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Von willebrands disease

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https://www.readbyqxmd.com/read/27913547/new-treatment-approaches-to-von-willebrand-disease
#1
Michelle Lavin, James S O'Donnell
von Willebrand disease (VWD) is the commonest inherited bleeding disorder and results from either a quantitative or qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). Recent large cohort studies have significantly enhanced our understanding of the molecular mechanisms involved in the pathogenesis of VWD. In contrast, however, there have been relatively few advances in the therapeutic options available for the treatment of bleeding in patients with VWD. Established treatment options include tranexamic acid, 1-deamino-8-d-arginine vasopressin (DDAVP), and plasma-derived VWF concentrates...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913546/diagnosing-von-willebrand-disease-genetic-analysis
#2
Anne Goodeve
Investigation of a patient with possible von Willebrand disease (VWD) includes a range of phenotypic analyses. Often, this is sufficient to discern disease type, and this will suggest relevant treatment. However, for some patients, phenotypic analysis does not sufficiently explain the patient's disorder, and for this group, genetic analysis can aid diagnosis of disease type. Polymerase chain reaction and Sanger sequencing have been mainstays of genetic analysis for several years. More recently, next-generation sequencing has become available, with the advantage that several genes can be simultaneously analyzed where necessary, eg, for discrimination of possible type 2N VWD or mild hemophilia A...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913545/what-have-we-learned-from-large-population-studies-of-von-willebrand-disease
#3
Robert R Montgomery, Veronica H Flood
Von Willebrand factor (VWF) is a critical regulator of hemostatic processes, including collagen binding, platelet adhesion, and platelet aggregation. It also serves as a carrier protein to normalize plasma factor VIII synthesis, release, and survival. While VWF protein measurements by immunoassay are reasonably comparable between institutions, the measurement of VWF ristocetin cofactor activity (VWF:RCo) has significant variability. Other tests of VWF function, including collagen binding or platelet glycoprotein IIb-IIIa binding, are not universally available, yet these functional defects may cause major bleeding even with normal VWF antigen (VWF:Ag) and VWF:RCo assays...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27889551/endothelial-dysfunction-and-cardiovascular-risk-factors-in-childhood-acute-lymphoblastic-leukemia-survivors
#4
Paola Giordano, Paola Muggeo, Maurizio Delvecchio, Santa Carbonara, Alberto Romano, Maria Altomare, Gabriella Ricci, Federica Valente, Annapaola Zito, Pietro Scicchitano, Luciano Cavallo, Marco Matteo Ciccone, Nicola Santoro, Maria Felicia Faienza
BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) survivors have increased risk of obesity, metabolic alterations and cardiovascular disease (CVD). Vascular endothelial function has been studied in adult cancers. Limited data exist regarding CVD risk factors among childhood ALL survivors. We aimed to assess endothelial function, metabolic and cardiovascular risk factors in young survivors of childhood ALL. METHODS: Auxological parameters, blood pressure, glucose, lipid profile, hemostatic markers (total adiponectin and high-molecular-weight subfraction, endothelin-1, von Willebrand factor antigen, thrombin-antithrombin complex, D-dimers, fibrinogen), high sensitive C-reactive protein and ultrasound parameters of endothelial function (flow-mediated dilation-FMD, common carotid intima-media thickness-C-IMT, and antero-posterior diameter of infra-renal abdominal aorta-APAO) were assessed in 52 ALL survivors and 34 sex and age-matched controls...
November 9, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27889474/the-von-willebrand-factor-a1-collagen-iii-interaction-is-independent-of-conformation-and-type-2-von-willebrand-disease-phenotype
#5
Venkata R Machha, Alexander Tischer, Laurie Moon-Tasson, Matthew Auton
The blood von Willebrand factor (VWF) mediates platelet adhesion to injured vessels by sequestering platelets from blood flow and depositing them to collagen and other exposed subendothelial matrix proteins. This process of capturing platelets to facilitate formation of platelet plugs occurs through transient interactions with platelet glycoprotein Ibα via the VWF A1 domain which also binds collagen. Using a conformationally diverse collection of natively folded and mutation-induced misfolded von Willebrand Disease (VWD) variants, we test a recently proposed affinity up-regulation hypothesis which states that collagen binding changes the conformation of the A1 domain to a high affinity GPIbα binding competent state...
