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https://www.readbyqxmd.com/read/28453889/discrepant-platelet-and-plasma-von-willebrand-factor-in-von-willebrand-disease-patients-with-p-pro2808leufs-24
#1
Mackenzie L Bowman, Fred G Pluthero, Angie Tuttle, Lara Casey, Ling Li, Hilary Christensen, K Sue Robinson, David Lillicrap, Walter H A Kahr, Paula James
BACKGROUND: A novel variant, p.Pro2808Leufs*24, in the von Willebrand factor (VWF) gene was previously identified in the Canadian von Willebrand disease (VWD) patient population. Clinical observations of type 3 VWD patients with this variant indicate a milder bleeding phenotype compared to other type 3 patients. OBJECTIVE: To assess the effect of the Pro2808Leufs*24 variant on the molecular pathogenesis of VWD and correlate with the phenotype observed in patients...
April 28, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28447419/controversies-in-the-diagnosis-of-type-1-von-willebrand-disease
#2
REVIEW
M L Bowman, P D James
von Willebrand disease (VWD) is the most common bleeding disorder. Type 1 VWD represents the majority of cases and results from a partial quantitative deficiency of von Willebrand factor (VWF). The diagnosis of Type 1 VWD presents many challenges, despite there being three established diagnostic criteria: a personal history of mucocutaneous bleeding, a family history and low VWF levels. These criteria do not always coexist, and there is great overlap of clinical phenotypes and laboratory parameters between healthy individuals and those with VWD...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28436749/differential-diagnosis-of-neonatal-alloimmune-thrombocytopenia-type-2b-von-willebrand-disease
#3
Mathilde Penel-Page, Sandrine Meunier, Mathilde Fretigny, Sandra Le Quellec, Pierre Boisseau, Christine Vinciguerra, Catherine Ternisien, Lucia Rugeri
At birth, severe thrombocytopenia without context of infection should mainly suggest neonatal alloimmune thrombocytopenia (NAIT), especially in case of a platelet count below 20 GL(-1). We report two cases of severe neonatal thrombocytopenia, first suspected as being NAIT. Both had a platelet count below 20 GL(-1) with platelet clumps. The absence of alloantibodies and failure of platelet transfusion and intravenous immunoglobulins to improve the platelet count led to question the diagnosis and to evoke inherited bleeding disorders...
April 24, 2017: Platelets
https://www.readbyqxmd.com/read/28394285/diagnosis-and-treatment-of-von-willebrand-disease-and-rare-bleeding-disorders
#4
REVIEW
Giancarlo Castaman, Silvia Linari
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF...
April 10, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28392945/pitfalls-in-interventional-pain-medicine-hyponatremia-after-ddavp-for-a-patient-with-von-willebrand-disease-undergoing-an-epidural-steroid-injection
#5
Talal W Khan, Abdulraheem Yacoub
Desmopressin (DDAVP), a synthetic analog of vasopressin, has been used in patients with von Willebrand disease (VWD), mild hemophilia A, and platelet dysfunction to reduce the risk of bleeding associated with surgical and interventional procedures. We report the case of a patient with VWD presenting with a bulging disc and radicular pain that underwent transforaminal epidural steroid injections. Her course was complicated with the interval development of headaches and dizziness symptomatic of moderate hyponatremia, likely due to excessive fluid intake...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28388959/autosomal-recessive-inherited-bleeding-disorders-in-pakistan-a-cross-sectional-study-from-selected-regions
#6
Arshi Naz, Muhammad Younus Jamal, Samina Amanat, Ikram Din Ujjan, Akber Najmuddin, Humayun Patel, Fazle Raziq, Nisar Ahmed, Ayisha Imran, Tahir Sultan Shamsi
BACKGROUND: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured...
April 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28379876/recombinant-von-willebrand-factor-for-severe-gastrointestinal-bleeding-unresponsive-to-other-treatments-in-a-patient-with-type-2a-von-willebrand-disease-a-case-report
#7
Racquel Brown
: A recombinant von Willebrand factor (rVWF) was recently approved in the United States for on-demand treatment and control of bleeding episodes in adults with von Willebrand disease (VWD). In contrast to plasma-derived VWF products available in the United States, rVWF does not contain factor VIII (FVIII). To date, there is no published experience of rVWF in clinical practice. We report the acute and prophylactic use of rVWF in a patient with VWD type 2A and severe gastrointestinal bleeding. Dosing with plasma-derived VWF/FVIII concentrates was constrained by FVIII accumulation; the bleeding was unresponsive, and multiple red blood cell transfusions were required...
