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Von willebrands disease

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https://www.readbyqxmd.com/read/29782255/-high-on-treatment-platelet-reactivity-determinants-on-dual-antiplatelet-therapy-in-patients-with-ischemic-heart-disease-before-elective-percutaneous-coronary-intervention
#1
E Z Golukhova, M V Grigoryan, M N Ryabinina, N I Bulaeva
OBJECTIVE: to determine impact of different laboratory and genetic factors on high on-treatment platelet reactivity (HOPR) during dual antiplatelet therapy (DAPT). METHODS: We included in this study 94 patients with stable ischemic heart disease (mean age 59±9.67 years). All patients underwent elective PCI with implantation of drug eluting stents at the background of dual antiplatelet therapy (DAPT) with aspirin and clopidogrel. Platelet reactivity was assessed using light transmission aggregometry with 5 μmol/L ADP (LTA 5ADP) and VerifyNow assay before PCI...
April 2018: Kardiologiia
https://www.readbyqxmd.com/read/29773546/kruse-jarres-r-johnsen-jm-how-i-treat-type-2b-von-willebrand-disease-blood-2018-131-12-1292-1300
#2
(no author information available yet)
No abstract text is available yet for this article.
May 17, 2018: Blood
https://www.readbyqxmd.com/read/29770800/current-and-future-circulating-biomarkers-for-cardiac-amyloidosis
#3
REVIEW
Marco Luciani, Luca Troncone, Federica Del Monte
Cardiac amyloidosis (CA) comprises a heterogeneous group of medical conditions affecting the myocardium. It presents with proteinaceous infiltration with variable degrees of severity, prevalence and evolution. Despite this heterogeneity, erroneous protein folding is the common pathophysiologic process, yielding the formation of a single misfolded protein (monomer) that progressively evolves and ultimately forms amyloid fibers. Additionally, by seeding out from the organs of origin, intermediates called oligomers metastasize and restart the process...
May 17, 2018: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29769259/novel-therapeutics-for-hemophilia-and-other-bleeding-disorders
#4
Michael U Callaghan, Robert Sidonio, Steven W Pipe
Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment for these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase III trial in people with hemophilia A and inhibitors...
May 16, 2018: Blood
https://www.readbyqxmd.com/read/29767844/comorbidities-associated-with-higher-von-willebrand-factor-vwf-levels-may-explain-the-age-related-increase-of-vwf-in-von-willebrand-disease
#5
Ferdows Atiq, Karina Meijer, Jeroen Eikenboom, Karin Fijnvandraat, Eveline P Mauser-Bunschoten, Karin P M van Galen, Marten R Nijziel, Paula F Ypma, Joke de Meris, Britta A P Laros-van Gorkom, Johanna G van der Bom, Moniek P de Maat, Marjon H Cnossen, Frank W G Leebeek
Some comorbidities, such as hypertension, are associated with higher von Willebrand factor (VWF) levels in the general population. No studies have been conducted to assess this association in patients with von Willebrand disease (VWD). Therefore, we studied this association in patients with type 1 (n = 333) and type 2 (n = 203) VWD from the 'WiN" study. VWF antigen (VWF:Ag) was higher in type 1 VWD patients with hypertension [difference: 0·23 iu/ml, 95% confidence interval (CI): 0·11-0·35], diabetes mellitus (0·11 iu/ml, 95% CI: -0·02 to 0·23), cancer (0·14 iu/ml, 95% CI: 0·03-0·25) and thyroid dysfunction (0·14 iu/ml, 95% CI: 0·03-0·26) than in patients without these comorbidities (all corrected for age, sex and blood group)...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29754468/bleeding-score-in-type-1-von-willebrand-disease-patients-using-the-condensed-mcmdm-1-vwd-validated-questionnaire
#6
A Pathare, F Al Hajri, S Al Omrani, N Al Obaidani, B Al Balushi, K Al Falahi
BACKGROUND: Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. AIMS: To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. METHODS: Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator...
May 13, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29750671/successful-treatment-of-severe-menorrhagia-at-menarche-with-recombinant-factor-viia-in-an-adolescent-girl-with-type-iii-von-willebrand-s-disease
#7
Dilek Gurlek Gokcebay, Vildan Culha, Nese Yarali, Namik Yasar Ozbek
: Type III von Willebrand's disease (vWD) is an inherited bleeding disorder, which is frequently associated with menorrhagia in women. Treatment options include antifibrinolytics, desmopressin, von Willebrand factor/factor VIII concentrates and in intractable bleeding circumstances recombinant factor VIIa (rFVIIa). We present an adolescent case with type III vWD who had a menorrhagia at menarche that was refractory to the standard treatment and ultimately was treated with rFVIIa successfully.
