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https://www.readbyqxmd.com/read/28640903/type-2b-von-willebrand-disease-with-or-without-large-multimers-a-distinction-of-the-two-sides-of-the-disorder-is-long-overdue
#1
Alessandra Casonato, Viviana Daidone, Eva Galletta, Antonella Bertomoro
Most, but not all patients with type 2B von Willebrand disease (VWD)-which features gain-of-function mutations in the A1 domain of von Willebrand factor (VWF)-have no circulating large VWF multimers. Similarities and differences were analysed in 33 type 2B patients, 12 with a normal and 21 with an abnormal multimer pattern, to see whether they should be considered separately. The minimum aggregating dose of ristocetin was similarly reduced in both patient groups, and modulated by their underlying VWF mutations...
2017: PloS One
https://www.readbyqxmd.com/read/28636092/impact-of-diagnosis-of-von-willebrand-disease-on-patient-outcomes-analysis-of-medical-insurance-claims-data
#2
R F Sidonio, K M Haley, D Fallaize
The inherited bleeding disorder von Willebrand disease (VWD) is challenging to diagnose owing to disease heterogeneity, lack of a definitive laboratory test and variations in diagnostic criteria. We evaluated the impact of diagnosis and diagnostic delay on patient outcomes. The PharMetrics Plus Database was interrogated for medical claims for VWD (ICD-9 286.4) and bleeding events between 1 January 2006 and 30 June 2015. Longitudinal analysis was performed of patients newly diagnosed with VWD (≥9 months' continuous enrolment before first VWD claim) through 24 months following diagnosis...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28634421/the-role-of-von-willebrand-factor-in-vascular-inflammation-from-pathogenesis-to-targeted-therapy
#3
REVIEW
Felice Gragnano, Simona Sperlongano, Enrica Golia, Francesco Natale, Renatomaria Bianchi, Mario Crisci, Fabio Fimiani, Ivana Pariggiano, Vincenzo Diana, Andreina Carbone, Arturo Cesaro, Claudia Concilio, Giuseppe Limongelli, Mariagiovanna Russo, Paolo Calabrò
Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28633902/calcium-sensing-receptor-initiating-cystathionine-gamma-lyase-hydrogen-sulfide-pathway-to-inhibit-platelet-activation-in-hyperhomocysteinemia-rat
#4
Yuwen Wang, Ziqing Zhao, Sa Shi, Fei Gao, Jichao Wu, Shiyun Dong, Weihua Zhang, Yanhong Liu, Xin Zhong
Hyperhomocysteinemia (HHcy, high homocysteine) induces the injury of endothelial cells (ECs). Hydrogen sulfide (H2S) protects ECs and inhibits the activation of platelets. Calcium-sensing receptor (CaSR) regulates the production of endogenous H2S. However, whether CaSR inhibits the injury of ECs and the activation of platelets by regulating the endogenous cystathionine-gamma-lyase (CSE, a major enzyme that produces H2S)/H2S pathway in hyperhomocysteinemia has not been previously investigated. Here, we tested the ultrastructure alterations of ECs and platelets, the changes in the concentration of serum homocysteine and the parameters of blood of hyperhomocysteinemia rats were measured...
June 19, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28628948/platelet-hyperaggregability-is-associated-with-decreased-adamts13-activity-and-enhanced-endotoxemia-in-patients-with-acute-cholangitis
#5
Hiroaki Takaya, Hideto Kawaratani, Takuya Kubo, Kenichiro Seki, Yasuhiko Sawada, Kosuke Kaji, Yasushi Okura, Kosuke Takeda, Mitsuteru Kitade, Kei Moriya, Tadashi Namisaki, Akira Mitoro, Masanori Matsumoto, Hiroshi Fukui, Hitoshi Yoshiji
AIM: Insufficient ADAMTS13 activity (ADAMTS13:AC) leads to increased levels of unusually large von Willebrand factor (VWF) multimers and causes microcirculatory disturbance and multiple organ failure (MOF). Endotoxin (Et) triggers the activation of coagulation and cytokine cascades, leading to MOF in severe inflammatory response syndrome (SIRS). Here, we investigated the potential role of endotoxemia-related ADAMTS13 in acute cholangitis. METHODS: Twenty-four patients with acute cholangitis, including 7 with severe acute cholangitis, were recruited in this study...