November 23, 2016: Journal of Molecular Biology
https://www.readbyqxmd.com/read/27885890/use-of-a-thrombopoietin-receptor-agonist-in-von-willebrand-disease-type-2b-p-v1316m-with-severe-thrombocytopenia-and-intracranial-hemorrhage
#6
O Espitia, C Ternisien, C Agard, P Boisseau, C V Denis, M Fouassier
We present here a 63-year old woman with a long history of immune thrombocytopenia. She was hospitalized for a traumatic intracranial hemorrhage with thrombocytopenia. Following inefficient treatment of four platelet transfusions, immunoglobulins, and corticosteroids, we initiated treatment with a thrombopoietin (TPO) receptor agonist (eltrombopag 25 mg/d) with a good efficacy. Her mother and sister also had chronic thrombocytopenia. Clinical history, hemostasis results, and gene analysis revealed von Willebrand disease (VWD) type 2B with the mutation (c...
November 25, 2016: Platelets
https://www.readbyqxmd.com/read/27882137/von-willebrand-factor-and-coagulation-factor-viii-in-moyamoya-disease-associated-with-graves-disease-a-case-report
#7
Shou-Chen Ren, Bao-Qin Gao, Wei-Li Yang, Wei-Xin Feng, Jian Xu, Shao-Wu Li, Yong-Jun Wang
The present study reported the case of a Chinese boy who was diagnosed with Moyamoya disease (MMD) associated with Graves' disease (GD). An overactivation of von Willebrand factor (vWF) and coagulation factor VIII (FVIII) was identified in the plasma of the patient. Thiamazole and metoprolol treatment was thus administrated. After 2 months of treatment, the patient's thyroid function returned to normal and the neurological symptoms improved gradually. At the same time, the activities of vWF and FVIII were depressed...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27876311/circulating-biomarkers-in-cerebral-autosomal-dominant-arteriopathy-with-subcortical-infarcts-and-leukoencephalopathy-patients
#8
Francesca Pescini, Ida Donnini, Francesca Cesari, Serena Nannucci, Raffaella Valenti, Valentina Rinnoci, Anna Poggesi, Anna Maria Gori, Betti Giusti, Angela Rogolino, Alessandra Carluccio, Silvia Bianchi, Maria Teresa Dotti, Antonio Federico, Maurizio Balestrino, Enrico Adriano, Rosanna Abbate, Domenico Inzitari, Leonardo Pantoni
BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral microangiopathy presenting with variable features, including migraine, psychiatric disorders, stroke, and cognitive decline and variable disability. On neuroimaging, CADASIL is characterized by leukoencephalopathy, multiple lacunar infarcts, and microbleeds. Previous studies suggest a possible role of endothelial impairment in the pathogenesis of the disease...
November 18, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27872729/mild-bleeders-diagnosis-is-elusive-in-large-number-of-patients
#9
Mrinalini Kotru, Deepti Mutereja, Abhishek Purohit, Seema Tyagi, Manoranjan Mahapatra, Renu Saxena, Hara Prasad Pati
BACKGROUND: Bleeding is a common clinical presentation. Even patients with mild bleeding disorders are extensively investigated for ascertaining the cause. The present study was conducted in order to evaluate the extent of the possibility of diagnosis in mild bleeding disorders. MATERIAL AND METHODS: This was a prospective study of patients referred for work up of mild bleeding for a period of 13 months. A complete blood count, peripheral smear examination, Prothrombin time, Partial Thromboplastin time and Thrombin Time, Platelet Aggregometry test, tests for von Willebrand's disease and Platelet Factor 3 availability were measured...
