Hermansky-Pudlak syndrome. Overview of clinical and molecular features and case report of a new HPS-1 variant.

abstract：:Low molecular weight heparins and fondaparinux have been the cornerstones for prevention of venous thromboembolism (VTE) in acutely ill medical patients for almost two decades. Guidelines recommend the use of these parenteral anticoagulants for 6 to 14 days but advise against extended-duration thromboprophylaxis after...

journal_title：Hamostaseologie

authors： Haas S

更新日期：2019-02-01 00:00:00

abstract：:In Germany one-third of the women in fertile age use combined oral contraceptives (COCs), which consist mostly of ethinylestradiol (EE) and a synthetic progestin. Older COCs with norethisterone or levonorgestrel have a lower risk for venous thromboembolism (VTE) than newer COCs with desogestrel, drospirenone, or gesto...

journal_title：Hamostaseologie

authors： Rott H

更新日期：2019-02-01 00:00:00

abstract：:Recurrent venous thromboembolism is associated with increased mortality in 5-9% of the patients. On the other hand prolonged anticoagulation can increase the bleeding risk which can also be responsible for an increased mortality. Therefore, it is necessary to validate the recurrence risk of venous thromboembolism on a...

journal_title：Hamostaseologie

authors： Lindhoff-Last E

更新日期：2011-02-01 00:00:00

abstract：:Children with an unexplained bleeding tendency are frequently referred to a haemostaseologist for further evaluation. Careful standardized history taking and clinical evaluation should allow for distinguishing bleeds after minor injury and trauma which are very common in all children. However, in two groups of childre...

journal_title：Hamostaseologie

authors： Knöfler R,Streif W,Watzer-Herberth I,Hahn G,Schmidt U

更新日期：2019-02-01 00:00:00

abstract：UNLABELLED:In recent years thrombophilia has gained much attention as a risk factor for pregnancy complications. Whereas there is an established correlation between antiphospholipid-antibodies and pregnancy loss, data for other risk factors of thrombosis are less well established. Data suggest associations with antithr...

journal_title：Hamostaseologie

authors： Pabinger I

更新日期：2008-01-01 00:00:00

abstract：:For the last decades, anticoagulation for stroke prevention in atrial fibrillation (AF) as well as for the prophylaxis and long-term treatment of venous thromboembolism has been entirely based on vitamin K antagonists (VKA). Although very effective under optimal conditions, long-term treatment with these drugs is flaw...

journal_title：Hamostaseologie

authors： Steffel J,Luscher TF

更新日期：2012-01-01 00:00:00

abstract：:Direct oral anticoagulants (DOACs) are increasingly prescribed substances in patients with indication for effective anticoagulation. Patients with chronic kidney disease (CKD) have a high burden of cardiovascular risk and are more likely to develop atrial fibrillation (AF) than patients without CKD. Patients with mild...

journal_title：Hamostaseologie

authors： Kcükköylü S,Rump LC

更新日期：2017-01-01 00:00:00

abstract：:Placenta-mediated pregnancy complications are clinically important conditions and include preeclampsia, placental abruption, intrauterine growth restriction, and late fetal loss. Pathophysiology is complex, and may be linked to prothrombotic disorders such as antiphospholipid syndrome, whose understanding is still evo...

journal_title：Hamostaseologie

authors： Skeith L,Blondon M,Ní Áinle F

更新日期：2020-08-01 00:00:00

abstract：:Patients with myeloproliferative neoplasms (MPNs), such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are at increased risk for arterial and venous thrombosis/thromboembolism. In particular, the risk of splanchnic venous thrombosis, such as portal vein thrombosis or Budd-Chiari syndrome, ...

journal_title：Hamostaseologie

authors： Koschmieder S

更新日期：2020-02-01 00:00:00

abstract：UNLABELLED:Clot waveform analysis extends the interpretation of aPTT measurement curves. The curve is mathematically processed to obtain information about fibrin formation kinetics including semiquantitative determination of thrombin, prothrombinase and tenase activity. PATIENTS, METHOD:In this study the feasibility o...

journal_title：Hamostaseologie

authors： Siegemund T,Scholz U,Schobess R,Siegemund A

更新日期：2014-01-01 00:00:00

abstract：:Platelet and coagulation factor-dependent thrombus formation is critical to limit posttraumatic blood loss at sites of vascular injury. However, under pathological conditions like rupture of an atherosclerotic plaque, it may also lead to vessel occlusion causing myocardial infarction or stroke. Therefore, antithrombot...