June 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28628220/importance-of-endothelial-dysfunction-biomarkers-in-patients-with-crimean-congo-hemorrhagic-fever
#6
Mustafa Arslan, Gürdal Yilmaz, Ahmet Mentese, Hülya Yilmaz, S Caner Karahan, Iftihar Koksal
BACKGROUND: The pathogenesis of the Crimean-Congo hemorrhagic fever (CCHF) and the cause of the hemorrhage are not yet fully understood. However, the endothelium plays a key role in the pathogenesis. The purpose of this study was to investigate endothelial dysfunction markers [asymmetrical dimethyl arginine(ADMA), endothelin 1(ET-1), thrombomodulin(TM), von Willebrand factor(vWf) and intercellular adhesion molecule(ICAM-1)] in serum in patients with CCHF and their associations with hemorrhage...
June 19, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28625868/distinct-characteristics-of-neonatal-platelet-reactivity
#7
REVIEW
Belay Tesfamariam
Platelets undergo a process of developmental maturation, and hence its regulation of vascular integrity and control of hemostasis at various stages of neonatal ages deserves better characterization. Functional assays for platelets require a larger volume of blood than what is feasible to collect in neonates, creating a technical hurdle that has been a challenge to investigate neonatal platelets. For this reason, the current knowledge of neonatal platelet function has been based on studies from cord blood-derived platelets as a surrogate for neonatal peripheral blood...
June 15, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28623399/endothelial-dysfunction-in-rectal-cancer-patients-chronically-exposed-to-ionizing-radiation
#8
Tolebay Rakhypbekov, Ken Inoue, Laura Pak, Noriyuki Kawano, Nobuo Takeichi, Masaharu Hoshi, Yoshihiro Noso, Nailya Chaizhunusova, Zukhra Manambayeva, Arman Khozhayev, Maulen Molgazhdarov, Sayakhat Olzhaev, Sholpan Tokanova, Madina Madiyeva
We sought to identify the features of endothelial function in rectal cancer patients who were exposed to chronic ionizing radiation from a nuclear test site in Kazakhstan. We examined 146 individuals, 76 of whom were rectal cancer patients. The existence of a complex of disturbances of the endothelium and hemostasis systems in patients vs non-patients was revealed. Endothelial dysfunction was expressed as an increase of nitric oxide (NO) production along with decreases in vasodilatation function, and increased levels of von Willebrand factor in blood, along with an increase in the number of circulating endotheliocytes...
June 16, 2017: Radiation and Environmental Biophysics
https://www.readbyqxmd.com/read/28622066/vascular-associated-muc4-vwf-co-localization-in-human-conjunctival-malignant-melanoma-specimens-tumor-metastasis-by-migration
#9
Vinodh Kakkassery, Sibylle Winterhalter, Ann-Christin Nick, Stephanie C Joachim, Antonia M Joussen, Norbert Kociok
PURPOSE: To investigate whether vascular differentiation marker von Willebrand factor (vWf) and proliferation marker KI67 expression correlate with MUC4 localization around stromal tumor vascularization in human conjunctival malignant melanoma (CMM). MATERIALS AND METHODS: For the purposes of this study, we analyzed samples from human CMMs (n = 4), conjunctival compound nevi (n = 7), and samples from healthy conjunctiva (n = 7) for MUC1, 4, and 16 by immunohistochemistry...
