Recombinant activated factor VII (rFVIIa) (NovoSeven; Novo Nordisk A/S, Bagsvaerd, Denmark) is a haemostatic agent first developed for bleeding associated with haemophilia and trauma, but for which the indications continue to expand. Recent reports have suggested efficacy for various types of intracranial haemorrhage and for patients with abnormalities of coagulation. ...

OBJECTIVE:: The inability to regulate the inflammatory response initiated upon infection leads to severe sepsis, characterized by widespread microvascular injury and thrombosis, organ ischemia, and dysfunction. A disintegrin-like and metalloprotease with thrombospondin (ADAMTS)-13 regulates primary hemostasis by proteolyzing von Willebrand factor (VWF). Decreased ADAMTS-13 has been reported in disseminated intravascular ...

INTRODUCTION: Heart failure (HF) is associated with coagulation activation, abnormal inflammation and endothelial dysfunction. High levels of von Willebrand factor (VWF) may manifest endothelial dysfunction and hypercoagulable state. The haemostatic activity of VWF is a function of multimers size; only large multimers of VWF are haemostatically active. Thrombospondin-1 (TSP-1) reduces ...

RATIONALE: Severe sepsis is associated with an exacerbated procoagulant state with protein C (PC) system impairment. In contrast, the inflammatory and coagulation status of nonseptic patients with organ failure (OF) is less documented. OBJECTIVES: To compare coagulation activation, focusing on the PC system, and inflammatory status in septic and nonseptic ...

In this article we present the history of a previously healthy female adolescent, who was seen at our hospital with abdominal pain. This was the result of a large floating thrombus in the aorta. Widespread embolism occurred, which lead to the loss of a limb and a left hemicolectomy. Although ...

Fibrin clots with reduced permeability, increased clot stiffness and reduced fibrinolysis susceptibility may predispose to cardiovascular disease (CVD). Little is known, however, about the structure of fibrin clots in patients with end-stage renal disease (ESRD). These patients suffer from a high risk of CVD in addition to their chronic low-grade ...

PURPOSE: An increased incidence of thromboembolic complications has been observed in multiple myeloma (MM), especially when patients are treated with anthracycline-based chemotherapy. In patients with MM, plasma levels of several prothrombotic coagulation factors are increased, and this can contribute to the prothrombotic state of these patients. Recently, an increased thrombosis ...

Large heterogeneity in bleeding pattern and arthropathy is observed among patients with severe hemophilia. Studies have reported a large variability in bleeding pattern among patients with severe hemophilia. Of special interest are some 10% of the patients with severe hemophilia who only rarely bleed and don't need prophylactic therapy. Prothrombotic ...

To determine the relationship between macrophage migration inhibitory factor (MIF) and disseminated intravascular coagulation (DIC) in patients with systemic inflammatory response syndrome (SIRS) and sepsis, and their relationship to multiple organ dysfunction syndrome (MODS) and prognosis, we conducted a prospective cohort study. Forty-eight patients with SIRS or sepsis were classified ...

We aimed to evaluate the reasons for, and timing of, treatment changes in a cohort of treatment-naïve patients initiating non-nucleoside reverse transcriptase inhibitor (NNRTI)-containing highly active antiretroviral therapy (HAART). All 268 patients initiating these regimens between January 1998 and September 2003 were included. Median follow up was 103 weeks. The ...

Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients ...

CONTEXT: The development of inhibitory antibodies against infused factor (F) VIII is a major complication of treatment of patients with severe hemophilia A. OBJECTIVE: This study was set up to examine the effects of treatment-related factors on inhibitor development among previously untreated patients with severe hemophilia A. DESIGN, SETTING AND ...

In Japan, early diagnosis and early treatment of disseminated intravascular coagulation (DIC) based on the old Japanese criteria have greatly improved the outcomes of DIC patients with hematopoietic malignancy. However, the prognoses of critically ill patients with DIC have remained poor. To overcome this situation, new Japanese DIC criteria for ...

