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Von willebrand's

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https://www.readbyqxmd.com/read/28810961/resuscitation-of-hypotensive-traumatic-brain-injured-animals-with-spray-dried-plasma-does-not-adversely-alter-physiology-and-improves-blood-brain-barrier-function
#1
Steven McDaniel, Stephanie Golla, Anthony N Moore, Joe DaCorta, Arthur Bode, Shibani Pati, Pramod K Dash, Jing Zhao
INTRODUCTION: According to the Defense and Veterans Brain Injury Center and the Armed Forces Health Surveillance Center, the number of soldiers who have sustained a traumatic brain injury (TBI) has risen dramatically over the past decade. Studies have shown that brain damage can be exacerbated if blood loss occurs (often occurring in polytrauma). As blood supply is critical for brain function and survival, TBI patients must be properly resuscitated to maintain blood volume, blood pressure, and cerebral perfusion...
July 2017: Military Medicine
https://www.readbyqxmd.com/read/28806860/patient-caregiver-and-provider-perceptions-of-pain-and-pain-management-in-adolescents-and-young-adults-with-bleeding-disorders
#2
Angela Lambing, Cynthia D Nichols, James E Munn, Terry L Anderson, Bartholomew J Tortella, Michelle L Witkop
INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28806755/acquired-von-willebrand-syndrome-in-cardiogenic-shock-patients-on-mechanical-circulatory-microaxial-pump-support
#3
Ulrike Flierl, Jörn Tongers, Dominik Berliner, Jan-Thorben Sieweke, Florian Zauner, Christoph Wingert, Christian Riehle, Johann Bauersachs, Andreas Schäfer
Early use of mechanical circulatory support, e.g. veno-arterial extracorporeal membrane oxygenation (ECMO) or left ventricular unloading by microaxial pump in refractory cardiogenic shock is recommended in current guidelines. Development of acquired von Willebrand Syndrome (AVWS) in patients with left ventricular assist devices (LVADs) and ECMO has been reported. There is an increasing number of patients treated with the Impella® CP microaxial pump for left ventricular unloading. However, the prevalence of AVWS in these high risk patients is unknown and needs to be determined...
2017: PloS One
https://www.readbyqxmd.com/read/28804850/laboratory-testing-for-von-willebrand-factor-multimers
#4
Susan Oliver, Kun Kan Edwin Lau, Kent Chapman, Emmanuel J Favaloro
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804849/ristocetin-induced-platelet-aggregation-ripa-and-ripa-mixing-studies
#5
Juan Pablo Frontroth, Emmanuel J Favaloro
Ristocetin-induced platelet aggregation (RIPA) is used as an in vitro test to determine the presence and integrity of the platelet glycoprotein (GP) Ibα-V-IX complex and von Willebrand factor (VWF) interaction and is usually performed using platelet-rich plasma (PRP). Impairment in the response of VWF/GPIbα-V-IX is measured with reference to several established concentrations of ristocetin and may indicate defects in VWF or in GPIbα-V-IX function. RIPA-based mixing studies comprise an additional approach to testing this interaction to help define whether defects identified by RIPA lie in VWF or in GPIbα-V-IX...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804848/laboratory-testing-for-von-willebrand-factor-factor-viii-binding-for-2n-vwd
#6
Soma Mohammed, Emmanuel J Favaloro
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes a protocol for assessment of VWF activity by means of VWF: factor VIII binding (VWF:FVIIIB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804847/laboratory-testing-for-von-willebrand-factor-activity-by-glycoprotein-ib-binding-assays-vwf-gpib
#7
Jürgen Patzke, Emmanuel J Favaloro
In addition to assessment of von Willebrand factor (VWF) antigen (VWF:Ag), the first-line laboratory investigation of possible von Willebrand disease (VWD) often includes an assay to measure GPIb (glycoprotein Ib) binding activity of VWF. A decreased GPIb binding activity is characteristic for most of the VWD types. For many years, the most frequently used assay for measuring GPIb binding activity was the ristocetin cofactor assay (VWF:RCo), which measures the agglutination of fixed human platelets by VWF in the presence of ristocetin...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804846/laboratory-testing-for-von-willebrand-factor-ristocetin-cofactor-vwf-rco
#8
Soma Mohammed, Emmanuel J Favaloro
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for these VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes several protocols for assessment of VWF activity by means of VWF ristocetin cofactor (VWF:RCo)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804845/laboratory-testing-for-von-willebrand-factor-collagen-binding-vwf-cb