June 16, 2017: Current Eye Research
https://www.readbyqxmd.com/read/28619108/scavenging-on-a-pulsed-resource-quality-matters-for-corvids-but-density-for-mammals
#10
Gjermund Gomo, Jenny Mattisson, Bjørn Roar Hagen, Pål Fossland Moa, Tomas Willebrand
BACKGROUND: Human food subsidies can provide predictable food sources in large quantities for wildlife species worldwide. In the boreal forest of Fennoscandia, gut piles from moose (Alces alces) harvest provide a potentially important food source for a range of opportunistically scavenging predators. Increased populations of predators can negatively affect threatened or important game species. As a response to this, restrictions on field dressing of moose are under consideration in parts of Norway...
June 15, 2017: BMC Ecology
https://www.readbyqxmd.com/read/28618874/von-willebrand-factor-multimeric-structure-and-functional-activity-in-patients-with-atrial-fibrillation-with-and-without-oral-anticoagulation
#11
Sandra Lopez-Castaneda, Ignacio Valencia-Hernández, Carlos Arean, Daniel Godínez-Hernández, Martha Eva Viveros-Sandoval
von Willebrand factor (vWF) is a multimeric glycoprotein present in blood plasma. It is synthesized in megakaryocytes and endothelial cells, secreted into circulation in the form of high-molecular-weight multimers (HMWMs), and cleaved into shorter, less active multimers by ADAMTS13. It is essential for platelet adhesion and aggregation. Previous studies have investigated the relationship between vWF levels and thromboembolic events with little regard to vWF multimeric structure. Patients with atrial fibrillation (AF) exhibit higher plasma vWF and lower ADAMTS13 levels...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28612573/-effects-of-danqi-capsule-on-platelet-function-in-healthy-people
#12
Li-Qin Ling, Juan Liao, Chong-Wei Zhang, Qian Niu, Hong Jiang, Jing Zhou
OBJECTIVES: To study the effects of Danqi Capsule on platelet function in healthy people. METHODS: Sixteen healthy volunteer were divided into low dose (4 capsules/time, 3 times/d) and high dose group (6 capsules/time, 3 times/d), and received Danqi Capsule by orally administration for 2 weeks. The venous blood were collect at 3 time points: one week before taking Danqi Capsule (control), one week after taking Danqi Capsule (1 week), two week after taking Danqi Capsule (2 weeks)...
January 2017: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28612396/diagnosis-of-inherited-bleeding-disorders-in-the-genomic-era
#13
REVIEW
Suthesh Sivapalaratnam, Janine Collins, Keith Gomez
Inherited bleeding disorders affect between 1 in 1000 individuals for the most common disorder, von Willebrand Disease, to only 8 reported cases worldwide of alpha-2-antiplasmin deficiency. Those with an identifiable abnormality can be divided into disorders of coagulation factors (87%), platelet count and function (8%) and the fibrinolytic system (3%). Of the patients registered in the UK with a bleeding disorder, the remaining 2% are unclassifiable. In addition to bleeding symptoms, patients with an inherited bleeding disorder can manifest other abnormalities, making an accurate and complete diagnosis that reflects the underlying molecular pathology important...
June 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28610597/anti-connective-tissue-growth-factor-ctgf-ccn2-monoclonal-antibody-attenuates-skin-fibrosis-in-mice-models-of-systemic-sclerosis
#14
Katsunari Makino, Tomoko Makino, Lukasz Stawski, Kenneth E Lipson, Andrew Leask, Maria Trojanowska
BACKGROUND: Systemic sclerosis (SSc) is characterized by fibrosis of the skin and internal organs. Although the involvement of connective tissue growth factor (CTGF/CCN2) has been well-documented in SSc fibrosis, the therapeutic potential of targeting CTGF in SSc has not been fully investigated. Our aim was to examine the therapeutic potential of CTGF blockade in a preclinical model of SSc using two approaches: smooth muscle cell fibroblast-specific deletion of CTGF (CTGF knockout (KO)) or a human anti-CTGF monoclonal antibody, FG-3019...
