Abstract:
:Haemophilic arthropathy is a complex multifactorial disorder that poses significant challenges to both the treating haematologist and arthroplasty surgeon. Its pathogenesis is incompletely understood. Recent literature has concentrated on the toxic effects of iron and the characteristic inflammatory synovitis. Discussion of the role of subchondral bleeding in joint damage has been neglected. A case of haemophilic arthropathy with extensive evidence of subchondral bleeding and related osteochondral destruction is presented. RESULT:The relevance of this mechanical pathway in the future management of haemophilic arthropathy is discussed with reference to recent literature. CONCLUSION:Clinicians should consider its importance when deciding whether to manage patients expectantly or with prophylactic factor replacement. :Die hämophile Arthropathie ist eine komplexe, multifaktorielle Störung, die sowohl für den behandelnden Hämatologen als auch für den Operateur eine erhebliche Herausforderung darstellt. Die Pathogenese ist noch nicht völlig geklärt. Die aktuelle Literatur konzentriert sich auf die toxische Wirkung von Eisen und die charakteristische Synovitis. Die Rolle der subchondralen Blutung bei der Gelenkschädigung wurde bisher nicht diskutiert. Es wird ein Fall einer hämophilen Arthropathie mit erheblichen Anzeichen für eine subchondrale Blutung und eine entsprechende osteochondrale Schädigung vorgestellt. ERGEBNIS:Die Bedeutung dieses mechanischen Ansatzes bei der zukünftigen Behandlung der hämophilen Arthropathie wird mit Verweis auf die aktuelle Literatur besprochen. SCHLUSSFOLGERUNG:Ärzte sollten diese Bedeutung bei der Entscheidung berücksichtigen, Patienten abwartend oder durch prophylaktische Faktorsubstitution zu behandeln.
journal_name
Hamostaseologiejournal_title
Hamostaseologieauthors
Wharton R,Austin S,Back Ddoi
10.5482/HAMO-15-12-0033subject
Has Abstractpub_date
2017-01-01 00:00:00pages
104-106issue
2eissn
0720-9355issn
2567-5761journal_volume
37pub_type
杂志文章相关文献
Hamostaseologie文献大全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
pub_type: 杂志文章
doi:10.1055/a-1184-8388
更新日期:2020-08-01 00:00:00
abstract::Platelets react immediately in response to traumatic vascular injury by adhesion, activation, aggregation and subsequent haemostatic plug formation. While this reaction pattern is essential for haemostasis, platelet responses can also cause occlusive thrombi in diseased arteries, leading to myocardial infarction or st...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.1055/s-0038-1675144
更新日期:2018-11-01 00:00:00
abstract::Patients suffering from haemophilia encounter various phases in life, in which individual needs, life situations, and self- and disease perception change rapidly. One of these phases spans from the beginning of puberty until early adulthood, in which individuals gain self-responsibility and reach independence and auto...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.1055/s-0039-3402804
更新日期:2020-02-01 00:00:00
abstract::Vitamin K 2,3-epoxide reductase complex, subunit 1 (VKORC1) is an enzyme essential for the vitamin K cycle. VKORC1 catalyses the reduction of vitamin K 2,3-epoxide to the quinone form of vitamin K and further to vitamin K hydroquinone. The generated vitamin K hydroquinone serves as substrate for the enzyme γ-glutamyl-...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:
更新日期:2016-11-08 00:00:00
abstract::Platelet cytoskeletal reorganization is essential for platelet adhesion and thrombus formation in hemostasis and thrombosis. The Rho GTPases RhoA, Rac1 and Cdc42 are the main players in cytoskeletal dynamics of platelets responsible for the formation of filopodia and lamellipodia to strongly increase the platelet surf...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.5482/HAMO-14-09-0046
更新日期:2016-08-03 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
pub_type: 杂志文章,评审
doi:10.5482/ha-1165
更新日期:2011-11-01 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
pub_type: 杂志文章
doi:10.1055/s-0038-1654721
更新日期:2018-08-01 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
pub_type: 杂志文章,评审
doi:10.1055/s-0039-1688800
更新日期:2019-06-01 00:00:00
abstract::Von Willebrand disease (VWD) is known for its marked heterogeneity which was already recognized by von Willebrand in 1926. The basis of phenotypic differentiation are quantitative and qualitative or functional differences between the different types and subtypes of VWD. One of the most important tools in the classific...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.1267/hamo04010027
更新日期:2004-02-01 00:00:00
abstract:UNLABELLED:An adequate number of qualified haemophilia centres is an essential requirement for effective and cost-efficient haemophilia care. During a reassessment of the delivery of haemophilia care in Germany a broad range of criteria relating to structure and quality of the centres were defined and a questionnaire w...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2012-01-01 00:00:00
abstract:UNLABELLED:The retrospective observational study surveys the relationship between development of inhibitors in the treatment of haemophilia patients and risk factors such as changing FVIII products. A total of 119 patients were included in this study, 198 changes of FVIII products were evaluated. RESULTS:During the ob...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2012-01-01 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
pub_type: 杂志文章,评审
doi:10.1055/s-0039-1677853
更新日期:2019-02-01 00:00:00
abstract::Studies describe benefits of exercise in Patients with haemophilia (PwH), but research on training sustainability is rare. Aim of this study was to observe the follow-up of a guided 6-month training intervention in PwH. This study included 28 PwH after a training intervention (RCT) over 6 months. N=17 continued traini...
