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Von willebrands disease

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https://www.readbyqxmd.com/read/28927148/association-of-abo-blood-groups-with-von-willebrand-factor-factor-viii-and-adamts-13-in-patients-with-lung-cancer
#1
Xia Liu, Xiaogang Chen, Jiezuan Yang, Renyong Guo
Coagulative and fibrinolytic disorders appear to be associated with the development of lung cancer. The aim of the present study was to determine plasma levels of von Willebrand factor (VWF) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13 (ADAMTS-13), and factor VIII (FVIII) activity, in association with O and non-O blood groups in patients with lung cancer. Plasma levels of VWF and ADAMTS-13, and FVIII activity were measured in 115 patients with lung cancer and 98 healthy subjects...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28926986/efficacy-of-octocog-alfa-advate-in-a-child-with-type-3-von-willebrand-disease-and-alloantibodies
#2
Gianluca Sottilotta, Francesca Luise, Elisabetta Massara, Vincenzo Oriana, Angela Piromalli
Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder and is caused by either a quantitative and/or qualitative defect of the multimeric glycoprotein vonWillebrand factor (VWF).[...].
September 18, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28915230/albuminuria-and-endothelial-dysfunction-in-patients-with-non-diabetic-chronic-kidney-disease
#3
Meng-Jie Huang, Ri-Bao Wei, Jing Zhao, Ting-Yu Su, Qing-Ping Li, Xi Yang, Xiang-Mei Chen
BACKGROUND Albuminuria has been associated with cardiovascular events, but whether such an association can be explained by endothelial dysfunction is not fully understood. In this study, we examined the relationship between the urine albumin-to-creatinine ratio (UACR) and biomarkers of endothelial function in patients with chronic kidney disease (CKD). MATERIAL AND METHODS The cross-sectional associations of renal dysfunction and UACR with procoagulant and inflammatory factors were evaluated for 151 consecutive CKD (stage 3-5) patients...
September 15, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28904675/von-willebrand-s-disease-case-report-and-review-of-literature
#4
REVIEW
Hanae Echahdi, Brahim El Hasbaoui, Mohamed El Khorassani, Aomar Agadr, Mohamed Khattab
Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL)...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28885052/migraine-and-vascular-disease-biomarkers-a-population-based-case-control-study
#5
Gretchen E Tietjen, Jagdish Khubchandani, Nabeel Herial, Inge H Palm-Meinders, Hille Koppen, Gisela M Terwindt, Mark A van Buchem, Lenore J Launer, Michel D Ferrari, Mark C Kruit
Background The underpinnings of the migraine-stroke association remain uncertain, but endothelial activation is a potential mechanism. We evaluated the association of migraine and vascular disease biomarkers in a community-based population. Methods Participants (300 women, 117 men) were recruited as a part of the Dutch CAMERA 1 (Cerebral Abnormalities in Migraine, an Epidemiologic Risk Analysis) study. Participants were aged 30-60 (mean 48) years, 155 migraine had with aura (MA), 128 migraine without aura (MO), and 134 were controls with no severe headaches...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/28874064/the-association-of-aging-with-von-willebrand-factor-levels-and-bleeding-risk-in-type-1-von-willebrand-disease
#6
Craig D Seaman, Margaret V Ragni
Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28866059/revascularization-operation-for-moyamoya-disease-with-concurrent-von-willebrand-disease
#7
Kenji Miki, Koichi Arimura, Ataru Nishimura, Koji Yoshimoto, Tetsuro Sayama, Koji Iihara
BACKGROUND: Although extracranial-intracranial (EC-IC) bypass is an effective treatment strategy for symptomatic moyamoya disease, surgeons need to be cautious regarding the possibility of postoperative hemorrhagic complications in patients with a concurrent coagulation disorder. Here, we describe a case of EC-IC bypass for moyamoya disease concurrent with von Willebrand disease type 1. CASE DESCRIPTION: Following perioperative replacement of the von Willebrand factor, the patient showed an uneventful and uncomplicated clinical course...
September 1, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28855217/decreased-severity-of-shiga-toxin-producing-escherichia-coli-haemolytic-uraemic-syndrome-stec-hus-in-a-child-with-type-1-von-willebrand-disease
#8
Matthew A Geramita, Johannes Hofer, James Cooper, Michael L Moritz
Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is characterised by haemolytic anaemia, thrombocytopenia and acute kidney injury. Von Willebrand Factor (vWF) is an important mediator of normal thrombi formation and indirect evidence suggests that vWF may play an important role in Shiga toxin-induced thrombi formation. Clinical evidence supporting the role of vWF in STEC-HUS is lacking. A 10-year-old girl with type 1 von Willebrand Disease (vWD) had a mild case of STEC-HUS, with nadir haemoglobin 7...
August 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28853522/-the-effect-of-vitamin-d-on-the-expression-of-adamts13-in-cultured-endothelial-cells-exposed-to-a-diabetic-like-environment
#9
Keren Cohen-Hagai, Gloria Rashid, Meital Ohana, Sydney Benchetrit, Tali Zitman-Gal
BACKGROUND: Diabetes mellitus (DM) is the leading cause of end-stage kidney disease. Inflammation, fibrosis, coagulability and oxidative stress exacerbate kidney disease. The glycoprotein, Von Willebrand Factor (VWF) has a crucial role in platelet thrombus formation. The enzyme ADAMTS13 is responsible for VWF cleavage. Both are important in the interface between diabetic nephropathy, hypercoagulability and atherosclerotic cardiovascular disease. Vitamin D inhibits endothelial proliferation, blunts angiogenesis and is a cardioprotective agent...
