Abstract
SUMMARY: During the last two decades major advances have been achieved in the management of haemophilia. Modern approaches aimed at preventing the recurrent bleedings and their sequelae have been widely adopted. Major challenges of intensive treatment regimens employed today, such a short half life...
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PMID: 20590852
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Abstract
SUMMARY: Deficient or defective coagulation factor VIII (FVIII) and von Willebrand factor (VWF) can cause bleeding through congenital deficiency or acquired inhibitory antibodies. Recent studies on type 1 von Willebrand's disease (VWD), the most common form of the disease, have begun to explain its...
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PMID: 20590861
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Abstract
SUMMARY: Despite major advances in diagnosis and treatment, the management of patients with mild haemophilia (MH) remains a major challenge. Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU mL(-1). The bleeding associated with mild haemophilia is most frequently episodic, occurr...
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PMID: 20590864
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Abstract
SUMMARY: Anamestic inhibitors represent the major complication of haemophilia therapy now that clotting factor concentrates are virtually free of pathogen-transmission risk. Conventional clotting factor replacement is usually insufficient to prevent or treat bleeding in a haemophilia patient with a...
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PMID: 20590857
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Abstract
SUMMARY: Antibody responses to clotting factor concentrates remain a major treatment limitation. In conjucation with ongoing clinical studies, the pathogenesis and potential treatment of clotting factor immune responses is being evaluated in a variety of animal models.
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PMID: 20590856
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Abstract
Transgenic pigs were produced using viral, chicken, mouse, human, and porcine promoter sequences with ubiquitous or cell type-specific activity. In addition to the expression of human complement regulatory proteins, which were efficient to prevent hyperacute rejection of pig-to-primate xenografts, n...
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PMID: 20061949
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Abstract
We present three cases of children developing factor V and thrombin inhibitors following repeated exposure during cardiac surgical procedures....
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PMID: 20576051
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Abstract
We have investigated whether the thrombin generation assay can detect changes in the haemostatic balance associated with common genetic variation affecting the level or function of coagulation factors and inhibitors. The study population consisted of 140 healthy individuals. Plasma levels of coagula...
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PMID: 20062924
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Abstract
The development of neutralizing antibodies (inhibitors) after factor VIII (FVIII) infusions is a serious complication that affects approximately one-quarter of hemophilia A patients who have access to replacement therapy. To investigate the differentiation of naive T cells into FVIII-specific helper...
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PMID: 19549986
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Abstract
ITI with high-dose FVIII, intravenous immunoglobulins, and rFVIIa is a beneficial treatment option for hemophiliac A patients with high-titer FVIII inhibitor....
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PMID: 19488019
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Abstract
Several groups have reported further evidence for the importance of preexisting and elicited non-GalT antibodies, which are capable of fixing complement and activating graft endothelial cells. One important study showed that without complete suppression of these antibodies, there is a progressive ac...
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PMID: 19469030
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Abstract
PAD patients with reduced endogenous anti-coagulation proteins show worse prognosis than those without. Surgeons must be aware of it to improve the outcome of arterial revascularization....
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PMID: 19367244
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Abstract
We have determined the activities of antithrombin, protein C and protein S in 74 women with pregnancy related thrombosis and in 45 healthy women who had at least two uncomplicated pregnancies. Among the women with the history of venous thromboembolism antithrombin deficiency was found in 4 (5.4%), p...
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PMID: 19514602
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Abstract
Revealing of inhibitors form of hemophilia A form patients of hemophilia in Republic of Uzbekistan was studied and peculiarities of this form of hemophilia also was studied. Shown, that revealing of inhibitors form from 405 patients of hemophilia A was 7,7%. Shown that from patients with inhibitors...
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PMID: 19953993
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Abstract
Haemophilia therapy is aimed at treating and preventing bleeding episodes and related complications and clinical studies have shown that regular prophylaxis, started at an early age, is able to reduce physical impairment from haemophilic arthropathy. Today, the development of anti-Factor VIII (FVIII...
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PMID: 19125935
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Abstract
Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder that can present with umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding and life-threatening intracranial haemorrhage. FXIII deficiency has also been associated with poor wound healing an...
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PMID: 19141159
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Abstract
Factor V (FV; proaccelerin or labile factor) is the plasma cofactor for the prothrombinase complex that activates prothrombin to thrombin. FV deficiency can be caused by mutations in the FV gene or in genes encoding components of a putative cargo receptor that transports FV (and factor VIII) from th...
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PMID: 19141156
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Abstract
We recommend prophylaxis in haemophilic children with an inhibitor as a way of preventing the musculoskeletal impairment that is likely to affect them. This approach has been used for children without inhibitors with excellent results. If prophylaxis is not feasible, we suggest that intensive on-dem...