#9
Emmanuel J Favaloro, Soma Mohammed
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. This chapter describes several protocols for assessment of VWF activity by means of VWF collagen binding (VWF:CB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804844/laboratory-testing-for-von-willebrand-factor-antigen-vwf-ag
#10
Emmanuel J Favaloro, Soma Mohammed, Jürgen Patzke
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders arise due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes protocols for assessment of VWF level by means of VWF antigen (VWF:Ag)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804843/diagnosis-or-exclusion-of-von-willebrand-disease-using-laboratory-testing
#11
Emmanuel J Favaloro
von Willebrand disease (VWD) is a common bleeding disorder diagnosed based on clinical features and following laboratory testing. VWD is due to deficiencies or defects in the plasma protein von Willebrand factor (VWF), a large adhesive protein with multiple activities. Laboratory testing therefore centers on assessment of VWF protein level using VWF antigen (VWF:Ag), as well as assays that measure VWF activity, most notably platelet glycoprotein (GP) Ib and collagen binding (VWF:CB) activities. Decreases in VWF:Ag and VWF activities, as well as the pattern of such changes, help define VWD and its type...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804817/the-interface-between-immunotransfusion-and-hemostasis-and-thrombosis-testing
#12
Massimo Franchini
Increasing evidence supports the existence of a close relationship between immunotransfusion, hemostasis, and thrombosis. The best example of such linkage is given by the influence of the ABO blood group antigens on von Willebrand factor (VWF) plasma levels and activity. It is well known, for instance, that individuals with non-O blood type (i.e., A, B, and AB) have higher VWF and factor VIII plasma levels than O blood type subjects and are consequently exposed to an increased thrombotic risk. There is also a close relationship between immunotransfusion, hemostasis, and thrombosis testing...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28802375/post-tonsillectomy-hemorrhagic-outcomes-in-children-with-bleeding-disorders-at-a-single-institution
#13
Priyesh N Patel, Alexandra M Arambula, Allison P Wheeler, Edward B Penn
OBJECTIVE: To report on the post-tonsillectomy bleeding outcomes and factors associated with hemorrhage among children with pre- or post-operatively diagnosed bleeding disorders treated with an institutional protocol. METHODS: Retrospective cohort study of patients with hematologic disorders who underwent tonsillectomy between 2003 and 2016 and were treated with perioperative desmopressin or factor replacement and/or aminocaproic acid. Postoperative outcomes were compared to controls matched for age, sex, and indication for surgery...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28800962/contacting-co-culture-of-human-retinal-microvascular-endothelial-cells-alters-barrier-function-of-human-embryonic-stem-cell-derived-retinal-pigment-epithelial-cells
#14
H Skottman, J Muranen, H Lähdekorpi, E Pajula, K Mäkelä, L Koivusalo, A Koistinen, H Uusitalo, K Kaarniranta, K Juuti-Uusitalo
Here we evaluated the effects of human retinal microvascular endothelial cells (hREC) on mature human embryonic stem cell (hESC) derived retinal pigment epithelial (RPE) cells. The hESC-RPE cells (Regea08/017, Regea08/023 or Regea11/013) and hREC (ACBRI 181) were co-cultured on opposite sides of transparent membranes for up to six weeks. Thereafter barrier function, small molecule permeability, localization of RPE and endothelial cell marker proteins, cellular fine structure, and growth factor secretion of were evaluated...
August 8, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28800323/platelet-activation-and-erythrocyte-lysis-during-brief-exposure-of-blood-to-pathophysiological-shear-stress-in-vitro
#15
Suvankar Majumdar, Chetan N Patil, Tammy Ladner-Threadgill, Erica Randolph, Greg W Burgreen, John C Kermode
BACKGROUND: Interaction of von Willebrand factor (VWF) with circulating platelets is the trigger for thrombosis in a region of arterial stenosis. These events are typically studied in vitro under conditions where platelets adhere to a VWF-coated surface. Our approach assesses platelet responses in the absence of adhesion. OBJECTIVE: To characterize extent of platelet activation and erythrocyte lysis in an artificial stenosis model. METHODS: Whole blood is perfused through a length of polyetheretherketone tubing that includes an artificial stenosis, comprising narrow-bore (89-381 μm) tubing...