journal_title：Hamostaseologie

authors： Hagedorn I,Vögtle T,Nieswandt B

更新日期：2010-08-01 00:00:00

abstract：:A survey is given on the clinical important haemostatic facts and diseases in gynaecology and obstetrics from the viewpoint of the gynecologist and obstetrician. ...

journal_title：Hamostaseologie

authors： Heilmann L

更新日期：2004-11-01 00:00:00

abstract：:Acquired haemophilia A (AHA) is a rare autoimmune disease caused by antibodies directed against clotting factor VIII. About half of cases are idiopathic, but AHA may also be secondary to autoimmune, dermatologic, or oncologic diseases. In approximately 10% of non-idiopathic cases, the disease occurs after or with the ...

journal_title：Hamostaseologie

authors： Pacholczak R,Bazan-Socha S,Zdziarska J,Iwaniec T,Walocha JA,Musiał J,Dropiński J

更新日期：2019-02-01 00:00:00

abstract：INTRODUCTION: Regular visits at haemophilia treatment centres (HTCs) in rural regions are often dependent on the access to a private car due to lack of or limited availability of public means. Therefore, a mobile haemophilia outpatient care (MHOC) concept providing home visits to haemophilia patients has been developed...

journal_title：Hamostaseologie

authors： Eichler H,Schleicher C,Heine S,Graf N,von Mackensen S

更新日期：2018-08-01 00:00:00

abstract：UNLABELLED:Atherosclerosis is a chronic inflammatory disease of the vascular wall that results from disturbed lipoprotein metabolism and increased oxidative stress. A major consequence of this is lipid peroxidation, which generates a number of breakdown products of membrane lipids that form so called oxidation-specific...

journal_title：Hamostaseologie

authors： Bartolini Gritti B,Binder CJ

更新日期：2016-05-10 00:00:00

abstract：:For decades, the options for therapeutic anticoagulation were limited to unfractionated heparin (UFH) and vitamin K antagonists (VKA), and their well-known limitations had to be accepted. With the introduction of the various LMWHs, the short- and medium-term anticoagulation could be much improved, but an alternative t...

journal_title：Hamostaseologie

authors： Alban S

更新日期：2008-12-01 00:00:00

abstract：:Trauma-induced coagulopathy (TIC) is a heterogeneous entity that contributes to a significant morbidity and mortality following trauma. The activated protein C system, endotheliopathy and platelet dysfunction have been implicated in the pathogenesis of TIC, although there are still controversies on the exact pathogene...

journal_title：Hamostaseologie

authors： Petros S

更新日期：2019-02-01 00:00:00

abstract：:This case report concerns a pregnant multipara (age: 27 years) in the 16th gestational week. She developed a sudden onset of paraesthesia in her left lower arm although injecting dalteparin 5000 IU once daily subcutaneously (s. c.) due to a heterozygous factor V Leiden mutation and a prior miscarriage in the first pre...

journal_title：Hamostaseologie

authors： Hecking C,Hendelmeier M,von Bardeleben R,Gröschel K,Schinzel H

更新日期：2014-01-01 00:00:00

abstract：:The impact of inherited bleeding disorders on the socioeconomic status (SES) of affected individuals is not clear. The SES of adult patients with congenital bleeding disorders (PWBD) from a centre in Germany (age 42.3 ± 15.0 years) was compared to that of a gender- and age-matched control group of patients with thromb...

journal_title：Hamostaseologie

authors： Holstein K,von Mackensen S,Bokemeyer C,Langer F

更新日期：2018-08-01 00:00:00

abstract：:By the use of conventional anticoagulants, significant improvements were achieved in all fields of medicine. Although efficacious and widely used, their use is limited in several respects. In particular, the use of vitamin K antagonists is restricted in the clinical routine setting. The main reasons are the delayed on...

journal_title：Hamostaseologie

authors： Haas S

更新日期：2002-08-01 00:00:00

abstract：:Superficial vein thrombosis (SVT) is a common disease, characterized by an inflammatory-thrombotic process in a superficial vein. Typical clinical findings are pain and a warm, tender, reddish cord along the vein. Until recently, no reliable epidemiological data were available. The incidence is estimated to be higher ...