April 4, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28362648/abnormal-angiogenesis-in-blood-outgrowth-endothelial-cells-derived-from-von-willebrand-disease-patients
#8
Soundarya N Selvam, Lara J Casey, Mackenzie L Bowman, Lindsey G Hawke, Avery J Longmore, Jeffrey Mewburn, Mark L Ormiston, Stephen L Archer, Donald H Maurice, Paula James
Bleeding associated with angiodysplasia is a common, often intractable complication in patients with von Willebrand disease (VWD). von Willebrand factor (VWF), the protein deficient or defective in VWD, is a negative regulator of angiogenesis, which may explain the pathologic blood vessel growth in VWD. This study explores the normal range of angiogenesis in blood outgrowth endothelial cells (BOECs) derived from healthy donors and compares this to angiogenesis in BOECs from VWD patients of all types and subtypes...
March 30, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28351684/tissue-distribution-and-functional-analysis-of-vitellogenin-6-of-toxocara-canis
#9
Hong-Hong Zhu, Guang-Xu Ma, Yong-Fang Luo, Yong-Li Luo, Sha-Sha Yin, Yi Xiong, Rong-Qiong Zhou
Toxocara canis is an common intestinal nematode of canids and the principal causative agent of human toxocariasis. Vitellogenin (Vg), a source of amino acids and lipids in the eggs, are considered to play an important role in embryo development of a wide range of organisms. In the present study, the transcriptional levels of Tc-vit-6 gene in male and female adult T. canis were determined by quantitative real-time PCR, which indicated high transcription of Tc-vit-6 in the intestine, reproductive tract and body wall of male and female adult T...
March 27, 2017: Experimental Parasitology
https://www.readbyqxmd.com/read/28338025/preventing-perioperative-bleeding-in-patients-with-inherited-bleeding-disorders
#10
Colin Watterson, Nicholas Beacher
Data sourcesCochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, a regularly updated database informed by trials identified within electronic databases including MEDLINE. Further defined searches were undertaken in PubMed, Embase, The Cochrane Library, ClinicalTrials.gov and WHO International Clinical Trials Registry Platform. Additional hand searching of relevant journals and books of conference proceedings was undertaken.Study selectionRandomised and quasi-randomised controlled trials in people of all ages with haemophilia or VWD undergoing oral or dental procedures using antifibrinolytic agents (tranexamic acid (TXA) or epsilon aminocaproic acid (EACA)) to prevent perioperative bleeding compared to no intervention with or without placebo...
March 2017: Evidence-based Dentistry
https://www.readbyqxmd.com/read/28335488/italian-registry-of-congenital-bleeding-disorders
#11
Adele Giampaolo, Francesca Abbonizio, Romano Arcieri, Hamisa Jane Hassan
In Italy, the surveillance of people with bleeding disorders is based on the National Registry of Congenital Coagulopathies (NRCC) managed by the Italian National Institute of Health (Istituto Superiore di Sanità). The NRCC collects epidemiological and therapeutic data from the 54 Hemophilia Treatment Centers, members of the Italian Association of Hemophilia Centres (AICE). The number of people identified with bleeding disorders has increased over the years, with the number rising from approx. 7000 in 2000 to over 11,000 in 2015...
March 19, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28306198/plasma-levels-of-plasminogen-activator-inhibitor-1-and-bleeding-phenotype-in-patients-with-von-willebrand-disease
#12
S Abdul, J Boender, J J M C Malfliet, J Eikenboom, K Fijn van Draat, E P Mauser-Bunschoten, K Meijer, J de Meris, B A P Laros-van Gorkom, J G van der Bom, F W G Leebeek, D C Rijken, S Uitte de Willige
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. AIM: To investigate whether plasminogen activator inhibitor-1 (PAI-1) level influences the variation in bleeding tendency in VWD patients...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28279040/-detection-and-analysis-of-plasma-vwf-levels-in-healthy-chinese-by-automatic-standardized-assay
#13
P Zhang, Z Q Yu, X H Zhang, W Zhang, X Bai, L J Cao, J Su, P P Sha, Z Y Wang, C G Ruan
Objective: To explore the normal range of plasma VWF levels of healthy Chinese and to analyze the influencing factors to VWF level. Methods: To detect the levels of von Willebrand factor antigen (VWF∶Ag) , von Willebrand factor ristocetin cofactor activity (VWF∶Rco) , von Willebrand factor collagen binding activity (VWF∶CB) , and the factor Ⅷ coagulation activity (FⅧ∶C) by using fully automatic and standardized testing instruments and matching reagent in 70 healthy Chinese. The effects of age, ABO blood type, gender and region were also analyzed...
February 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28276537/recombinant-von-willebrand-factor-a-first-of-its-kind-product-for-von-willebrand-disease
#14
M Singal, P A Kouides
von Willebrand disease (VWD) is caused by quantitative or qualitative defects in von Willebrand factor (VWF). The mainstay of therapy is desmopressin, which is, however, not useful in certain forms of VWD notwithstanding adverse events. For these patients, plasma-derived factor VIII (pdFVIII)/VWF concentrates have been available for close to three decades but have a theoretical risk of disease transmission, hypersensitivity/allergic reactions, inhibitors and thrombosis. A recombinant VWF (vonicog alfa, Vonvendi™; manufactured by Baxalta, now part of Shire) was approved by the U...