May 9, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#8
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29742662/patterns-of-von-willebrand-disease-screening-in-girls-and-adolescents-with-heavy-menstrual-bleeding
#9
Amanda E Jacobson, Sara K Vesely, Terah Koch, Janis Campbell, Sarah H OʼBrien
OBJECTIVE: To estimate the frequency of von Willebrand disease screening and factors that affect screening frequency in a national sample of girls and adolescents with heavy menstrual bleeding. METHODS: In this retrospective cohort study, we used a national claims database for privately and publicly insured patients between 2011 and 2013 for girls aged 10-17 years. Diagnostic criteria of heavy menstrual bleeding were the presence of one inpatient or two outpatient International Classification of Diseases, 9th Revision codes for heavy menstrual bleeding...
May 7, 2018: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29742318/a-comparative-analysis-of-different-automated-von-willebrand-factor-glycoprotein-ib-binding-activity-assays-in-well-typed-von-willebrand-disease-patients
#10
I Vangenechten, K Mayger, P Smejkal, O Zapletal, J J Michiels, G W Moore, A Gadisseur
BACKGROUND: von Willebrand Disease (VWD) is an inherited bleeding disorder caused by quantitative (type 1, 3) or qualitative (type 2) von Willebrand factor (VWF) defect. VWD diagnosis and classification require numerous laboratory tests. VWF: Glycoprotein Ib (GPIb)-binding activity assays are used to distinguish type 1 from type 2 VWD. OBJECTIVES: Three different automated VWF:GPIb-binding activity assays were compared. PATIENTS AND METHODS: BC-VWF:RCo (Siemens Healthcare Diagnostics), HemosIL® VWF:RCo (Instrumentation Laboratory) and INNOVANCE® VWF:Ac (Siemens Healthcare Diagnostics) were performed in a well typed VWD cohort (n=142)...
May 9, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29742079/current-approaches-to-pregnancy-and-childbirth-in-women-with-von-willebrand-disease
#11
Jill M Johnsen
No abstract text is available yet for this article.
April 2018: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29741250/how-i-investigate-for-bleeding-disorders
#12
REVIEW
C P M Hayward
INTRODUCTION: Laboratory investigations for bleeding disorders are warranted when an individual has a personal and/or family history of bleeding, and/or laboratory findings that suggest the possibility of an inherited or acquired bleeding disorder. METHODS: This review summarizes author's experience with ordering and reporting on diagnostic investigations for common and rare bleeding disorders, with consideration of recent articles on diagnosing bleeding disorders...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29734512/correction-of-a-dominant-negative-von-willebrand-factor-multimerization-defect-by-small-interfering-rnas-mediated-allele-specific-inhibition-of-mutant-von-willebrand-factor
#13
A de Jong, R J Dirven, J A Oud, D Tio, B J M van Vlijmen, J Eikenboom
BACKGROUND: Treatment of the bleeding disorder von Willebrand disease (VWD) focusses on increasing von Willebrand factor (VWF) levels through administration of desmopressin or VWF-containing concentrates. Both therapies leave production of mutant VWF unhindered which may have additional consequences, like thrombocytopenia in VWD type 2B, competition between mutant and normal VWF for platelet receptors, and potential development of intestinal angiodysplasia. Most VWD is caused by dominant-negative mutations in VWF and we hypothesize that diminishing expression of mutant VWF positively affects VWD phenotypes...
May 7, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29727892/hemostasis-during-extreme-exertion
#14
Cécile Hélène Kicken, Adam Miszta, Hilde Kelchtermans, Bas De Laat
Exercise is protective against cardiovascular disease, but can also provoke sudden cardiac death, a phenomenon referred to as "the exercise paradox." Extreme exertion is known to induce a rebalanced hemostatic state by causing hypercoagulability and concomitantly enhanced fibrinolysis. Over the past decade, novel techniques for quantifying hemostasis have been introduced, which may provide new insights into this process. This review summarizes recent literature on the effect of extreme exertion of both short and long duration on coagulation, fibrinolysis, and recovery of hemostatic balance...
May 4, 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29727738/differential-sensitivity-of-von-willebrand-factor-activity-assays-to-reduced-vwf-molecular-weight-forms-a-large-international-cross-laboratory-study
#15
Emmanuel J Favaloro, Roslyn Bonar, Martine J Hollestelle, Ian Jennings, Soma Mohammed, Piet Meijer, Timothy Woods, Muriel Meiring
INTRODUCTION: von Willebrand disease (VWD), the most common inherited bleeding disorder, is due to deficiencies/defects in von Willebrand factor (VWF). Effective diagnosis requires testing for FVIII, VWF antigen and one or more VWF 'activity' assays. Classically, 'activity' is assessed using ristocetin cofactor (VWF:RCo), but collagen binding (VWF:CB) and/or other assays are used by many laboratories. This extensive international cross-laboratory study has specifically evaluated contemporary VWF activity assays for comparative sensitivity to reduction in high molecular weight (HMW) VWF, and their ability to differentiate type 1 vs 2A VWD-like samples...