June 13, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28610577/coronary-angiogenic-effect-of-long-term-administration-of-nigella-sativa
#15
Lubna I Al Asoom
BACKGROUND: Coronary angiogenesis is one of the preferable adaptive responses of aerobic training. Previous studies found inotropic and hypertrophic cardiac effects for long-term administration of Nigella sativa (NS), but no studies have explored its coronary angiogenic effect. The present study compared the effect of long-term NS- administration and exercise training on the induction of coronary angiogenesis. METHOD: Fifteen adult male Wistar rats were divided into three groups: control, NS-fed, and exercise-trained (Ex)...
June 13, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28609796/successful-use-of-a-highly-purified-plasma-von-willebrand-factor-concentrate-containing-little-fviii-for-the-long-term-prophylaxis-of-severe-type-3-von-willebrand-s-disease
#16
Tomas Simurda, Miroslava Dobrotova, Ingrid Skornova, Juraj Sokol, Peter Kubisz, Jan Stasko
No abstract text is available yet for this article.
June 13, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28601338/circulating-platelet-aggregates-damage-endothelial-cells-in-culture
#17
Chandrakala Aluganti Narasimhulu, Mukesh Nandave, Diana Bonilla, Janani Singaravelu, Chittoor B Sai-Sudhakar, Sampath Parthasarathy
BACKGROUND: Presence of circulating endothelial cells (CECs) in systemic circulation may be an indicator of endothelial damage and/or denudation, and the body's response to repair and revascularization. Thus, we hypothesized that aggregated platelets (AgPlts) can disrupt/denude the endothelium and contribute to the presence of CEC and EC-derived particles (ECDP). METHODS: Endothelial cells were grown in glass tubes and tagged with/without 0.5 μm fluorescent beads...
June 1, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28599697/paraneoplastic-cushing-syndrome-due-to-wilm-s-tumor
#18
Mahwish Faizan, Jaida Manzoor, Muhammad Saleem, Saadia Anwar, Qaiser Mehmood, Ambreen Hameed, Agha Shabbir Ali
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28598970/seroprevalence-and-clinical-characteristics-of-viral-hepatitis-in-transfusion-dependent-thalassemia-and-hemophilia-patients
#19
Tyng-Yuan Jang, Pei-Chin Lin, Ching-I Huang, Yu-Mei Liao, Ming-Lun Yeh, Yu-Sheng Zeng, Po-Cheng Liang, Wan-Yi Hsu, Shih-Pien Tsai, Zu-Yau Lin, Shinn-Cherng Chen, Jee-Fu Huang, Chia-Yen Dai, Chung-Feng Huang, Shyh-Shin Chiou, Wan-Long Chuang, Ming-Lung Yu
BACKGROUND/AIMS: Transfusion dependent subjects are at a great risk of viral hepatitis infection. We aimed to evaluate the prevalence and factors associated with hepatitis B virus (HBV) and hepatitis C virus (HCV) infection among transfusion-dependent patients in Taiwan. METHODS: A total of 140 patients (67 thalassemic patients, 70 hemophilic patients, two patients with hereditary spherocytosis and one patient with von Willebrand disease) were prospectively enrolled to evaluate the prevalence and factors associated with viral hepatitis and spontaneous HCV clearance...
2017: PloS One
https://www.readbyqxmd.com/read/28598382/syk-activity-is-dispensable-for-platelet-gp1b-ix-v-signaling
#20
Rachit Badolia, John C Kostyak, Carol Dangelmaier, Satya P Kunapuli
The binding of von Willebrand factor (VWF) to the platelet membrane glycoprotein 1b-IX (GP1b-IX) leads to activation of platelets. GP1b was shown to signal via the FcRγ-ITAM (Fc Receptor γ-Immunoreceptor tyrosine-based activation motif) pathway, activating spleen tyrosine kinase (Syk) and other tyrosine kinases. However, there have been conflicting reports regarding the role of Syk in GP1b signaling. In this study, we sought to resolve these conflicting reports and clarify the role of Syk in VWF-induced platelet activation...
June 9, 2017: International Journal of Molecular Sciences
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