665716 Wed, 30 Mar 2011 23:00:00 +0100 4665716 http://www.medworm.com/index.php?rid=4665715&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273092 Semin Thromb Hemost 2011; 37: 280-297DOI: 10.1055/s-0031-1273092ABSTRACTObstructive sleep apnea syndrome (OSAS) is a chronic disease characterized by recurrent episodes of partial or complete upper airway collapse and obstruction during sleep, associated with intermittent oxygen desaturation, sleep fragmentation, and symptoms of disruptive snoring and daytime sleepiness. Increasing focus is being placed on the relationship between OSAS and all-cause and cardiovascular disease-related mortality, but it still largely unclear whether this association is causative or simply speculative and epidemiological. Basically, reliable clinical evidence supports the hypothesis that OSAS might be associated with essential and resistant hypertension, as well as with an incremental risk of developing strok... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665715 Wed, 30 Mar 2011 23:00:00 +0100 4665715 http://www.medworm.com/index.php?rid=4665714&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273091 Semin Thromb Hemost 2011; 37: 275-279DOI: 10.1055/s-0031-1273091ABSTRACTFactor V Leiden is a common gain-of-function gene mutation resulting in a genetic predisposition to thromboembolic complications. Growing evidence in the literature indicates an interaction between factor V Leiden thrombophilia and acquired prothrombotic conditions such as contraceptive use or hormone replacement therapy, resulting in an increased risk of venous thromboembolism (VTE). Similarly, when combined with the prothrombotic influence of pregnancy, women who are carriers of factor V Leiden are faced with an increased risk of adverse pregnancy outcomes, including VTE, pre-eclampsia, fetal loss, placental abruption, and fetal growth restriction. The results of the most important meta-analyses on the relationship b... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665714 Wed, 30 Mar 2011 23:00:00 +0100 4665714 http://www.medworm.com/index.php?rid=4665713&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273090 Semin Thromb Hemost 2011; 37: 267-274DOI: 10.1055/s-0031-1273090ABSTRACTCurrent guidelines for venous thromboembolism (VTE) primary prophylaxis are based on randomized clinical trials that exclude subjects at a potentially high bleeding risk. Thus no specific recommendation/algorithm for pharmacological prophylaxis in patients with thrombocytopenia and/or platelet dysfunction is available. Because at least 25% of subjects admitted to medical departments exhibit these conditions, information on this subject is provided here to optimize their VTE prophylaxis. Low platelet number/function and clotting abnormalities are common in patients with liver cirrhosis. However, these patients have a high incidence of portal and idiopathic venous thromboses, implying that cirrhotic coagulopathy does not... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665713 Wed, 30 Mar 2011 23:00:00 +0100 4665713 http://www.medworm.com/index.php?rid=4665712&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273089 This article reviews current strategies of pharmacological thromboprophylaxis. The level of VTE risk following surgery depends on a variety of factors that the surgeon should take into account, including the type of surgery and the presence of additional risk factors, such as elderly age and cancer. In patients undergoing minor general surgery, early mobilization is sufficient as prophylaxis, whereas in those undergoing major general surgery, thromboprophylaxis with low molecular weight heparin (LMWH), low-dose unfractionated heparin, or the pentasaccharide fondaparinux is recommended. Patients undergoing major orthopedic surgery have a particularly high risk of VTE, and routine thromboprophylaxis with LMWH, fondaparinux, or a vitamin K antagonist (international normalized ratio target: 2.... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665712 Wed, 30 Mar 2011 23:00:00 +0100 4665712 http://www.medworm.com/index.php?rid=4665711&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273088 In conclusion, although the preventive benefits of mechanical prophylaxis on VTE might be circumscribed to select medical and surgical settings, there appears to exist no clinical reason to discourage adoption of these measures when indicated.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665711 Wed, 30 Mar 2011 23:00:00 +0100 4665711 http://www.medworm.com/index.php?rid=4665710&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273087 Semin Thromb Hemost 2011; 37: 226-236DOI: 10.1055/s-0031-1273087ABSTRACTSickle cell disease (SCD) is characterized by the presence of sickle hemoglobin, which has the unique property of polymerizing when deoxygenated. The pathophysiology of acute and chronic clinical manifestations of SCD have shown the central role of dense, dehydrated red cells in acute and chronic clinical manifestations of this pathology. Recent studies have indicated that SCD is characterized by a hypercoagulable state that contributes to the vaso-occlusive events in microcirculation, leading to acute and chronic sickle cell-related organ damage. This review discusses, in the context of SCD, (1) abnormalities in the coagulation system, (2) perturbation of platelet activation and aggregation, (3) vascular endothelial d... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665710 Wed, 30 Mar 2011 23:00:00 +0100 4665710 http://www.medworm.com/index.php?rid=4665709&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273086 Semin Thromb Hemost 2011; 37: 220-225DOI: 10.1055/s-0031-1273086ABSTRACTVenous and arterial thromboembolism are both serious extraintestinal manifestations of inflammatory bowel disease (IBD). Acquired risk factors seem to play a more prominent role than congenital in promoting thrombotic events. Prevention of thromboembolism is thus mainly aimed at minimizing the acquired/reversible risk factors (e.g., inflammation, immobility, hospitalization, steroid therapy, central intravenous catheters, smoking, oral contraceptives, and deficiency of B vitamins and folate). The diagnosis of venous and arterial thromboembolism is extremely challenging and requires a high degree of vigilance. Deep vein thrombosis and pulmonary embolism may be clinically silent or manifest with only few specific symptom... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665709 Wed, 30 Mar 2011 23:00:00 +0100 4665709 http://www.medworm.com/index.php?rid=4665708&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273085 Semin Thromb Hemost 2011; 37: 209-219DOI: 10.1055/s-0031-1273085ABSTRACTVenous thromboembolism (VTE) is a disease with a high prevalence in elderly people, affecting > 5% of the population > 65 years of age. Cancer patients have a 4.3-fold higher incidence of thrombotic complications, due to multiple risk factors that are not always related to the disease. Among hematologic malignancies, multiple myeloma (MM) confers a high risk of developing such complications, with a VTE rate of nearly 10%. Multiple factors are involved in MM-related VTE, such as increased blood viscosity, high levels of immunoglobulin, procoagulant activity of monoclonal protein, and inflammatory cytokines. Since the introduction of the immunomodulatory derivatives (IMiDs) thalidomide and lenalidomide in the thera... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665708 Wed, 30 Mar 2011 23:00:00 +0100 4665708 http://www.medworm.com/index.php?rid=4665707&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273084 This article provides an up-to-date review of the role of nutrient and nonnutrient supplements on platelet aggregation and risk of thrombosis.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665707 Wed, 30 Mar 2011 23:00:00 +0100 4665707 http://www.medworm.com/index.php?rid=4665706&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273083 This article reviews the role of gender as a risk factor for first and recurrent VTE.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665706 Wed, 30 Mar 2011 23:00:00 +0100 4665706 http://www.medworm.com/index.php?rid=4665705&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273082 Semin Thromb Hemost 2011; 37: 181-192DOI: 10.1055/s-0031-1273082ABSTRACTPeriodontitis is a chronic inflammatory response to bacterial plaque in which the anchoring bone and soft tissues supporting teeth are destroyed, resulting in tooth mobility and loss. Dental caries involves the spread of infection from the dentine to the vascular dental pulp and periapical bony tissues, before involvement of adjacent soft tissues and spreading sepsis. Several case-controlled, cross-sectional, and cohort studies report correlation between periodontitis and increased cardiovascular, cerebrovascular, and peripheral artery disease, as determined by clinical disease, angiography, ultrasonography, and reduced flow-mediated dilation. Some studies report a similar relationship of atherosclerosis with periapica... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665705 Wed, 30 Mar 2011 23:00:00 +0100 4665705 http://www.medworm.com/index.php?rid=4665704&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0031-1273081 Semin Thromb Hemost 2011; 37: 175-180DOI: 10.1055/s-0031-1273081© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4665704 Wed, 30 Mar 2011 23:00:00 +0100 4665704 http://www.medworm.com/index.php?rid=4541761&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270345 Semin Thromb Hemost 2011; 37: 165-174DOI: 10.1055/s-0030-1270345ABSTRACTTwo dangerous obstetric complications, amniotic fluid embolism and preterm prelabor rupture of membranes (PROM), can be caused by amniotic fluid (AF) constituents. Disseminated intravascular coagulation (DIC) is related to the former complication, whereas local thrombin/plasmin-dependent collagenolysis in the decidua and fetal membranes is associated with the latter. In AF, most proteins of the coagulation and fibrinolysis system, known as plasma constituents, have been identified based on the activity and/or presence of antigen. The AF levels of most of these proteins are low (< 2 to 5% of the respective maternal plasma levels). However, there are some exceptions: tissue factor (TF), urokinase plasminogen activator... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541761 Wed, 02 Mar 2011 00:00:00 +0100 4541761 http://www.medworm.com/index.php?rid=4541760&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270344 Semin Thromb Hemost 2011; 37: 158-164DOI: 10.1055/s-0030-1270344ABSTRACTInvasion of the decidua by extravillous trophoblasts (EVTs) is accompanied by thrombin generation from decidual cell (DC)-expressed tissue factor (TF). This TF protects against hemorrhage as EVTs breach capillaries and subsequently invade and remodel spiral arteries and arterioles. Pre-eclampsia (P-EC) is the world's leading cause of fetal and maternal morbidity and mortality. It is associated with decidual hemorrhage and maternal thrombophilias, which form excess thrombin from DCs, and with maternal infections and other inflammatory conditions that are associated with excess expression of the proinflammatory cytokines interleukin (IL)-1 β and tumor necrosis factor (TNF) α. In human first-trimester leukocyte-... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541760 Wed, 02 Mar 2011 00:00:00 +0100 4541760 http://www.medworm.com/index.php?rid=4541759&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270343 Semin Thromb Hemost 2011; 37: 153-157DOI: 10.1055/s-0030-1270343ABSTRACTA plethora of evidence exists to show that endothelial perturbations underlie many of the clinical features of pre-eclampsia (P-EC), and consequently the markers signifying endothelial disturbance exhibit a rise in plasma. Among others, plasma thrombomodulin (TM) level rises significantly. TM is a transmembrane glycoprotein expressed abundantly on the endothelium of the microvasculature. It neutralizes the thrombotic potential of thrombin, mediating this anticoagulant effect through activation of protein C. Endothelial injury results in a localized loss of TM with a focal impairment of protein C activation and resultant thrombotic tendency. Mainly expressed on the endothelial cells, a small amount of TM is found in pla... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541759 Wed, 02 Mar 2011 00:00:00 +0100 4541759 http://www.medworm.com/index.php?rid=4541758&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270342 Semin Thromb Hemost 2011; 37: 146-152DOI: 10.1055/s-0030-1270342ABSTRACTPre-eclampsia (P-EC), a heterogenic multisystem disorder characterized by hypertension and proteinuria, usually develops in the second half of pregnancy. The incidence is 2 to 5%, and P-EC is therefore a major cause of maternal and perinatal morbidity and mortality. Although the exact etiology is unknown, placental factors released into the maternal circulation lead to systemic maternal inflammation and endothelial dysfunction. Growing evidence indicates that placenta-derived microparticles, best known as syncytiotrophoblast microparticles (STBM), are important among these factors. This review provides an overview of the presence and function(s) of STBM and other cell-derived microparticles and exosomes.[...]© Thieme ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541758 Wed, 02 Mar 2011 00:00:00 +0100 4541758 http://www.medworm.com/index.php?rid=4541757&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270341 Semin Thromb Hemost 2011; 37: 141-145DOI: 10.1055/s-0030-1270341ABSTRACTAntiphospholipid syndrome (APS) is defined as an autoimmune disorder characterized by recurrent thrombosis or obstetrical morbidity. These features are linked to the presence in blood of autoantibodies against negatively charged phospholipids or phospholipid-binding proteins. Obstetric morbidity includes recurrent abortion (early and late) and severe pre-eclampsia (P-EC)/hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome, and/or severe placental insufficiency. Criteria that define the major clinical and laboratory events were published in revised forms in the Sydney recommendations in 2006. We analyzed the blood of patients with severe P-EC according to the subgroups based on the 2006 revised criteria de... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541757 Wed, 02 Mar 2011 00:00:00 +0100 4541757 http://www.medworm.com/index.php?rid=4541756&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270340 Semin Thromb Hemost 2011; 37: 137-140DOI: 10.1055/s-0030-1270340ABSTRACTPre-eclampsia (P-EC) remains a significant problem in modern obstetrics occurring in 2 to 4% of women. The disease is still responsible for 60,000 maternal deaths worldwide annually. An increased understanding of the underlying pathophysiology has resulted in a more scientific approach to prophylaxis and prevention, yet the underlying disease mechanisms are not fully understood. The role of combining good prediction with prevention has yet to be established but has the potential to target health resources far more efficiently. Good prediction may also impact on tailoring antenatal care appropriately, with prophylactic measures for high-risk women becoming the highly preferred management option. Antiplatelet agents redu... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541756 Wed, 02 Mar 2011 00:00:00 +0100 4541756 http://www.medworm.com/index.php?rid=4541755&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270339 Semin Thromb Hemost 2011; 37: 131-136DOI: 10.1055/s-0030-1270339ABSTRACTPronounced hemostatic changes occur during pregnancy, and the balance shifts markedly in favor of hypercoagulability. Although primarily a result of a marked rise in the levels of several procoagulants and a fall in some natural anticoagulants, platelet activation also contributes to this prothrombotic tendency. Several studies have confirmed the accentuation of platelet activation in pre-eclampsia (P-EC), which remains an important obstetric complication affecting ~2 to 4% of pregnancies. Although there is still a long way to go, significant inroads have been made in the understanding of this enigmatic condition. Whereas the pathogenesis of P-EC is protean and involves a complex interplay of placental and maternal tis... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541755 Wed, 02 Mar 2011 00:00:00 +0100 4541755 http://www.medworm.com/index.php?rid=4541754&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270338 Semin Thromb Hemost 2011; 37: 125-130DOI: 10.1055/s-0030-1270338ABSTRACTPre-eclampsia (P-EC) is a major contributor to perinatal and maternal morbidity and mortality worldwide. Its etiology and pathogenesis remains poorly understood, and differential diagnosis is problematic. During a normal pregnancy, coagulation activation is essential for physiological placental hemostasis, but women with P-EC tend to be more hypercoagulable than normal pregnant women. A common proposed mechanism for P-EC is utero-placental thrombosis. Indeed, multiple placental microthrombi are frequently observed in women with P-EC, and these may compromise placental perfusion and fetal development. This suggests that predisposing factors to thrombosis could contribute to the development of P-EC. Thus studying circula... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541754 Wed, 02 Mar 2011 00:00:00 +0100 4541754 http://www.medworm.com/index.php?rid=4541753&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270337 Semin Thromb Hemost 2011; 37: 118-124DOI: 10.1055/s-0030-1270337ABSTRACTPre-eclampsia (P-EC) is a major cause of maternal and fetal morbidity and mortality. Inherited thrombophilia has been suggested to increase the risk of P-EC or may accelerate the pathophysiological process. In the past 20 years, numerous studies and meta-analyses have yielded contradictory results regarding the association between inherited thrombophilia and P-EC/hemolysis, elevated liver enzymes, low platelets syndrome. Although studies published until 2002 reported on an increased prevalence of inherited thrombophilia in pre-eclamptic women, most subsequent studies did not. Inconsistency of data may be due to numerous confounders such as ethnicity, different definitions of P-EC, severity of illness, and methods of te... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541753 Wed, 02 Mar 2011 00:00:00 +0100 4541753 http://www.medworm.com/index.php?rid=4541752&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270336 Semin Thromb Hemost 2011; 37: 111-117DOI: 10.1055/s-0030-1270336ABSTRACTPre-eclampsia (P-EC) is a multisystem disorder exclusive to pregnancy. It complicates ~2 to 8% of all pregnancies and remains a major cause of maternal mortality. P-EC is characterized by a profound hypercoagulable state. The delicate hemostatic balance that must be maintained in the uteroplacental circulation during pregnancy makes this system vulnerable to perturbation. An abnormal hemostatic pattern occurs within the uteroplacental circulation in P-EC compared with normal pregnancy. Much recent research has focused on the epidemiological link between inherited thrombophilia and P-EC. The data suggest a weak statistical association, indicating an improbable primary role in the pathogenesis. Without clear evidence, lo... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541752 Wed, 02 Mar 2011 00:00:00 +0100 4541752 http://www.medworm.com/index.php?rid=4541751&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270335 Semin Thromb Hemost 2011; 37: 106-110DOI: 10.1055/s-0030-1270335ABSTRACTPre-eclampsia (P-EC) poses an increased risk for fetal and maternal morbidity and sometimes even mortality. Several risk factors have been identified, among which an important extensively studied group is formed by the early recognizable genetic factors resulting in thrombophilia. The discussion is ongoing whether the associations found for thrombophilia are true and clinically relevant. In this review, we address the association between P-EC and thrombophilia, its concerns, and the necessity of screening after a pregnancy complicated by hypertension.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541751 Wed, 02 Mar 2011 00:00:00 +0100 4541751 http://www.medworm.com/index.php?rid=4541750&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270334 Semin Thromb Hemost 2011; 37: 097-105DOI: 10.1055/s-0030-1270334ABSTRACTPre-eclampsia (P-EC) is a multisystem disorder of pregnancy, characterized by new-onset hypertension and proteinuria. Deregulation of the coagulation cascade and hypofibrinolysis appear to play a central role in the development of this disease. After a brief review of the genetic basis of P-EC and the role of genes encoding proteins involved in coagulation, we focus on polymorphisms of the plasminogen activator inhibitor (PAI-1) gene. The most relevant association studies between PAI-1 gene polymorphisms and P-EC are reviewed. Results indicate that the 4G/4G genotype of the -675 4G/5G polymorphism represents a weak risk factor for P-EC.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |�... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541750 Wed, 02 Mar 2011 00:00:00 +0100 4541750 http://www.medworm.com/index.php?rid=4541749&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270333 Semin Thromb Hemost 2011; 37: 095-096DOI: 10.1055/s-0030-1270333© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4541749 Wed, 02 Mar 2011 00:00:00 +0100 4541749 http://www.medworm.com/index.php?rid=4456362&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1271292 Semin Thromb Hemost 2011; 37: 095-095DOI: 10.1055/s-0030-1271292© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  FREE: Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4456362 Wed, 09 Feb 2011 00:00:00 +0100 4456362 http://www.medworm.com/index.php?rid=4456361&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270073 Semin Thromb Hemost 2011; 37: 066-076DOI: 10.1055/s-0030-1270073ABSTRACTThe liver plays a central role in the maintenance of a normal hemostatic balance by synthesizing several factors belonging to the pathways of coagulation, anticoagulation, and fibrinolysis. It is thereby unsurprising that patients with chronic liver disease (CLD) or cirrhosis may experience a kaleidoscope of hemostatic disorders. A bleeding tendency represents the most frequent and clinically severe hemostatic complication of CLD or cirrhosis. Perhaps less anticipated, growing evidence now suggests that a procoagulant state may be also associated with CLD, so that patients with CLD or cirrhosis, irrespective of its etiology, rather than be “naturally anticoagulated” might also experience a large spectrum of... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4456361 Wed, 09 Feb 2011 00:00:00 +0100 4456361 http://www.medworm.com/index.php?rid=4456360&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270071 In this report, we present a review of the endocrinologic manifestations associated with APS by evaluating the medical literature from 1968 to 2009 using MEDLINE and these keywords: APS, antiphospholipid syndrome, antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant, anti β-2 glycoprotein I, pituitary, adrenal, thyroid, parathyroid, ovary, testes, diabetes mellitus, and diabetes insipidus. Adrenal insufficiency was found to be the most common endocrine manifestation associated with APS. Autoimmune thyroid disease was associated with increased titers of antiphospholipid antibodies (aPL) without any APS clinical manifestations. In addition, hypopituitarism and Sheehan syndrome are increasingly being reported in association with aPL. Data regarding the prevalence ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4456360 Wed, 09 Feb 2011 00:00:00 +0100 4456360 http://www.medworm.com/index.php?rid=4456359&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270069 Semin Thromb Hemost 2011; 37: 035-040DOI: 10.1055/s-0030-1270069ABSTRACTAcquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). Unlike VWD, AVWS usually occurs in individuals with no personal or family history of bleeding. The prevalence of AVWS in the general population is unknown because data from large prospective studies of this syndrome are not available. Although AVWS is more frequently associated with lympho-myeloproliferative and cardiovascular disorders, it can also occur in solid tumors and in immunological and other miscellaneous conditions. Among these miscellaneous conditions, hypothyroidism has been associated with AVWS type 1 with a frequency of ∼2 to 5%. In the 47 cases ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4456359 Wed, 09 Feb 2011 00:00:00 +0100 4456359 http://www.medworm.com/index.php?rid=4456358&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270066 Semin Thromb Hemost 2011; 37: 011-016DOI: 10.1055/s-0030-1270066ABSTRACTThis review focuses attention on some practical aspects of the relationship between thyroid disorders and hypocoagulability, as related to an impairment of hemostasis and fibrinolysis. An understanding of this topic in daily clinical practice is important given that the interaction between hemostatic abnormalities and thyroid disorders is still poorly recognized by the medical community. Even if the bleeding tendency is in general mild and may be reversed by restoration of an euthyroid state, severe hemorrhagic events may complicate the course of both hyper- and hypothyroidism, as precipitated by such conditions as thrombocytopenia, acquired von Willebrand syndrome, and acquired hemophilia. The pathogenesis of the hemo... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4456358 Wed, 09 Feb 2011 00:00:00 +0100 4456358 http://www.medworm.com/index.php?rid=4373810&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270075 Semin Thromb Hemost 2011; 37: 087-094DOI: 10.1055/s-0030-1270075ABSTRACTMen have a higher incidence of cardiovascular disease (CVD) than women, and adverse thrombotic events increase with age. Recent experimental cross-sectional, and case-control studies have shown that testosterone may affect the hemostatic/fibrinolytic system in men in several ways. It has been hypothesized that physiological doses of testosterone would have a beneficial effect on tissue factor-induced thrombin generation and the development of CVD. The search for eternal youth has created a market for testosterone treatment in aging men during the last few years. However, whether testosterone supplementation could be useful in the treatment of testosterone-deficient elderly men is still controversial. The present review... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373810 Wed, 19 Jan 2011 00:00:00 +0100 4373810 http://www.medworm.com/index.php?rid=4373809&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270074 This articles reviews recent evidence for these changes. During the menstrual cycle, changes are observed in levels of von Willebrand factor (VWF), fibrinogen, and activated factor VII. No such effect has been demonstrated in protein S or protein C levels or activated protein C resistance. Pregnancy is a procoagulant state with progressive increase in levels of factors VII, VIII, X, and XII, fibrinogen, and VWF, as well as increased resistance to activated protein C. Hormonal contraceptives and HRT are widely used and have undergone many changes over the years. Recent modifications to the preparations used in combined oral contraceptives (COC) aimed at improving side-effect profiles have also been shown to increase the risk of VTE for third- and fourth-generation COC compared with second-g... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373809 Wed, 19 Jan 2011 00:00:00 +0100 4373809 http://www.medworm.com/index.php?rid=4373808&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270072 Semin Thromb Hemost 2011; 37: 058-065DOI: 10.1055/s-0030-1270072ABSTRACTThis review summarizes current knowledge of the adverse effects of type 1 diabetes mellitus on coagulation and fibrinolysis. Although further larger studies are needed to provide more definitive information, patients with type 1 diabetes exhibit a proinflammatory/procoagulant condition deriving from increased platelet adhesiveness, activation of the coagulation system, and decreased plasma fibrinolytic potential. This review also focuses on recent data from large prospective studies suggesting a strong association between procoagulant imbalance and development of chronic vascular complications in people with type 1 diabetes. It is likely that a greater appreciation of the intimate interactions between endothelial integ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373808 Wed, 19 Jan 2011 00:00:00 +0100 4373808 http://www.medworm.com/index.php?rid=4373807&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270070 Semin Thromb Hemost 2011; 37: 041-048DOI: 10.1055/s-0030-1270070ABSTRACTEndocrine disorders can influence the hemostatic balance. Abnormal coagulation test results have been observed in patients with abnormal hormone levels. The present review updates the available evidence on the influence of pituitary, adrenal, and parathyroid hormones on the coagulation and the fibrinolytic system, and their possible clinical implications. The literature supports a possible relevant clinical effect of the imbalance between coagulation and fibrinolysis on thrombotic events in endogenous Cushing's syndrome. An effect on markers of coagulation and fibrinolysis has been shown for hyperprolactinemia, growth hormone excess or deficiency, exogenous hypercortisolism, pheochromocytoma, primary hyperaldosteronism... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373807 Wed, 19 Jan 2011 00:00:00 +0100 4373807 http://www.medworm.com/index.php?rid=4373806&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270068 Semin Thromb Hemost 2011; 37: 027-034DOI: 10.1055/s-0030-1270068ABSTRACTSubclinical hypothyroidism (SH) is a frequent condition affecting millions of people around the world. Defined by increased thyrotropin-stimulating hormone (TSH) and accompanied by normal thyroid hormone levels, SH reflects a mild tissue hypothyroidism that has been associated with metabolic derangements and—although this issue is still contentious—possibly with increased cardiovascular risk. Depending on the degree of TSH elevation, SH has accordingly been associated with hyperlipidemia, arterial hypertension, and cardiovascular disease (CVD), as well as, increasingly, newly emerging CVD risk factors such as serum C-reactive protein and retinol binding protein 4 levels. There have also been reports of abno... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373806 Wed, 19 Jan 2011 00:00:00 +0100 4373806 http://www.medworm.com/index.php?rid=4373805&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270067 In conclusion, adequate further prospective clinical studies of high quality including a larges series of patients are needed to explain the degree and type of coagulation/fibrinolytic abnormalities in patients with thyroid diseases.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373805 Wed, 19 Jan 2011 00:00:00 +0100 4373805 http://www.medworm.com/index.php?rid=4373804&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270065 Semin Thromb Hemost 2011; 37: 007-010DOI: 10.1055/s-0030-1270065ABSTRACTThe hemostatic balance is a complex system where the delicate equilibrium is regulated by several factors including hormones. A variety of endocrine disorders have been reported to be associated with coagulation abnormalities, ranging from mild laboratory changes to clinically relevant thrombotic or bleeding manifestations. In this review, we summarize the current knowledge on the main abnormalities of the coagulation and fibrinolytic systems associated with thyroid dysfunctions. Overall, although mostly based on uncontrolled studies, data in the literature suggest that patients with hyperthyroidism or subclinical hypothyroidism have a hypercoagulative state, whereas patients with overt hypothyroidism have a bleeding t... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373804 Wed, 19 Jan 2011 00:00:00 +0100 4373804 http://www.medworm.com/index.php?rid=4373803&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270064 Semin Thromb Hemost 2011; 37: 003-006DOI: 10.1055/s-0030-1270064© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373803 Wed, 19 Jan 2011 00:00:00 +0100 4373803 http://www.medworm.com/index.php?rid=4373802&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1270063 Semin Thromb Hemost 2011; 37: 001-002DOI: 10.1055/s-0030-1270063© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4373802 Wed, 19 Jan 2011 00:00:00 +0100 4373802 http://www.medworm.com/index.php?rid=4156438&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267044 Semin Thromb Hemost 2010; 36: 907-916DOI: 10.1055/s-0030-1267044ABSTRACTMicroparticles (MP) are shed membrane vesicles released by various cell types following apoptosis or activation. MP circulate in human plasma and also accumulate in atherosclerotic lesions. A growing body of evidence has highlighted their involvement in inflammation, angiogenesis, coagulation, and the regulation of vascular tone. MP may therefore contribute to the initiation and development of atherosclerosis and its complications. Plasma MP originate from platelets, leukocytes, erythrocytes, and endothelial cells, and their levels increase in patients with cardiovascular diseases; specific cardiovascular medications also affect plasma MP levels. Most recent data suggest a potential prognostic role of circulating MP fo... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4156438 Wed, 10 Nov 2010 00:00:00 +0100 4156438 http://www.medworm.com/index.php?rid=4136057&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267777 Semin Thromb Hemost 2010; 36: 930-932DOI: 10.1055/s-0030-1267777© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  FREE: Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136057 Wed, 03 Nov 2010 00:00:00 +0100 4136057 http://www.medworm.com/index.php?rid=4136056&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267046 Semin Thromb Hemost 2010; 36: 925-929DOI: 10.1055/s-0030-1267046ABSTRACTThis review presents an overview of the functions of microparticles and exosomes in gynecologic neoplasias. Growing evidence suggests that vesicles released from cancer cells in gynecologic malignancies contribute to the hypercoagulable state of these patients and contribute to tumor progression by suppressing the immune system, facilitating extracellular matrix degradation and removal of cytostatics from the tumor cell. Exosomes from ovarian carcinoma cells were shown to be present in peripheral blood and to augment tumor growth, suggesting that these vesicles directly support growth of tumor cells.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136056 Wed, 03 Nov 2010 00:00:00 +0100 4136056 http://www.medworm.com/index.php?rid=4136055&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267045 Semin Thromb Hemost 2010; 36: 917-924DOI: 10.1055/s-0030-1267045ABSTRACTSerious bleeding and thrombotic complications are frequent in acute promyelocytic leukemia (APL) and are major causes of morbidity and mortality. Microparticles (MP) have been used to study the risk and pathogenesis of thrombosis in many malignant disorders. To date, from published articles, this approach had not been applied to APL. In this article, the hemostatic dysfunction in this disorder is briefly reviewed. A study design to address this problem using MP is described. MP bearing tissue factor, profibrinolytic factors (tissue plasminogen activator and annexin A2), and the antifibrinolytic factor plasminogen activator inhibitor type 1 were measured using flow cytometry. The cellular origin of the MP was identified... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136055 Wed, 03 Nov 2010 00:00:00 +0100 4136055 http://www.medworm.com/index.php?rid=4136054&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267043 Semin Thromb Hemost 2010; 36: 888-906DOI: 10.1055/s-0030-1267043ABSTRACTMicroparticles (MP) are vesicular structures released from cells upon activation, malignant transformation, stress, or death. MP may be derived from the plasma membrane (shed microvesicles), produced by endosomal pathway (exosomes), or arise from membrane blebs of apoptotic cells. The terms microparticles or microvesicles (MV) are often used as general and interchangeable descriptors of all cellular vesicles, but a more rigorous terminology is still to be established. The cargo of MP/MV consists of proteins, lipids, and nucleic acids (DNA, mRNA, microRNA), all of which may be transferred horizontally between cells. In cancer, oncogenic pathways drive production of MP/MV, and oncoproteins may be incorporated into the ca... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136054 Wed, 03 Nov 2010 00:00:00 +0100 4136054 http://www.medworm.com/index.php?rid=4136053&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267042 Semin Thromb Hemost 2010; 36: 881-887DOI: 10.1055/s-0030-1267042ABSTRACTPlatelet microparticles are the most abundant cell-derived microparticle subtype in the circulation. Yet the mechanism by which platelet microparticles are formed is poorly defined. This review highlights the concept of the generation of microparticles from platelets and their precursor cells, megakaryocytes. Special emphasis is placed on the mechanisms of microparticle formation and novel functions for microparticles in normal physiology and disease states.