BACKGROUND/PURPOSE: The pharmacokinetic (PK) study of recombinant human factor IX (rFIX) has been done in patients with hemophilia B in areas other than Taiwan. However, wide patient-related variability in recovery is noted and there is no PK study of rFIX in Taiwanese patients with hemophilia B. The purpose of this ...

Prothrombotic coagulation abnormalities were analyzed in patients with untreated multiple myeloma. Increases in factor VIII, in von Willebrand factor (vWF) and a decrease in protein S were observed and these changes were strongly associated with disease stage. No difference in baseline coagulation parameters was found between patients with and without ...

OBJECTIVE: To investigate the effects of angiotensin converting enzyme (ACE) inhibitors/angiotensin receptor blockers (ARBs) and other anti-hypertensive agents on recombinant human erythropoietin (rHuEPO) in chronic renal failure (CRF) patients. METHODS: The present study was conducted at the Nephrology Department, Khan Research Laboratories Hospital and Quaid-i-Azam University, Islamabad, Pakistan during March ...

Plasminogen activator inhibitor 1 is known to be elevated in patients with hepatic VOD after intensive chemotherapy. To re-establish endogenous fibrinolysis and to inhibit thrombin formation, we used non-APC (zymogen) to normalize PAI-1 levels. As a consequence of thrombin formation inhibition and the consecutive inhibition of the coagulation cascade, this ...

Heparin-induced thrombocytopenia (HIT) is known to complicate disseminated intravascular coagulation (DIC), but rarely to be complicated by DIC. We measured the titers of anti-PF4/hepatin complex antibodies by ELISA (HIT-Elisa) and examined 4 parameters of coagulation and fibrinolysis [D-dimer, thrombin/antithrombin complex (TAT), plasmin/alpha2-plasmin inhibitor complex (PIC), and antithrombin levels] in 80 ...

Beta-thalassemia/hemoglobin (Hb) E is a hereditary hemolytic anemia with varying degrees of severity. Severely affected patients are treated with blood transfusion and/or splenectomy in order to maintain an optimum level of hemoglobin for normal growth and physical activities. As thrombosis has been observed among splenectomized patients, we have investigated alterations ...

We studied the state of ultra-large von Willebrand factor (ULVWF) proteolysis in 21 pediatric patients with severe sepsis and found that the overall group of patients had moderately reduced ADAMTS-13 activity, but 31% had severe enzymatic deficiency. The severe deficiency correlated with greater adhesion activity of von Willebrand factor, severity ...

INTRODUCTION: We conducted a prospective study to test the hypothesis that the activation of neutrophil elastase-mediated fibrinolysis is insufficient to overcome the fibrinolytic shutdown of disseminated intravascular coagulation (DIC) in patients associated with systemic inflammation. MATERIALS AND METHODS: We investigated 45 consecutive patients with systemic inflammatory response syndrome (SIRS) and ...

The development of inhibitors in previously untreated patients (PUPs) with haemophilia A is correlated with a variety of endogenous and exogenous risk factors. It is still controversial whether recombinant factor VIII (rFVIII) products pose a higher risk for inhibitor development than plasma-derived (pd) FVIII concentrates, particularly with intact von Willebrand ...

Cerebral venous thrombosis (CVT) rarely recurs, and the factors associated with a recurrence remain unclear. Recently, however, elevated plasma coagulation factor VIII has been considered a factor related to recurrent venous thromboembolism. Here we report a patient who had recurrent CVT associated with significantly elevated levels of factor VIII despite ...

OBJECTIVE: CgA has been shown to be an excellent marker for neuroendocrine tumours. However, there are two major drawbacks with CgA measurements; elevated levels are common in patients with decreased renal function and in patients on treatment with proton pump inhibitors. These problems are not seen with CgB measurements. We ...