August 11, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28800150/heterozygosity-for-p2y12-receptor-gene-mutation-associated-with-postoperative-hemorrhage-in-a-greater-swiss-mountain-dog
#16
Rebecca S Flores, Mary K Boudreaux, Barbara Vasquez, Poppy Bristow, Lillian R Aronson, Kari Santoro-Beer, Mary Beth Callan
A 3-year-old, female Greater Swiss Mountain dog developed a hemoperitoneum following an exploratory laparotomy and ovariohysterectomy. Platelet count, PT, APTT, and plasma von Willebrand factor antigen concentration were within RIs. A buccal mucosal bleeding time (BMBT) was prolonged. Given the probability of a hereditary thrombopathia, the dog was administered desmopressin, fresh platelet transfusions, and aminocaproic acid to control hemorrhage. Subsequently, DNA testing for the P2Y12 receptor gene mutation identified the dog as being a heterozygote (carrier)...
August 11, 2017: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/28796276/colistin-dampens-fibrinolysis-and-endothelial-activation-during-endotoxaemia-a-randomised-double-blind-trial
#17
Christian Schoergenhofer, Peter Matzneller, Marion Mußbacher, Johannes A Schmid, Petra Jilma-Stohlawetz, Markus Zeitlinger, Bernd Jilma
Colistin electrostatically interacts with lipopolysaccharides (LPS). Pre-clinical studies demonstrated beneficial effects of colistin on LPS-induced coagulation and fibrinolysis. The objective of this trial was to investigate the effects of colistin during experimental endotoxaemia. In this randomised, double-blind, placebo-controlled, crossover trial 16 healthy volunteers received a 2 ng/kg LPS bolus after infusion of 2.5 million IU colistin or placebo. Plasma levels of F1+2 prothrombin fragments, thrombin-antithrombin complexes (TAT), von Willebrand factor antigen levels (vWF), E-selectin, plasmin-antiplasmin complexes (PAP), tissue-type plasminogen activator (t-PA) antigen and activity, plasminogen activator inhibitor-1 (PAI-1) were measured...
August 10, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28791655/current-and-emerging-options-for-the-management-of-inherited-von-willebrand-disease
#18
REVIEW
Jessica M Heijdra, Marjon H Cnossen, Frank W G Leebeek
Von Willebrand disease (VWD) is the most common inherited bleeding disorder with an estimated prevalence of ~1% and clinically relevant bleeding symptoms in approximately 1:10,000 individuals. VWD is caused by a deficiency and/or defect of von Willebrand factor (VWF). The most common symptoms are mucocutaneous bleeding, hematomas, and bleeding after trauma or surgery. For decades, treatment to prevent or treat bleeding has consisted of desmopressin in milder cases and of replacement therapy with plasma-derived concentrates containing VWF and Factor VIII (FVIII) in more severe cases...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28789675/life-threatening-subdural-hematoma-after-aortic-valve-replacement-in-a-patient-with-heyde-syndrome-a-case-report
#19
Tetsuro Uchida, Azumi Hamasaki, Eiichi Ohba, Atsushi Yamashita, Jun Hayashi, Mitsuaki Sadahiro
BACKGROUND: Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding...
August 8, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28775254/adamts-13-regulates-neutrophil-recruitment-in-a-mouse-model-of-invasive-pulmonary-aspergillosis
#20
Astrid Alflen, Steve Prüfer, Katharina Ebner, Sebastian Reuter, Pamela Aranda Lopez, Inge Scharrer, Fumiaki Banno, Michael Stassen, Hansjörg Schild, Kerstin Jurk, Markus Bosmann, Hendrik Beckert, Markus P Radsak
Von Willebrand factor (VWF) is secreted as an acute phase protein during inflammation. ADAMTS-13 regulates the size and prothrombotic activity of VWF by it's specific proteolytic activity. To determine the relevance of this regulatory pathway for the innate inflammatory response by polymorphonuclear neutrophils (PMN), we employed a mouse model of invasive pulmonary aspergillosis (IPA) where PMN functionality is crucial for fungal clearance and survival. IPA was induced by intratracheal application of Aspergillus fumigatus (A...
August 3, 2017: Scientific Reports
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