August 2017: Harefuah
https://www.readbyqxmd.com/read/28846462/immunohistochemical-markers-for-prospective-studies-in-neurofibromatosis-1-porcine-models
#10
David K Meyerholz, Georgina K Ofori-Amanfo, Mariah R Leidinger, J Adam Goeken, Rajesh Khanna, Jessica C Sieren, Benjamin W Darbro, Dawn E Quelle, Jill M Weimer
Neurofibromatosis type 1 (NF1) is a common, cancer-predisposing disease caused by mutations in the NF1 tumor gene. Patients with NF1 have an increased risk for benign and malignant tumors of the nervous system (e.g., neurofibromas, malignant peripheral nerve sheath tumors, gliomas) and other tissues (e.g., leukemias, rhabdomyosarcoma, etc.) as well as increased susceptibility to learning disabilities, chronic pain/migraines, hypertension, pigmentary changes, and developmental lesions (e.g., tibial pseudoarthrosis)...
August 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28833332/pharmacotherapeutic-management-of-gastrointestinal-bleeding-in-patients-with-continuous-flow-left-ventricular-assist-devices
#11
Adam Sieg, Jeremy D Moretz, Edward Horn, Douglas L Jennings
Continuous-flow left ventricular assist devices (CF-LVADs) have become an integral component of the management in patients with advanced heart failure, serving as destination therapy or as a bridge to heart transplantation. Despite significant advances in the design and longevity of the device, the ongoing risk for bleeding remains a significant concern. The genesis of gastrointestinal bleeding (GIB) in patients with CF-LVADs is likely multifactorial and may include components of acquired von Willebrand disease, angiodysplasia, and gastrointestinal arteriovenous malformations, as well as additional risk factors such as history of GIB and increased age...
August 22, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28828021/effects-of-endothelial-and-mesenchymal-stem-cells-on-improving-myocardial-function-in-a-sheep-animal-model
#12
Shahram Rabbani, Masoud Soleimani, Mohammad Sahebjam, Mohammad Imani, Seyed Mahdi Nassiri, Amir Atashi, Morteza Daliri Joupari, Ali Ghiaseddin, Mostafa Latifpour, Seyed Hossein Ahmadi Tafti
Background: Myocardial infarction is the main cause of death worldwide. Angiogenesis, a promising new therapy for the treatment of diffuse coronary artery disease, shows a poor response to conventional revascularization techniques. This study focused on improving myocardial function using endothelial cells (ECs) and mesenchymal stem cells (MSCs) in a sheep animal model. Methods: Acute myocardial infarction was induced in 18 sheep (12 treated cases and 6 controls). Autologous MSCs and ECs were injected in the infarcted area and the border zone...
April 2017: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28822718/role-of-the-coagulation-system-in-genitourinary-cancers-review
#13
REVIEW
Axel John, Christian Gorzelanny, Alexander T Bauer, Stefan W Schneider, Christian Bolenz
Tumor progression is associated with aberrant hemostasis, and patients with malignant diseases have an elevated risk of developing thrombosis. A crosstalk among the vascular endothelium, components of the coagulation cascade, and cancer cells transforms the intravascular milieu to a prothrombotic, proinflammatory, and cell-adhesive state. We review the existing evidence on activation of the coagulation system and its implication in genitourinary malignancies and discuss the potential therapeutic benefit of antithrombotic agents...
July 26, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28820407/-evaluation-of-parameters-of-hemostasis-in-the-third-trimester-in-pregnant-women-with-congen%C3%A4-tal-hemorrhagic-diathesis
#14
L Rzaguliyeva, G Afandiyeva, N Malikqasımova
The aim is to study the coagulogram indices in the III trimester of pregnancy in women with hemophilia and von Willebrand disease. Twenty-seven pregnant women with hereditary coagulopathies of mild and moderate form (mean age 27.9±3.3 years) in the third trimester of pregnancy were examined. Of the examined 12 women - carriers of hemophilia A and B, 15 - with Willebrand disease (BV). Clinico-laboratory examination, clinical and biochemical blood tests were carried out. Coagulologic examination was performed on 17 parameters...
July 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28806915/evaluating-the-maintenance-of-disease-associated-variation-at-the-blood-group-related-gene-b4galnt2-in-house-mice
#15
Marie Vallier, Maria Abou Chakra, Laura Hindersin, Miriam Linnenbrink, Arne Traulsen, John F Baines
BACKGROUND: B4galnt2 is a blood group-related glycosyltransferase that displays cis-regulatory variation for its tissue-specific expression patterns in house mice. The wild type allele, found e.g. in the C57BL/6 J strain, directs intestinal expression of B4galnt2, which is the pattern observed among vertebrates, including humans. An alternative allele class found in the RIIIS/J strain and other mice instead drives expression in blood vessels, which leads to a phenotype similar to type 1 von Willebrand disease (VWD), a common human bleeding disorder...
August 14, 2017: BMC Evolutionary Biology
https://www.readbyqxmd.com/read/28806860/patient-caregiver-and-provider-perceptions-of-pain-and-pain-management-in-adolescents-and-young-adults-with-bleeding-disorders
#16
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/28804850/laboratory-testing-for-von-willebrand-factor-multimers
#17
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
#18
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
#19
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
#20
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
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