April 19, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29723894/anxiety-related-bleeding-and-thrombosis
#16
Silvia Hoirisch-Clapauch
Anxiety, a normal response to stressful situations, is characterized by increased levels of factor VIII, fibrinogen, and von Willebrand factor, and by enhanced platelet aggregability. One would expect acute anxiety to be a prothrombotic state, but since acute mental stress induces tissue plasminogen activator (tPA) release from endothelial and chromaffin cells, fibrinolysis counteracts procoagulant stimuli. It could be said that procoagulant changes accompanying the fight-or-flight response reduce the risk of bleeding in case of potential injuries, while activation of fibrinolysis counteracts activation of hemostasis to prevent intravascular thrombus formation before injuries occur...
May 3, 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29715362/characterizing-the-heartmate-ii-left-ventricular-assist-device-outflow-using-particle-image-velocimetry
#17
Grant W Rowlands, Bryan C Good, Steven Deutsch, Keefe B Manning
Ventricular assist devices (VADs) are implanted in patients with a diseased ventricle to maintain peripheral perfusion as a bridge-to-transplant or as destination therapy. However, some patients with continuous flow VADs (e.g., HeartMate II (HMII)) have experienced gastrointestinal (GI) bleeding, in part caused by the proteolytic cleavage or mechanical destruction of von Willebrand factor (vWF), a clotting glycoprotein. in vitro studies were performed to measure the flow located within the HMII outlet cannula under both steady and physiological conditions using particle image velocimetry (PIV)...
July 1, 2018: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/29707035/treatment-of-danhong-injection-combined-with-naoxintong-capsule-in-acute-coronary-syndrome-patients-undergoing-pci-operation-study-for-a-randomized-controlled-and-double-blind-trial
#18
Shuai Zhao, Yong Tang, Hairong Cai, Weifeng Liu, Lieyuan Zhang, Dongjie Chen, Bojun Chen
Objective: This double-blind and randomized placebo-controlled trial evaluated the safety and efficacy of Danhong injection combined with Naoxintong capsule in patients with acute coronary syndrome (ACS) after percutaneous coronary intervention (PCI). Methods: ACS patients scheduled to undergo PCI ( n = 130) were equally and randomly apportioned to either a treatment or control group. After PCI, the treatment group received Danhong injection combined with Naoxintong capsule for 12 weeks, while the control group was given placebo...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/29704684/improved-molecular-platform-for-the-gene-therapy-of-rare-diseases-by-liver-protein-secretion
#19
REVIEW
Mickael Quiviger, Aristeidis Giannakopoulos, Sebastien Verhenne, Corinne Marie, Eleana F Stavrou, Karen Vanhoorelbeke, Zsuzsanna Izsvák, Simon F De Meyer, Aglaia Athanassiadou, Daniel Scherman
Many rare monogenic diseases are treated by protein replacement therapy, in which the missing protein is repetitively administered to the patient. However, in several cases, the missing protein is required at a high and sustained level, which renders protein therapy far from being adequate. As an alternative, a gene therapy treatment ensuring a sustained effectiveness would be particularly valuable. Liver is an optimal organ for the secretion and systemic distribution of a therapeutic transgene product. Cutting edge non-viral gene therapy tools were tested in order to produce a high and sustained level of therapeutic protein secretion by the liver using the hydrodynamic delivery technique...
April 25, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29703054/impact-of-von-willebrand-factor-on-coronary-plaque-burden-in-coronary-artery-disease-patients-treated-with-statins
#20
Yuta Kato, Atsushi Iwata, Makito Futami, Motoki Yamashita, Satoshi Imaizumi, Takashi Kuwano, Amane Ike, Makoto Sugihara, Hiroaki Nishikawa, Bo Zhang, Shin'ichiro Yasunaga, Keijiro Saku, Shin-Ichiro Miura
High von Willebrand factor (VWF) levels have been reported to be associated with an increased risk of cardiovascular events. However, the relationship between VWF levels and coronary atherosclerosis in patients with coronary artery disease (CAD) who have already received stain treatment is still unclear. We examined the association between VWF levels and coronary plaque as assessed by intravascular ultrasound (IVUS) in CAD patients treated with statins. Ninety-one CAD patients who underwent percutaneous coronary intervention under IVUS guidance, and who were already receiving statin treatment based on Japanese guidelines, were included...
April 2018: Medicine (Baltimore)
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