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https://www.readbyqxmd.com/read/29035661/outcome-of-laparoscopic-ovariohysterectomy-or-ovariectomy-in-dogs-with-von-willebrand-disease-or-factor-vii-deficiency-20-cases-2012-2014
#1
Thomas P Keeshen, J Brad Case, Jeffrey J Runge, Ameet Singh, Philipp D Mayhew, Michele A Steffey, William T N Culp
OBJECTIVE To describe surgical techniques and perioperative management of dogs with von Willebrand disease (VWD) or factor VII (FVII) deficiency undergoing laparoscopic ovariohysterectomy or ovariectomy and evaluate outcomes. DESIGN Retrospective case series. ANIMALS 20 client-owned dogs with VWD (n = 16) or FVII deficiency (4). PROCEDURES Dogs with VWD or FVII deficiency that underwent laparoscopic ovariohysterectomy or ovariectomy between 2012 and 2014 were retrospectively identified via a multi-institutional review of medical records...
November 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/28987708/von-willebrand-disease-mutation-spectrum-and-associated-mutation-mechanisms
#2
REVIEW
Annika de Jong, Jeroen Eikenboom
Von Willebrand disease (VWD) is a bleeding disorder that is mainly caused by mutations in the multimeric protein von Willebrand factor (VWF). These mutations may lead to deficiencies in plasma VWF or dysfunctional VWF. VWF is a heterogeneous protein and over the past three decades, hundreds of VWF mutations have been identified. In this review we have organized all reported mutations, spanning a timeline from the late eighties until early 2017. This resulted in an overview of 750 unique mutations that are divided over the VWD types 1, 2A, 2B, 2M, 2N and 3...
September 23, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28980759/comparison-of-an-automated-chemiluminescent-assay-to-a-manual-elisa-assay-for-determination-of-von-willebrand-factor-collagen-binding-activity-on-vwd-plasma-patients-previously-diagnosed-through-molecular-analysis-of-vwf
#3
E Jousselme, Y Jourdy, L Rugeri, C Négrier, C Nougier
INTRODUCTION: The correct diagnosis and classification of VWD (von Willebrand disease) is crucial and must be optimized by including the collagen binding assay (VWF:CB). VWF:CB remains an under-recognized tool, not fully automated. The objective of this study was to evaluate and to compare the previously evaluated automated chemiluminescent assay (HemosIL AcuStar VWF:CB) to the ELISA ASSERACHROM(®) assay used routinely in our laboratory in patients with molecular diagnosis of VWD. METHODS: A plasma sample from 49 patients previously diagnosed with VWD (type 1; type 2A, type 2M, type 2B) through phenotype and VWF (von Willebrand factor) analysis and 15 healthy controls was analysed...
October 5, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28969551/aptamer-oligonucleotides-as-potential-therapeutics-in-hematologic-diseases
#4
Weibin Li, Meng Zhao, Kaiyu Wang, Huihui Yan, XIaopeng Lan
Aptamers are single-stranded DNA or RNA oligonucleotides generated by a novel in vitro selection technique termed Systematic Evolution of Ligands by Exponential Enrichment (SELEX). During the past two decades, various aptamer drugs have been developed and many of them have entered into clinical trials. In the present review, we focus on aptamers as potential therapeutics for hematological diseases, including anemia of chronic inflammation (ACI) and anemia of chronic disease (ACD), hemophilia, thrombotic thrombocytopenic purpura (TTP) or VWD type-2B, and sickle cell disease (SCD), in particular, those that have entered into clinical trials...
October 2, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28934260/characterization-of-the-nasal-and-oral-microbiota-of-detection-dogs
#5
Anitha Isaiah, Aline Rodrigues Hoffmann, Russ Kelley, Paul Mundell, Jörg M Steiner, Jan S Suchodolski
Little is known about physiological factors that affect the sense of olfaction in dogs. The objectives of this study were to describe the canine nasal and oral microbiota in detection dogs. We sought to determine the bacterial composition of the nasal and oral microbiota of a diverse population of detection canines. Nasal and oral swabs were collected from healthy dogs (n = 81) from four locations-Alabama, Georgia, California, and Texas. Nasal and oral swabs were also collected from a second cohort of detection canines belonging to three different detection job categories: explosive detection dogs (SP-E; n = 22), patrol and narcotics detection dogs (P-NDD; n = 15), and vapor wake dogs (VWD-E; n = 9)...
2017: PloS One
https://www.readbyqxmd.com/read/28926986/efficacy-of-octocog-alfa-advate-in-a-child-with-type-3-von-willebrand-disease-and-alloantibodies
#6
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/28916584/novel-insights-into-the-clinical-phenotype-and-pathophysiology-underlying-low-vwf-levels
#7
Michelle Lavin, Sonia Aguila, Sonja Schneppenheim, Niall Dalton, Kenneth L Jones, Jamie M O'Sullivan, Niamh M O'Connell, Kevin Ryan, Barry White, Mary Byrne, Marie Rafferty, Mairead M Doyle, Margaret Nolan, Roger J S Preston, Ulrich Budde, Paula James, Jorge Di Paola, James S O'Donnell
Critical clinical questions remain unanswered regarding diagnosis and management of patients with Low VWF levels (30-50 IU/dL). To address these questions, the Low VWF Ireland Cohort (LoVIC) study investigated 126 patients registered with Low VWF. Interestingly, despite their marginally reduced plasma VWF levels, ISTH BAT and Condensed MCMDM-1 VWD scores both confirmed significant bleeding phenotypes in the majority of LoVIC patients. For example, among female patients with Low VWF, 77% had ISTH BAT scores ≥6...
September 15, 2017: Blood
https://www.readbyqxmd.com/read/28904675/von-willebrand-s-disease-case-report-and-review-of-literature
#8
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/28874064/the-association-of-aging-with-von-willebrand-factor-levels-and-bleeding-risk-in-type-1-von-willebrand-disease
#9
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
#10
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
#11
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/28806915/evaluating-the-maintenance-of-disease-associated-variation-at-the-blood-group-related-gene-b4galnt2-in-house-mice
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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