Although the presence of decreased plasma fibrinogen has been regarded as an indicator of ongoing disseminated intravascular coagulation (DIC), fibrinogen, which is one of the acute phase reactants, is often increased in the patients with DIC. We investigated the diagnostic and prognostic utility of a new parameter [the fibrinogen/C-reactive protein ...

We report the case of a 71-year-old man on warfarin for chronic atrial fibrillation presenting with a massive spontaneous soft tissue bleed. Despite reversing the effects of warfarin with large doses of intravenous vitamin K and fresh frozen plasma, bleeding continued, and his prothrombin time and activated partial thromboplastin time ...

BACKGROUND: Angioedema without major urticarial flares (hives) is poorly understood. Its causes are diverse, and little is known about its pathogenic mechanisms. We report on our 11 years of experience with this condition and propose a classification of patients affected by angioedema unaccompanied by urticaria. METHODS: From 1993 through 2003 ...

There are many complex pathophysiologic changes of the coagulation system in sepsis. The fibrinolytic system was evaluated in septic children using the global fibrinolytic capacity (GFC), a new technique reflecting the overall fibrinolytic activity. The study consisted of 24 children with sepsis, 36 children with sepsis plus disseminated intravascular coagulation ...

The objective was to estimate the effect of antithrombin therapy on mortality in disseminated intravascular coagulation (DIC) of severe sepsis and septic shock. Randomized clinical trials (RCT) on patients with DIC and severe sepsis or septic shock assigned to intravenous antithrombin or placebo were searched. Eligible studies reported death as ...

AIM: To explore the association between the coagulation protein fibrinogen and the fibrinolytic biomarker plasminogen activator inhibitor-1 (PAI-1) and the long-term mortality after coronary artery bypass grafting (CABG). PATIENTS AND METHODS: In 729 patients undergoing CABG at Sahlgrenska University Hospital, a blood sample for fibrinogen and PAI-1 was collected prior ...

Thesis (M.Sc.)--Chulalongkorn University, 2004

We wanted to see whether the active inflammation in Behcet's disease (BD) can cause a thrombotic disorder by decreasing the protein S (PS) activity, and we evaluated the relationship between the decreased PS activity and the disease activity of BD. We included 122 patients with BD whose PS activity levels ...

The authors describe breast augmentation in a patient with von Willebrand disease (vWD), providing a template for treating such patients. Because there is no single ideal laboratory test predictive of surgical bleeding, preoperative history remains the most important diagnostic tool. A vigilant pursuit of signs of undiagnosed vWD or other ...

BACKGROUND: The aim of the present paper was to determine the mechanism by which the level of coagulation factor XIIIA declines during the active phase of Crohn's disease. METHODS: The relationship between the activity of Crohn's disease and factor XIIIA was observed in 31 patients with Crohn's disease prospectively. The ...

We evaluated the score for disseminated intravascular coagulation (DIC) recently published by the International Society for Thrombosis and Haemostasis (ISTH) in a well-defined series of sepsis patients. Thirty-two patients suffering from severe sepsis and eight patients with septic shock were evaluated following the ISTH DIC score. Fibrin monomer and D-dimer ...

The development of inhibitors in patients with mild/moderate hemophilia A is an increasingly recognized occurrence and is manifested by the patients' bleeding pattern becoming more severe. Inherited (hemophilia genetic mutations) and acquired (type and delivery of factor VIII replacement therapy) factors have been associated with an increased likelihood of developing ...

Gaucher disease is the most prevalent inherited lysosomal storage disorder caused by deficiency of beta-glucocerebrosidase enzyme. Clinically, 3 forms of Gaucher disease are recognized, of which type 1 is the mild to moderately severe, slowly progressive, nonneuropathic form. Bleeding disorders in Gaucher disease are believed to be due to thrombocytopenia ...

PURPOSE: Valproic acid (VPA) is an antiepileptic drug (AED) commonly used for generalized and focal epilepsies. The clinical relevance of coagulopathies, known as side effects of VPA therapy, especially thrombocytopenia, von Willebrand disease, and a decrease of factor XIII, is still unclear. METHODS: In our institute, we noticed a high ...