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27868463/postural-changes-in-different-stages-of-schizophrenia-is-associated-with-inflammation-and-pain-a-cross-sectional-observational-study
#10
Viviane Batista Cristiano, Michele Fonseca Vieira Szortyka, Maria Inês Lobato, Keila Maria Ceresér, Paulo Belmonte-de-Abreu
OBJECTIVES: To assess the relationship between posture and inflammatory response markers (C-reactive protein [CRP] and von Willebrand factor [vWF]) in schizophrenics. METHODS: Forty patients with stable schizophrenia were divided into early-stage (<10 years since first episode, n = 15) and late-stage (≥10 years since first episode, n = 25) groups. Both groups were compared to controls (n = 26). All participants underwent postural assessment by biophotogrammetry...
November 21, 2016: International Journal of Psychiatry in Clinical Practice
https://www.readbyqxmd.com/read/27865793/multiplexed-mass-spectrometry-monitoring-of-biomarker-candidates-for-osteoarthritis
#11
Patricia Fernández-Puente, Valentina Calamia, Lucía González-Rodríguez, Lucía Lourido, María Camacho-Encina, Natividad Oreiro, Cristina Ruiz-Romero, Francisco J Blanco
: The methods currently available for the diagnosis and monitoring of osteoarthritis (OA) are very limited and lack sensitivity. Being the most prevalent rheumatic disease, one of the most disabling pathologies worldwide and currently untreatable, there is a considerable interest pointed in the verification of specific biological markers for improving its diagnosis and disease progression studies. Considering the remarkable development of targeted proteomics methodologies in the frame of the Human Proteome Project, the aim of this work was to develop and apply a MRM-based method for the multiplexed analysis of a panel of 6 biomarker candidates for OA encoded by the Chromosome 16, and another 8 proteins identified in previous shotgun studies as related with this pathology, in specimens derived from the human joint and serum...
November 16, 2016: Journal of Proteomics
https://www.readbyqxmd.com/read/27863189/hemophilia-in-sports-a-case-report-and-prophylactic-protocol
#12
Mark Maffet, Jimmy Roton
OBJECTIVE:   To describe a successful prophylactic protocol for managing an athlete with hemophilia playing at a high level of contact sports. BACKGROUND:   Published data show that team physicians are not comfortable either treating athletes with bleeding disorders or allowing them to participate in contact sports. Much of the literature historically has recommended against allowing athletes with bleeding disorders to play sports at all and certainly against playing contact sports...
November 18, 2016: Journal of Athletic Training
https://www.readbyqxmd.com/read/27856811/increased-heart-rate-is-associated-with-a-prothrombotic-state-the-framingham-heart-study
#13
Geoffrey H Tofler, Joseph Massaro, Daniel A Levy, Patrice A Sutherland, Thomas Buckley, Ralph B D'Agostino
BACKGROUND: Although a higher heart rate is associated with an increased risk of cardiovascular disease, the mechanism is not well understood. As thrombosis has an important role in plaque development and acute coronary syndromes, the increase related to heart rate may result from a prothrombotic imbalance. METHODS: We investigated the relation between heart rate and thrombotic potential in 3451 participants from the Offspring Cohort of the Framingham Heart Study (mean age 54 years, 55% women)...
November 17, 2016: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/27851358/1723-acquired-von-willebrand-disease-in-essential-thrombocythemia
#14
Kathryn Kaye Berlin, Patrick Foy
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27848918/development-and-validation-of-technical-assays-of-von-willebrand-disease-in-routine-laboratory
#15
Sara Derdabi, Laurence Rozen, Carole Nagant, Anne Demulder
Von Willebrand disease (MW) is the most common constitutional bleeding disorders. It is caused by a quantitative or qualitative abnormality of the von Willebrand factor (VWF). The laboratory assessment of the disease combines a FVIII assay, and a determination of the antigen and activity of VWF. The analytical validation of VWF:Ag, VWF:Act, vWF:CB is reported in this work and demonstrates good test performance of all three assays, with a coefficient of variation lower than 10% for both the repeatability and reproducibility, stability with a deviation of less than 5% from the target value after six hours at room temperature...
