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acquired von willebrand

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https://www.readbyqxmd.com/read/28899852/identification-of-extant-vertebrate-myxine-glutinosa-vwf-evolutionary-conservation-of-primary-hemostasis
#1
Marianne A Grant, David L Beeler, Katherine C Spokes, Junmei Chen, Harita Dharaneeswaran, Tracey E Sciuto, Ann M Dvorak, Gianluca Interlandi, José A Lopez, William C Aird
Hemostasis in vertebrates involves both a cellular and protein component. Previous studies in jawless vertebrates (cyclostomes) suggest that the protein response, which involves thrombin-catalyzed conversion of a soluble plasma protein, fibrinogen, into a polymeric fibrin clot, is conserved in all vertebrates. However, similar data are lacking for the cellular response, which in gnathostomes, is regulated by von Willebrand factor (VWF), a glycoprotein that mediates the adhesion of platelets to the subendothelial matrix of injured blood vessels...
September 12, 2017: Blood
https://www.readbyqxmd.com/read/28883275/successful-management-of-intraperitoneal-bleeding-with-platelet-apheresis-and-von-willebrand-factor-supplementation-in-a-patient-with-essential-thrombocythemia-and-acquired-von-willebrand-syndrome
#2
Toshimitsu Ueki, Kanako Takeshige, Masahiko Sumi, Mayumi Ueno, Naoaki Ichikawa, Hikaru Kobayashi
A 36-year-old woman with essential thrombocythemia (ET) was admitted to our hospital for acute lower abdominal pain. Given no family history of bleeding disorder, she was diagnosed with acquired von Willebrand syndrome. Despite having a medical history of venous thrombosis, she had never been treated for ET because of her preferences. On admission, CT scan revealed massive hemorrhage in the ascending colon with the leakage of a contrast agent. Furthermore, a delayed enhancement of fluid collection in the Douglas fossa followingcontrast CT indicated bloody ascites...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28833767/the-roles-of-mucus-forming-mucins-peritrophins-and-peritrophins-with-mucin-domains-in-the-insect-midgut
#3
R O Dias, C Cardoso, A C Pimentel, T F Damasceno, C Ferreira, W R Terra
Most insects have a gut lined with a peritrophic membrane (PM) consisting of chitin and proteins, mainly peritrophins that have chitin-binding domains. The PM is proposed to originate from mucus-forming mucins (Mf-mucins), which acquired a chitin-binding domain that interlocked with chitin, replacing mucus in function. We evaluated the expression of Mf-mucins and peritrophins by RNA-sequencing (RNA-seq) throughout the midgut of four distantly related insects. Mf-mucins were identified as proteins with high o-glycosylation and a series of uninterrupted Pro/Thr/Ser residues...
August 21, 2017: Insect Molecular Biology
https://www.readbyqxmd.com/read/28833332/pharmacotherapeutic-management-of-gastrointestinal-bleeding-in-patients-with-continuous-flow-left-ventricular-assist-devices
#4
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/28833243/a-novel-homozygous-frameshift-mutation-in-exon-7-of-the-adamts13-gene-in-a-patient-with-congenital-thrombotic-thrombocytopenic-purpura-from-india-a-case-report
#5
Sneha Yadav, Shrimati Shetty, Bipin Kulkarni
BACKGROUND: Thrombotic thrombocytopenia purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by deficiency of ADAMTS13 metalloprotease, which cleaves ultra-large von Willebrand factor into smaller functional units. TTP may be congenital or acquired, and the congenital form is caused by inherited mutations in the ADAMTS13 gene, leading to deficiency of protein or reduced protein activity...
August 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28806755/acquired-von-willebrand-syndrome-in-cardiogenic-shock-patients-on-mechanical-circulatory-microaxial-pump-support
#6
Ulrike Flierl, Jörn Tongers, Dominik Berliner, Jan-Thorben Sieweke, Florian Zauner, Christoph Wingert, Christian Riehle, Johann Bauersachs, Andreas Schäfer
Early use of mechanical circulatory support, e.g. veno-arterial extracorporeal membrane oxygenation (ECMO) or left ventricular unloading by microaxial pump in refractory cardiogenic shock is recommended in current guidelines. Development of acquired von Willebrand Syndrome (AVWS) in patients with left ventricular assist devices (LVADs) and ECMO has been reported. There is an increasing number of patients treated with the Impella® CP microaxial pump for left ventricular unloading. However, the prevalence of AVWS in these high risk patients is unknown and needs to be determined...
2017: PloS One
https://www.readbyqxmd.com/read/28804850/laboratory-testing-for-von-willebrand-factor-multimers
#7
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/28804848/laboratory-testing-for-von-willebrand-factor-factor-viii-binding-for-2n-vwd
#8
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/28804846/laboratory-testing-for-von-willebrand-factor-ristocetin-cofactor-vwf-rco
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/28789675/life-threatening-subdural-hematoma-after-aortic-valve-replacement-in-a-patient-with-heyde-syndrome-a-case-report
#12
Tetsuro Uchida, Azumi Hamasaki, Eiichi Ohba, Atsushi Yamashita, Jun Hayashi, Mitsuaki Sadahiro
BACKGROUND: Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding...
August 8, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28768626/pathophysiology-of-thrombotic-thrombocytopenic-purpura
#13
REVIEW
J Evan Sadler
The discovery of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) revolutionized our approach to thrombotic thrombocytopenic purpura (TTP). Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes, and tissue injury that characterize TTP. Most patients with acquired TTP respond to a combination of plasma exchange and rituximab, but some die or acquire irreversible neurological deficits before they can respond, and relapses can occur unpredictably...