journal_title：Hamostaseologie

authors： Bauersachs RM

更新日期：2013-08-01 00:00:00

abstract：:Nutrient excess in obesity drives metabolic reprogramming in multiple tissues involving extensive interorgan and intercellular crosstalk. Experimental and clinical studies show that prolonged nutrient excess often compromises metabolic adaptation propagating proobesogenic and proinflammatory responses. Chronic inflamm...

journal_title：Hamostaseologie

authors： Madhusudhan T,Ruf W

更新日期：2019-06-01 00:00:00

abstract：:Clinically overt, preoperative, disseminated intravascular coagulation (DIC) is not common in patient with aortic aneurysm. However, cases with large and expanding aneurysm are especially prone for this coagulopathy. Contrary to the clinically overt form compensated DIC in patients with aortic aneurysm seems frequent ...

journal_title：Hamostaseologie

authors： Jeleńska MM

更新日期：2004-08-01 00:00:00

abstract：:Current antithrombotic therapy in patients with acute coronary syndrome (ACS) comprises antiplatelet and anticoagulant therapy. Dual antiplatelet therapy composed of aspirin plus a third generation P2Y12 inhibitor (prasugrel or ticagrelor) represents the gold standard, while aspirin plus second generation P2Y12 inhibi...

journal_title：Hamostaseologie

authors： Ahrens I,Bode C,Zirlik A

更新日期：2014-01-01 00:00:00

abstract：:The antiphospholipid syndrome (APS) is defined by the association of arterial and/or venous thrombosis and/or pregnancy complications with the presence of at least one among the main antiphospholipid antibodies (aPL) (i. e., Lupus anticoagulants, LA, IgG and/or IgM anticardiolipin antibodies, aCL, IgG and/or IgM antiβ...

journal_title：Hamostaseologie

authors： Galli M

更新日期：2011-11-01 00:00:00

abstract：UNLABELLED:Secondary arthrosis of the upper ankle joint (talocalcanean joint) is one of the most frequent forms of haemophilic arthropathy. It is a secondary form of arthrosis not only because of chronic synovitis and cartilage injury resulting from chronic recurrent intraarticular bleeds, but also due to the misalignm...

journal_title：Hamostaseologie

authors： Berdel P,Schott D,Pagenstert G,Pennekamp P,Oldenburg J,Wirtz DC,Seuser A,Gravius S

更新日期：2009-10-01 00:00:00

abstract：:Myocardial infarction (MI) shows a strong heritability. For a long time the identification of responsible genes has been rather unsuccessful. However, with the advent of genome wide association studies (GWAS) using DNA-array technology a number of significant loci for MI have been identified which were widely replicat...

journal_title：Hamostaseologie

authors： Linsel-Nitschke P,Erdmann J,Schunkert H

更新日期：2010-11-01 00:00:00

abstract：UNLABELLED:Clinical expression of inadequately treated haemophilia is dominated by orthopedic complications, requiring invasive or non-invasive interventions. OBJECTIVE:In Romania, with under dosed and late introduced "on demand" substitution, we aimed at highlighting the experience of orthopedic treatment and its out...

journal_title：Hamostaseologie

authors： Serban M,Mihailov MD,Poenaru D,Pop L,Branea I,Bataneant M,Lăcătuşu A,Barna L,Tepeneu N,Schramm W

更新日期：2008-10-01 00:00:00

abstract：:Nearly every second patient, who needs an oral anticoagulation with vitamin K antagonists, is feasible to perform INR-self-management after participation in a structured teaching program. Patient self-testing or measuring the INR value by relatives or nurses would increase this method of control by the aid of telemedi...

journal_title：Hamostaseologie

authors： Völler H,Braun S

更新日期：2008-10-01 00:00:00

abstract：OBJECTIVE: This article aims to analyze the phenotype and genotype of an inherited dysfibrinogenemia pedigree associated with a heterozygous mutation in the FGA gene, and to investigate the pathogenesis of this disease. CLINICAL PRESENTATION: The proband of interest is a 29-year-old woman. She was in her 37 weeks of g...

journal_title：Hamostaseologie

authors： Li S,Wang M,Li X,Xu Q,Liu S,Luo S,Chen Y

更新日期：2020-12-01 00:00:00