December 1, 2016: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/27834763/acquired-von-willebrand-syndrome-an-under-recognized-cause-of-major-bleeding-in-the-cardiac-intensive-care-unit
#16
Melissa B Jones, Karthik Ramakrishnan, Fahad A Alfares, Kendal M Endicott, Gary Oldenburg, John T Berger, Venkat Shankar, Dilip S Nath, Yaser A Diab
BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27832863/endothelial-progenitor-cells-from-human-fetal-aorta-cure-diabetic-foot-in-a-rat-model
#17
Wan-Ni Zhao, Shi-Qing Xu, Jian-Feng Liang, Liang Peng, Hong-Lin Liu, Zai Wang, Qing Fang, Meng Wang, Wei-Qin Yin, Wen-Jian Zhang, Jin-Ning Lou
OBJECTIVE: Recent evidence has suggested that circulating endothelial progenitor cells (EPCs) can repair the arterial endothelium during vascular injury. However, a reliable source of human EPCs is needed for therapeutic applications. In this study, we isolated human fetal aorta (HFA)-derived EPCs and analyzed the capacity of EPCs to differentiate into endothelial cells. In addition, because microvascular dysfunction is considered to be the major cause of diabetic foot (DF), we investigated whether transplantation of HFA-derived EPCs could treat DF in a rat model...
December 2016: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/27826972/risk-factors-detection-in-chronic-thromboembolic-pulmonary-hypertension-a-tool-for-risk-quantification
#18
M Bohacekova, M Kaldararova, T Valkovicova, A Remkova, J Vesely, I Simkova
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic thromboembolic obstruction in the pulmonary bed. The definitive pathogenesis remains incompletely explained, although multiple risk factors of CTEPH have been identified. The purpose of the study was to evaluate the risk profile of patients with CTEPH and the representativeness of risk factors, identify possible new CTEPH risk factors and specify the epidemiology of CTEPH in our country. METHODS: In 81 patients with CTEPH, well known risk factors were analyzed, and a detailed analysis of selected hematological parameters was investigated at a specialized hematology laboratory...
2016: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/27822882/plasma-von-willebrand-factor-levels-predict-in-hospital-survival-in-patients-with-acute-on-chronic-liver-failure
#19
K S Prasanna, Ashish Goel, G Jayakumar Amirtharaj, Anup Ramachandran, K A Balasubramanian, Ian Mackie, Uday Zachariah, K G Sajith, Elwyn Elias, C E Eapen
BACKGROUND AND AIMS: Circulating levels of von Willebrand factor (vWF) predict mortality in patients with cirrhosis. We hypothesized that systemic inflammation in acute-on-chronic liver failure (ACLF) will stimulate endothelium, increase vWF levels, and promote platelet microthrombi causing organ failure. METHODS: In this prospective study, we correlated plasma vWF levels with organ failure, liver disease severity, sepsis, and systemic inflammatory response syndrome (SIRS) and also analyzed if vWF levels predicted in-hospital composite poor outcome (i...
November 8, 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/27819553/pt-vwd-posing-diagnostic-and-therapeutic-challenges-small-case-series
#20
Analía Sánchez-Luceros, Adriana I Woods, Emilse Bermejo, Shilpa Shukla, Suchitra Acharya, Michelle Lavin, Natalia Rydz, Maha Othman
Despite the increased worldwide awareness, over the last decade, of the platelet-type von Willebrand Disease (PT-VWD), many uncertainties remain around this rare platelet bleeding disorder. This report aims to correctly identify and study the phenotype of new patients and highlights the diagnostic and therapeutic challenges this disease remains to pose. We describe four PT-VWD cases confirmed by genetic analysis in which either the diagnosis and/or the treatment posed challenge. We provide the details of the clinical presentation, laboratory analysis, and the treatment and the responses in each case...
November 7, 2016: Platelets
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