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136053 Wed, 03 Nov 2010 00:00:00 +0100 4136053 http://www.medworm.com/index.php?rid=4136052&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267041 Semin Thromb Hemost 2010; 36: 876-880DOI: 10.1055/s-0030-1267041ABSTRACTIt is emerging that cell-derived microparticles (MP) have multiple functional activities in areas including hemostasis, thrombosis, inflammation, and as messengers in the transport of bioactive lipids, cytokines, complement, and immune signaling. Some of these activities may be performed by distinct phenotypic subsets of MP, even if derived from the same cell type. The focus of this article concerns the size classes of MP, covering methods of MP size measurement, differences in composition between size classes, and relation of size to functional (procoagulant) activity. Some of the issues considered remain to be resolved, such as whether the MP known as exosomes are truly a distinct class of MP, as well as the detailed... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136052 Wed, 03 Nov 2010 00:00:00 +0100 4136052 http://www.medworm.com/index.php?rid=4136051&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267040 Semin Thromb Hemost 2010; 36: 865-875DOI: 10.1055/s-0030-1267040ABSTRACTTissue factor (TF) is a transmembrane protein that initiates coagulation following contact with factor VII/VIIa. Recent experimental evidence, in particular from animal models, suggests an important role for circulating TF in thrombosis. This has led to a growing interest in the measurement of TF in whole blood and in cell-free plasma, where functionally active TF is carried on cell-derived microparticles. In this review, we address the range of assays for measuring circulating TF antigen and activity that have been published. We comment on some of the crucial preanalytical and analytical variables that influence the results and their interpretation.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of ... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136051 Wed, 03 Nov 2010 00:00:00 +0100 4136051 http://www.medworm.com/index.php?rid=4136050&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267039 Semin Thromb Hemost 2010; 36: 857-864DOI: 10.1055/s-0030-1267039ABSTRACTEvidence is emerging that tissue-factor–bearing microparticles and other microparticles arise from regions of the parent cell's plasma membrane that are rich in lipid rafts. In this brief review, we summarize the evidence for the raft origins of microparticles and the implications of these origins for the biological and medical consequences of microparticle production and for therapeutic strategies to diminish their production and potential to do harm.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis) Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136050 Wed, 03 Nov 2010 00:00:00 +0100 4136050 http://www.medworm.com/index.php?rid=4136049&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267038 Semin Thromb Hemost 2010; 36: 845-856DOI: 10.1055/s-0030-1267038ABSTRACTAll cell types shed ectosomes and exosomes, collectively known as microparticles (MP; 0.1 to 1.5 μm in diameter), when activated or stressed; normal human plasma contains ~2 μg MP protein/mL. The cellular composition of plasma MP is altered in many diseases, including acute coronary syndrome, diabetes mellitus, sepsis, and sickle cell disease. We measured the plasma MP protein composition of 42 patients (median age 69.5 years, most with cardiovascular disease) by label-free liquid chromatography coupled to tandem mass spectrometry. Among 458 proteins detected with high confidence (identified by at least two unique peptides with SEQUEST XCor (Thermo Electron Corp., San Jose, CA) ≥2.0, 2.2, and 3.3 for ch... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136049 Wed, 03 Nov 2010 00:00:00 +0100 4136049 http://www.medworm.com/index.php?rid=4136048&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267037 Semin Thromb Hemost 2010; 36: 833-844DOI: 10.1055/s-0030-1267037ABSTRACTMembrane remodeling, phosphatidylserine (PS) exposure, and subsequent microparticle (MP) shedding regulation is a critical step in maintaining vascular homeostasis. Shed MP, more particularly those of platelet origin, could be viewed as a way to increase the catalytic procoagulant surface relying on the essential presence of PS for optimal hemostatic response. Whether “flip-flop” is mandatory for the release of MP is suggested from the phenotype of Scott's syndrome, a rare bleeding disorder in which both PS exposure and MP shedding are deficient. PS exposure results from a specific cytoskeleton degradation pathway involving caspases, tuned by mitochondria permeability changes, and requiring a sustained incr... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136048 Wed, 03 Nov 2010 00:00:00 +0100 4136048 http://www.medworm.com/index.php?rid=4136047&cid=s_36599_19_f&fid=36599&url=http%3A%2F%2Fdx.doi.org%2F10.1055%2Fs-0030-1267036 Semin Thromb Hemost 2010; 36: 824-832DOI: 10.1055/s-0030-1267036ABSTRACTNew light scattering methods offer many advantages to particle size distribution characterization. In addition to ease of operation, speed, and accuracy, the particle size, particle surface characteristics, interaction of the surface with specific ligands, and hydrodynamic volume of the particle are easily obtained. Extensions of these methods also permit the assessment of surface reactions in real time and without reporter group conjugation to the reactant. These methods offer the ability to examine binding constants and kinetics of binding without chemical modification and offer true advantages in product development and clinical diagnostics and therapeutic monitoring.[...]© Thieme Medical PublishersArticle in Thiem... Seminars in Thrombosis and Hemostasis journals http://www.medworm.com/rss/comments.php?id=4136047 Wed, 03 Nov 2010 00:00:00 +0100 4136047