September 7, 2017: Blood
https://www.readbyqxmd.com/read/28765701/levels-and-activities-of-von-willebrand-factor-and-metalloproteinase-with-thrombospondin-type-1-motif-number-13-in-inflammatory-bowel-diseases
#14
Dorota Cibor, Danuta Owczarek, Saulius Butenas, Kinga Salapa, Tomasz Mach, Anetta Undas
AIM: To evaluate the levels of von Willebrand factor (VWF) and metalloproteinase with thrombospondin type-1 motif, number 13 (ADAMTS13) in inflammatory bowel disease (IBD) and correlate them with the disease activity. METHODS: Consecutive patients with IBD aged 18 years or older were enrolled in the study. Forty-seven patients with ulcerative colitis (UC), 38 with Crohn's disease (CD), and 50 healthy controls were included. The white blood cell count, haematocrit, platelet count, fibrinogen, partial activated thromboplastin time, C-reactive protein, albumin, VWF antigen level (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), VWF collagen-binding activity (VWF:CB), and ADAMTS13 antigen level (ADAMTS13:Ag) and activity (ADAMTS13act) were measured...
July 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28759473/novel-therapeutic-approaches-for-thrombotic-thrombocytopenic-purpura
#15
Yvette C Tanhehco, Gowthami Arepally, Ara Metjian
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with therapeutic plasma exchange and immunosuppression with steroids results in high remission and low mortality rates. However, a number of patients remain refractory to frontline therapy and/or experience multiple relapses. This study reviews emerging therapies for thrombotic thrombocytopenic purpura...
July 28, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28756118/von-willebrand-factor-disruption-and-continuous-flow-circulatory-devices
#16
Alastair G Proudfoot, Simon J Davidson, Martin Strueber
Bleeding events remain a significant and frequent complication of continuous-flow left ventricular assist devices (VADs). von Willebrand factor (VWF) is critical to hemostasis by acting as a bridging molecule at sites of vascular injury for normal platelet adhesion as well as promoting platelet aggregation under conditions of high shear. Clinical and experimental data support a role for acquired von Willebrand disease in VAD bleeding episodes caused by shear-induced qualitative defects in VWF. Pathologic shear induces VWF unfolding and proteolysis of large multimers into smaller less hemostatic multimers via ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)...
June 12, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28702343/eb-virus-reactivation-triggers-thrombotic-thrombocytopenic-purpura-in-a-healthy-adult
#17
Satoko Oka, Masaharu Nohgawa
Thrombotic thrombocytopenic purpura (TTP) is rare but life-threatening disease, characterized typically by microangiopathic hemolytic anemia (MAHA), profound peripheral thrombocytopenia and severe deficiency in the von Willebrand factor-cleaving prortease ADAMTS13. It has been reported that acquired immune TTP is closely associated with human immunodeficiency virus infection and influenza infection or vaccination. However, it has not been reported to be associated with Epstein Barr Virus infection or reactivation...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/28679991/acquired-immune-mediated-von-willebrand-syndrome-accompanied-by-antiphospholipid-syndrome
#18
Nobuhiko Kobayashi, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Hideki Uchiumi, Nobuaki Suzuki, Tadashi Matsushita, Akitada Ichinose, Hiroshi Handa
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings resembling those of congenital von Willebrand disease. AvWS usually occurs in association with a variety of underlying disorders, such as lymphoproliferative disease or cardiovascular disease, but autoimmune AvWS is very rare. We now describe the case of a 42-year-old woman with autoimmune AvWS with concurrent antiphospholipid syndrome (APS). The patient was suffering from epistaxis and menorrhagia from few years prior to referral...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28662310/thrombotic-thrombocytopenic-purpura-pathogenesis-diagnosis-and-potential-novel-therapeutics
#19
REVIEW
M Saha, J K McDaniel, X L Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS-13. In general, severe deficiency of plasma ADAMTS-13 activity (< 10 IU dL(-1) ) with or without detectable inhibitory autoantibodies against ADAMTS-13 supports the diagnosis of TTP. A patient usually presents with thrombocytopenia and microangiopathic hemolytic anemia (i.e. schistocytes, elevated serum lactate dehydrogenase, decreased hemoglobin and haptoglobin) without other known etiologies that cause thrombotic microangiopathy (TMA)...
June 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28652649/bleeding-with-the-artificial-heart-gastrointestinal-hemorrhage-in-cf-lvad-patients
#20
EDITORIAL
Grigoriy E Gurvits, Elena Fradkov
Continuous-flow left ventricular assist devices (CF-LVADs) have significantly improved outcomes for patients with end-stage heart failure when used as a bridge to cardiac transplantation or, more recently, as destination therapy. However, its implantations carries a risk of complications including infection, device malfunction, arrhythmias, right ventricular failure, thromboembolic disease, postoperative and nonsurgical bleeding. A significant number of left ventricular assist devices (LVAD) recipients may experience recurrent gastrointestinal hemorrhage, mainly due to combination of antiplatelet and vitamin K antagonist therapy, activation of fibrinolytic pathway, acquired von Willebrand factor deficiency, and tendency to develop small intestinal angiodysplasias due to increased rotary speed of the pump...
June 14, 2017: World Journal of Gastroenterology: WJG
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