Vonicog Alfa Safe for Bleeds in Severe von Willebrand Disease (CME/CE)

(MedPage Today) -- Treatment with recombinant von Willebrand factor stabilizes endogenous FVIII:C
Source: MedPage Today Hematology/Oncology - Category: Hematology Source Type: news

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We present a 3D coarse-grained computer model of VWF multimers in flowing viscous fluid that explicitely grasps the dynamics, the conformational changes and the hydrodynamics-induced activation of adhesivity of these protein concatamers to GPIb receptor of blood platelets. The model is based on the fluctuating Lattice Boltzmann method for modelling the hydrodynamics in the simulation box and the Lagrangian particle dynamics coupled to the fluid by a viscous drag force. The model has been validated by the comparison with the experimental data found in literature. We studied the effect of hydrophobic interactions on the conf...
Source: Journal of Theoretical Biology - Category: Biology Source Type: research
Abstract The proband's von Willebrand factor (VWF) antigen and VWF collagen-binding capacity were 14% and 10%, respectively; his sister's were 16% and 9%, respectively; and his nephew's were 30% and 15%, respectively. No apparent loss of high-molecular weight VWF multimers was observed in the plasma of these patients. A single-nucleotide substitution of T to C was found at nucleotide position 113042 in their VWF gene, converting Leu1733 to Pro in the A3 domain. These results suggest that p.Leu1733Pro is responsible for type 2M von Willebrand disease in this family. PMID: 31605304 [PubMed - as supplied by publisher]
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research
Bansal et al. demonstrated that the fully magnetically levitated centrifugal-flow HeartMate3 (HM3) left ventricular assist device is associated with greater preservation of the structure of vWF HMWMs than the HeartMateII (HMII) mechanical bearing axial-flow pump.1 The findings confirm our prior work regarding the development of AVWS in VAD patients and the less distinct loss of the vWF high molecular weight multimers in HM3 patients compared with HMII patients.2,3 However, there are some differences: Bansal et al.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
In this study we analyzed the quality and quantity of the structure of vWF by using mulitmer gel electrophoresis and also evaluated VWF functional activities, including platelet glycoprotein I β binding (VWF:Act LIA) and Factor VIII activity, to assess for acquired vWD (aVWD) with commercially available methods that are used in clinical practice and previously validated in congenital vWD.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Acetaminophen (paracetamol, APAP) overdose is a leading cause of drug-induced acute liver injury and acute liver failure (ALF) in the western world.[1] Accumulating evidence from experimental and clinical studies suggests that the hemostatic system contributes to the progression of acute liver injury after APAP overdose.[2 –4] Although not associated with clinically significant bleeding[5], substantial alterations in the hemostatic system are evident in patients with AAP-induced liver failure, including a reduced platelet count.
Source: Journal of Hepatology - Category: Gastroenterology Authors: Source Type: research
CONCLUSION:  Our results suggest that high-speed centrifugation is applicable to the majority of routine hemostasis parameters. The coverage probability was more sensitive than Spearman's rank correlation to detect disagreement among centrifugation schemes. PMID: 31600807 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Thromb Haemost Source Type: research
Angiogenesis and endothelial activation and dysfunction have been associated with acute graft-vs.-host disease (aGVHD), pointing to the endothelium as a potential target for pharmacological intervention. Defibrotide (DF) is a drug with an endothelium-protective effect that has been approved for the treatment of veno-occlusive disease/sinusoidal obstruction syndrome after allogeneic hematopoietic cell transplantation. Clinical data suggest that DF also reduces the incidence of aGVHD; however, the mechanisms of DF-mediated aGVHD regulation have not been examined. To investigate possible DF-mediated prophylactic and therapeut...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
We present a case of a 68-year-old Hispanic female with a history of factor VII deficiency who presented with shortness of breath, chest pain, and palpitations and was found to have pulmonary embolism. Our patient did not have any of the above-mentioned thrombotic risk factors. Our case and review of the literature show that factor VII deficiency does not provide protection against thrombosis. PMID: 31590173 [PubMed - as supplied by publisher]
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
Abstract Presently, it is difficult to accurately diagnose sepsis, a common cause of childhood death in sub-Saharan Africa, in malaria-endemic areas, given the clinical and pathophysiological overlap between malarial and non-malarial sepsis. Host biomarkers can distinguish sepsis from uncomplicated fever but are often abnormal in malaria in the absence of sepsis. To identify biomarkers that predict sepsis in a malaria-endemic setting, we retrospectively analyzed data and sera from a case-control study of febrile Malawian children (aged 6-60 months) with and without malaria who presented to a community health cente...
Source: Am J Trop Med Hyg - Category: Infectious Diseases Authors: Tags: Am J Trop Med Hyg Source Type: research
This study was designed to evaluate the effect of ulinastatin on vascular endothelial cell damage and coagulation in pregnant women with severe pre-eclampsia (PE).From October 2015 to November 2017 at Tianjin Central Hospital of gynecology and obstetrics in China. Eighty pregnant women with severe PE, who elected to deliver by cesarean section, were randomly assigned to a control group or an ulinastatin group. The plasma concentration of von Willebrand factor (vWF) and platelet granule membrane protein (GMP-140), platelet count, fibrinogen levels, prothrombin time (PT), and partial prothrombin activation time (APTT) w...
Source: An Acad Bras Cienc - Category: Science Authors: Tags: An Acad Bras Cienc Source Type: research
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