December 2016: Drugs of Today
https://www.readbyqxmd.com/read/28220580/molecular-diagnosis-of-von-willebrand-disease
#15
REVIEW
L Baronciani, A Goodeve, F Peyvandi
The role of molecular characterization in the diagnosis of von Willebrand disease (VWD) is not essential if the patients have been extensively investigated using phenotypic analysis. On the other hand, if some of these phenotype assays are not available, the identification of the mutation causing the disease could be crucial for an accurate diagnosis. Nevertheless, there are several reasons for performing molecular analysis in patients phenotypically well characterized, e.g. to identify the mutation causing VWD can be useful for patients and their family members when prenatal diagnosis is required (type 3 or severe type 2)...
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28218427/two-vitellogenins-in-the-loliginid-squid-uroteuthis-edulis-identification-and-specific-expression-in-ovarian-follicles
#16
Hajime Kitano, Naoki Nagano, Keishi Sakaguchi, Michiya Matsuyama
Vitellogenenesis is a physiological process common in oviparous animals. The molecular profile, modifications, and utilization of vitellogenin (VTG), a precursor of yolk protein, have been characterized in various taxa to understand oogenesis within different modes of reproduction. Hormonal regulation of VTGs has been investigated in invertebrates, such as insects and crustaceans; conversely, little is known for cephalopods. In this study, we isolated two VTG genes (ue-VTG1 and ue-VTG2) from the loliginid swordtip squid, Uroteuthis edulis, via a comprehensive survey of a transcriptome database and subsequent cDNA cloning...
February 20, 2017: Molecular Reproduction and Development
https://www.readbyqxmd.com/read/28196380/bleeding-scores-for-the-diagnosis-of-von-willebrand-disease
#17
Mackenzie L Bowman, Paula D James
Obtaining a personal history of bleeding is a critical component to the diagnosis of von Willebrand disease (VWD). The collection of this information can be challenging for physicians, however, as the reporting and interpretation of bleeding symptoms is subjective. The need for more precise quantification of bleeding symptoms was recognized and the Vicenza Bleeding Questionnaire was developed in 2005. This questionnaire collects data regarding the presence and severity of bleeding symptoms and generates a bleeding score by summing the severity of all symptoms reported by a patient...
February 14, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28191359/simultaneous-determination-of-5-flavonoids-and-7-saponins-for-quality-control-of-traditional-chinese-medicine-preparation-xinnaoshutong-capsule-using-hplc-vwd-elsd
#18
Jin Li, Yang Bai, Peng Zhang, Jun He, Jun Cao, Mingrui An, Li-Min Hu, Xiu-Mei Gao, Yan-Xu Chang
Xinnaoshutong capsule (XC) is a traditional Chinese prescription derived from the ripe fruit of Tribulus terrestris L. (TT). Although XC has long been considered as an important herbal medicine, no analytical method of marker compounds for quality assessment is registered in the Chinese Pharmacopoeia. A simple analytical method of twelve marker components was developed and validated by HPLC-VWD-ELSD method. Chromatographic separation by HPLC was carried out on a Hedera ODS 2 column (4.6 × 250 mm, 5 μm) by gradient elution with acetonitrile-water (0...
2017: Journal of Analytical Methods in Chemistry
https://www.readbyqxmd.com/read/28187737/evaluation-of-a-web-based-registry-of-inherited-bleeding-disorders-a-descriptive-study-of-the-brazilian-experience-with-hemovidaweb-coagulopatias
#19
Suely Meireles Rezende, Silvia Helena Lacerda Rodrigues, Kelly Neves Pinheiro Brito, Diego Lima Quintino da Silva, Marcos Lázaro Santo, Bárbara de Jesus Simões, Guilherme Genovez, Helder Teixeira Melo, João Paulo Baccara Araújo, Danila Augusta Accioly Varella Barca
BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28181360/screening-of-female-family-members-of-von-willebrand-disease-patients-utility-of-a-modified-screening-tool-in-a-high-risk-population
#20
A S Faiz, A Kaveney, S Guo, S Murphy, C S Philipp
INTRODUCTION: Family members of Von Willebrand disease (VWD) patients may have low levels of VWF without major bleeding episodes and often remain undiagnosed. AIM: The purpose of this study was to assess the utility of a modified Screening Tool in identifying previously untested reproductive age female family members of VWD patients for haemostatic evaluation. METHODS: Ninety-four reproductive age women including 41 previously untested family members of VWD patients, 26 previously diagnosed VWD patients and 27 healthy controls were administered a modified Screening Tool and had blood drawn for CBC, ferritin, and VWF testing...
February 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
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