journal_title:Hamostaseologie
pub_type: 杂志文章,随机对照试验
doi:10.5482/HAMO-17-04-0016
更新日期:2017-01-01 00:00:00
abstract:UNLABELLED:Antibodies against annexin-V, a potent anticoagulant abundant in placental tissues, were recently controversially reported to be associated with recurrent miscarriages or failures of in-vitro-fertilization (IVF) attempts. PATIENTS, MATERIAL, METHODS:We screened 56 women (34.7 +/- 4.3 years of age: mean +/- ...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2005-11-01 00:00:00
abstract::Methylation, CpG island, promoter, intron 1 Haemophilia A is the most common X-linked inherited coagulation disorder caused by a deficiency of the factor VIII protein (FVIII). A plethora of different mutations in the factor VIII gene (F8) have been identified as causative for this bleeding disease including a few prom...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2013-01-01 00:00:00
abstract::Platelets play a crucial role in haemostasis and several pathophysiological processes. Collagen is a main initiator for platelet activation and aggregation. Given that Wnt signalling negatively regulates platelet function, and IWR-1 (a small molecule inhibitor for Wnt signalling) has the potential of inhibiting collag...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:10.1055/s-0038-1676822
更新日期:2019-11-01 00:00:00
abstract::Thrombocytosis (defined as platelets >450 x 109/l) has several aetiologies. After having excluded spurious thrombocytosis (e. g., due to microspherocytes, schistocytes, cryoglobulins, or bacteria), the differential diagnosis of true thrombocytosis encompasses secondary causes (as diverse as inflammation, infection, ma...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.5482/HAMO-14-11-0074
更新日期:2016-11-07 00:00:00
abstract::We describe the case of a 30-year-old woman with homozygous prothrombin G20210A transition and heterozygous FV Leiden mutation and a history of postpartum venous thrombosis. Despite the high thrombotic risk to be assumed in the presence of this combined thrombophilia our patient suffered her first venous thrombotic ev...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2003-08-01 00:00:00
abstract::Three selected casuistic studies of individual patient problems from my long-standing professional experience are presented. I intend to highlight the potential contribution of case studies, including new approaches of laboratory investigation, for the advancement of pathophysiological knowledge and would encourage to...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:10.1055/a-1191-7198
更新日期:2020-11-01 00:00:00
abstract::This article reviews the roles of blood platelets in haemostasis as well as in the pathogenesis of thromboembolic diseases. Besides the basic processes in primary haemostasis, platelet adhesion, platelet secretion, platelet aggregation, clot retraction, the new model of thrombin formation on the platelet surface is pr...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:
更新日期:2008-12-01 00:00:00
abstract::The antiphospholipid syndrome (APS) has occupied haemostaseologists, rheumatologists and obstetricians since its initial description 35 years ago. Its name has been coined because of the antibodies against phospholipids which were the common property of affected patients. In particular, the pathogenesis of APS has bee...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.1055/s-0038-1675355
更新日期:2019-06-01 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
pub_type: 杂志文章,评审
doi:10.5482/HAMO-13-09-0048
更新日期:2014-01-01 00:00:00
abstract::Atherosclerosis and its sequelae have a major impact on morbidity and mortality. The rupture of an inflamed atherosclerotic plaque is a crucial event, since it can result in acute thrombotic closure of an arterial vessel, resulting e. g. in myocardial infarction or stroke. Not only detection of early plaque rupture wi...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.5482/HAMO-14-11-0061
更新日期:2015-01-01 00:00:00
abstract::Upon vascular injury or tissue damage, the exposed intracellular material such as nucleic acids, histones and other macromolecules may come into contact with vessel wall cells and circulating blood cells and may thus, have an enduring influence on wound healing and body defence processes. This short review summarizes ...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:10.5482/HAMO-13-01-0001
更新日期:2013-01-01 00:00:00
abstract::This is a very short review on quantitative coagulation factor assays for the beginner. For systematic training several excellent textbooks in German language are available. Quantitative functional assays of coagulation factors and of physiological inhibitor proteins are based on the principle of parallel-line or slop...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:
更新日期:2006-01-01 00:00:00
abstract:UNLABELLED:With the development of clotting-factors in the seventies the haemophilia patients were released from being handicapped and began to live a quite normal life. Thus, psychosocial counselling did not seem to be necessary. But the impact of HIV-infection to the world of haemophilia was so intense that professio...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2010-11-01 00:00:00
abstract:AIM:In children, screening for haemorrhagic disorders is further complicated by the fact that infants and young children with mild disease in many cases most likely will not have a significant history of easy bruising or bleeding making the efficacy of a questionnaire even more questionable. PATIENTS, METHODS:We compa...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2009-10-01 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
pub_type: 杂志文章
doi:
更新日期:2008-10-01 00:00:00
abstract::Percutaneous coronary intervention (PCI) has significantly improved clinical outcomes in coronary artery disease patients. Since PCI is associated with platelet activation, antiplatelet therapy with aspirin, clopidogrel and GPIIb/IIIa inhibitors comprise the cornerstone strategy during and following PCI. The latter ag...
journal_title:Hamostaseologie
pub_type: 杂志文章,评审
doi:
更新日期:2009-11-01 00:00:00
abstract:UNLABELLED:The retrospective cohort study surveys the influence of age, co-morbidity and laboratory values on FVIII-activity (FVIII:C) in patients with haemophilia A with (mild n = 48, moderate n = 10, severe n = 7 and carriers n = 23). Median observation was 19 years for patients with haemophilia A and 9,5 years for c...
journal_title:Hamostaseologie
pub_type: 杂志文章
doi:
更新日期:2011-11-01 00:00:00