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PMID: 19134036
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Abstract
Although no consensus on the exact dosing schedule of prophylaxis in non-inhibitor haemophilia patients has been achieved, regular administration of factor VIII (FVIII) or factor IX (FIX) concentrates is currently accepted as the most effective treatment model in order to prevent sick days, hospital...
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PMID: 19134030
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Abstract
Haemophilia patients with inhibitor have a higher level of arthropathy and more severe joint morbidity than patients without inhibitors. In recent years, interest has grown in the possibility that bypassing agent regimens could prevent bleeding and, consequently, arthropathy in inhibitor patients. N...
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PMID: 19134031
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Abstract
Recurrent bleeding into joints represents the clinical hallmark of haemophilia and, if not adequately treated, it may cause chronic synovitis and degenerative arthropathy. In haemophilia patients with inhibitors, a more severe degree of synovitis is often observed owing to the fact that treatment is...
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PMID: 19134034
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Abstract
Treatment of haemophilia patients with inhibitors against factor VIII/IX (FVIII/IX) is still challenging and recurrent haemarthroses cause arthropathy with associated restrictions on participation in physical activities and sports. Rehabilitation is a multidisciplinary approach which includes physio...
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PMID: 19134033
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Abstract
We believe that it is necessary to unify criteria and that the EFFISEVEN protocol may contribute data that improve standards which, in turn, will influence the degenerative process of joints, and consequently affect the quality of life of haemophilia patients with inhibitors. Echographical control o...
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PMID: 19134032
PDF is available here.
Abstract
The incidence of haemarthrosis is similar in young haemophilia patients with or without inhibitors, but whether this is due to the effect of modern therapy is unknown. In comparison with younger patients with haemophilia, older haemophilia patients have a lower incidence of haemarthrosis because of...
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PMID: 19134029
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Abstract
Our view is that the well-known concepts of prophylaxis in patients without inhibitors should be followed and applied to patients with inhibitors. In fact, prophylaxis seems to be the only way to prevent the development of haemophilic arthropathy in patients with haemophilia. Until this goal can be...
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PMID: 19134035
PDF is available here.
Abstract
Increasing evidence suggests that a single dose of 270 microg/kg recombinant activated factor VII (rFVIIa) may be a convenient, safe, and effective alternative to the repeat-dose regimen for hemophilia patients with inhibitors. Three recent trials investigating on-demand treatment (Kavakli et al 200...
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PMID: 18820600
PDF is available here.
Haemophilia (14 Suppl 4)s4 2008
Abstract
We present the new developments in the treatment and laboratory monitoring of these patients. First, we discuss a general treatment algorithm to control severe bleeding episodes in these patients, established by an international panel of haemophilia specialists. The main features of this algorithm c...
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PMID: 18494689
PDF is available here.
Haemophilia (14 Suppl 3)s3 2008
Abstract
The immune response to factor VIII and the development of inhibitory antibodies is a complex multi-factorial process involving a variety of immune regulatory genes and cells, several of which have the potential to determine risk. A better understanding of the mechanisms involved will increase the li...
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PMID: 18510520
PDF is available here.
Haemophilia (14 Suppl 3)s3 2008
Author(s) unavailable
Abstract
During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. D...
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PMID: 18510543
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Abstract
We observed abnormalities in prothrombin time (PT) (8%) and activated partial thromboplastin time (APTT) (>200 seconds). FV activity was less than 3%, and a mixing test did not correlate with PT. FV inhibitor assay demonstrated 240 Bethesda units/ml. The patient also showed markedly decreased activi...
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PMID: 18646609
PDF is available here.
Abstract
Acquired hemophilia A is an uncommon but potentially life-threatening hemorrhagic disorder caused by the onset of autoantibodies against coagulation factor VIII. Acquired hemophilia A is most frequently associated with autoimmune diseases, neoplasia, pregnancy and drug reactions but in approximately...
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PMID: 18243727
PDF is available here.
Abstract
We report two consecutively treated patients whose acquired FVIII inhibitors did not respond to standard immunosuppressive regimens, and only when rituximab was added to therapy, complete response and prolonged remission were obtained....
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PMID: 18414981
PDF is available here.
Abstract
Recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) is well established as an effective hemostatic agent for the management of hemorrhage in hemophilia patients with inhibitors. Its use in prophylaxis is currently being investigated. On-demand treatment schedules u...
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PMID: 18544424
PDF is available here.
Abstract
Inhibitor development represents the most significant complication of hemophilia treatment today. Although it has long been established that hemophilia patients with inhibitors face a higher risk (and a greater extent) of joint morbidity than their non-inhibitor counterparts, there remains a paucity...