Surgical treatment of patients with bleeding disorders requires careful planning. The use of fibrin sealants following a dental extraction in a patient with von Willebrand's disease is presented in this report. A female patient with von Willebrand's disease had an extraction of a maxillary right third molar. After evaluation by ...

OBJECTIVE: In bleeding patients who are coagulopathic, the clinical response to administration of recombinant factor VIIa (rFVIIa) relates to the changes in prothrombin time (PT). DESIGN: Retrospective review of all surgical and trauma patients who were coagulopathic and received factor VIIa at the authors' institution over the past 27 months. ...

Patients with cirrhosis and impaired coagulation often pose major therapeutic problems during bleeding episodes or invasive procedures. Recombinant activated factor VII (rFVIIa), which has been licensed for the treatment of haemophilia patients with factor VIII or IX inhibitors, has been occasionally used in cirrhotic patients. We present five patients with ...

This survey was conducted to evaluate coagulation factor VIII:C inhibitors among 102 hemophilia A patients from different cities of Khorasan province in north east of Iran in order to identify and characterize the pattern of inhibitor formation in these patients population. For this purpose, we randomly obtained plasma samples of ...

OBJECTIVE: The concentration of the complex between activated protein C (APC) and protein C inhibitor (PCI) is a measure of thrombin generation. We studied whether it can provide information useful for the diagnosis and treatment of arterial vascular disease. METHODS: Blood was obtained from 429 vascular patients admitted consecutively during ...

Neutralizing alloantibodies (inhibitors) to factor VIII or factor IX develop in approximately 25% of patients with haemophilia A and <3% of patients with haemophilia B treated with factor concentrate. Patients with high titre inhibitors, in whom immune tolerance therapy fails, have few treatment options. Targeted anti-B-cell therapy with rituximab (chimeric ...

BACKGROUND: In patients with Henoch-Schoenlein purpura (HSP), particularly with severe gastrointestinal symptoms, an associated decrease of plasma factor XIII has been observed. PATIENT: The authors report a case of HSP in a boy and describe the development of factor XIII activities throughout the course of the disease. Every relapse of ...

Chronic urticaria (CU), with or without angioedema, is a frequent disorder defined as the occurrence of pruritic wheals for > 6 weeks. Studies carried out in the last two decades showed that the origin of the disease is autoimmune in up to 50% of cases. Currently available treatments include antihistamines, ...

The objective of this study was to investigate the correlation between factor XIII (FXIII) activity and disseminated intravascular coagulation (DIC) parameters and also to evaluate the clinical usefulness of DIC diagnosis. Citrated plasma from eighty patients with potential DIC was analyzed for FXIII activity. The primary patient conditions (48 male ...

We evaluated the feasibility and effectiveness of therapeutic drug monitoring (TDM) and adherence support (collectively, AT) vs standard of care (SOC) in patients receiving HIV protease inhibitors (PIs) and nonnucleoside reverse transcriptase inhibitors (NNRTIs) within a nurse-led clinic. Primary end points were failure to achieve viral load of <50 copies/mL ...

BACKGROUND: Effective therapeutic drug monitoring for antiretrovirals requires a better understanding of intraindividual variability in pharmacokinetics. METHODS: We determined concentrations of human immunodeficiency virus (HIV) protease and nonnucleoside reverse-transcriptase inhibitors for 10 patients with undetectable plasma HIV RNA levels who had been receiving stable regimens for > or = 11 ...

Patients with primary hyperparathyroidism (PHPT) have impaired vasodilation both dependent and independent of endothelium. The aims of our study were to measure three different biochemical markers of endothelial activation, i. e., plasma thrombomodulin, soluble(s) E-selectin, and von Willebrand factor, in PHPT patients before and one year after successful parathyroidectomy, and ...