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PMID: 18544425
PDF is available here.
Abstract
Until recently, orthopedic surgery was strongly contraindicated in patients with hemophilia and inhibitors. However, recent advances in our knowledge of bypassing agents (particularly recombinant activated factor VII [rFVIIa]) that provide effective surgical hemostasis have allowed us to successfull...
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PMID: 18544428
PDF is available here.
Abstract
The inhibitor titer is the most important clinical measurement in inhibitor patients, and the Nijmegen method is preferable to the original and well-established Bethesda assay for this purpose; however, both methods have high inter-laboratory variability. Monitoring inhibitor patients after treatmen...
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PMID: 18544420
PDF is available here.
Abstract
In March 2007, the European Medicines Agency (EMEA) approved the use of single-dose recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) 270 microg/kg for the treatment of mild-to-moderate bleeding episodes in patients with hemophilia A or B with inhibitors. The pro...
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PMID: 18544423
PDF is available here.
Abstract
Arthropathy is prevalent in patients with haemophilia and inhibitors and is a major source of pain and disability, significantly reducing quality of life. Recombinant activated factor VII (rFVIIa; NovoSeven is one of the treatments available for acute life-threatening bleeding episodes in haemophili...
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PMID: 18081827
PDF is available here.
Abstract
Two bypassing agents are currently available to circumvent the need for factor FVIII in hemophilia A patients with inhibitors: the activated prothrombin complex FEIBA VH and recombinant activated factor VII (NovoSeven. Both products are highly effective in controlling bleeding in the presence of inh...
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PMID: 18485996
PDF is available here.
Abstract
We report the case of a 9-year-old boy with severe hemophilia B with an inhibitor and a history of anaphylaxis to factor IX (FIX), who was successfully treated with immune tolerance (IT) that included rituximab (Genentech, San Francisco, CA) and desensitization. The patient began the first course of...
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PMID: 18176193
PDF is available here.
Abstract
This is a two-part unit on haemophilia and inhibitors. This article, part 1, examines the condition, the problem of inhibitors and treatment options for patients with inhibitors. It also discusses the practical challenges nurses may face in patients' surgical management.
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PMID: 18669400
PDF is available here.
Abstract
Part 1 of this two-part unit on haemophilia and inhibitors examined the condition of haemophilia, the problem of inhibitors and practical challenges nurses may face in surgical management. This second part examines a case study of a 58-year-old man with severe haemophilia and an inhibitor. Communica...
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PMID: 18683391
PDF is available here.
Abstract
The safety of full-length sucrose-formulated recombinant factor VIII (rFVIII-FS; Kogenate FS) for up to 24 months of use was evaluated in a postmarketing observational study in Europe. Long-term safety and efficacy data were available for 212 patients with severe haemophilia A, including 13 previous...
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PMID: 18217134
PDF is available here.
Author(s) unavailable
Abstract
We and others have previously shown that inhibitor containing plasma from patients with congenital haemophilia A sometimes reacts less with von Willebrand factor (VWF) containing concentrates compared with highly purified plasma-derived or recombinant factor VIII (FVIII) concentrates. To further sub...
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PMID: 18078401
PDF is available here.
Abstract
We evaluated skeletal maturation (bone age), pubertal progression, serum testosterone levels, height velocity, and stature in the multicenter Hemophilia Growth and Development Study. A total of 333 children and adolescents (mean age, 12.4 years) were enrolled from 1989 to 1990 and followed for 7 yea...
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PMID: 17715388
PDF is available here.
Abstract
We compared various hemostatic and thrombotic markers in blood from 15 Hb E/beta-thal patients who were not splenectomized (NS), 15 who had been splenectomized (S), and 15 normal controls (NC). Levels of plasma thrombin-antithrombin, beta2 thromboglobulin, C-reactive protein, tissue plasminogen acti...
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PMID: 17654509
PDF is available here.
Abstract
This is the case of a 28-year-old man with severe congenital haemophilia A, who had a relatively mild bleeding course during early childhood, with limited factor VIII (FVIII) exposure. He was infected with HIV before the age of 7 years, and demonstrated profound immunodeficiency from childhood, with...
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PMID: 17973846
PDF is available here.
Abstract
Systematic monitoring of anti-Xa activity in elderly patients treated with enoxaparin at prophylactic doses does not seem to be necessary to prevent the occurrence of major bleeding....
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PMID: 17509040
PDF is available here.
Abstract
We conducted an open-label, randomized, multicenter trial comparing the efficacy and safety of rFVIIa administered by BI or CI for the surgical management of haemophilia A or B patients with inhibitors to FVIII or FIX. Safety was compared with that of a control group of non-inhibitor patients receiv...
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PMID: 17938794
PDF is available here.