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Von willebrands disease

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https://www.readbyqxmd.com/read/29140542/perioperative-von-willebrand-factor-dynamics-are-associated-with-liver-regeneration-and-predict-outcome-after-liver-resection
#1
Patrick Starlinger, David Pereyra, Stefanie Haegele, Paul Braeuer, Lukas Oehlberger, Florian Primavesi, Andreas Kohler, Florian Offensperger, Thomas Reiberger, Arnulf Ferlitsch, Barbara Messner, Guido Beldi, Stefan Staettner, Christine Brostjan, Thomas Gruenberger
Von Willebrand Factor (vWF) was found to mediate platelet influx during the early phase of liver regeneration in mice. Further, increased vWF-antigen (vWF-Ag) levels were shown to be predictive for outcome of patients with chronic liver disease. Accordingly, we aimed to assess the relevance of perioperative vWF-Ag dynamics in terms of liver regeneration and clinical outcome in patients undergoing liver resection (LR). Accordingly, we observed that vWF-Ag and its activity - estimated via ristocetin cofactor measurement - increased immediately after induction of liver regeneration and was associated with platelet accumulation within the liver...
November 15, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29138837/vegf-secreted-by-mesenchymal-stem-cells-mediates-the-differentiation-of-endothelial-progenitor-cells-into-endothelial-cells-via-paracrine-mechanisms
#2
Quanhu Ge, Hongwei Zhang, Jixue Hou, Longfei Wan, Wenzhe Cheng, Xiaoyi Wang, Dan Dong, Congzhe Chen, Jie Xia, Jun Guo, Xueling Chen, Xiangwei Wu
Stem cell therapy is a promising treatment strategy for ischemic diseases. Mesenchymal stem cells (MSCs) and endothelial progenitor cells (EPCs) adhere to each other in the bone marrow cavity and in in vitro cultures. We have previously demonstrated that the adhesion between MSCs and EPCs is critical for MSC self‑renewal and their multi‑differentiation into osteoblasts and chondrocytes. In the present study, the influence of the indirect communication between EPCs and MSCs on the endothelial differentiation potential of EPCs was investigated, and the molecular mechanisms underlying MSC‑mediated EPC differentiation were explored...
November 14, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29133328/associations-of-time-of-day-with-cardiovascular-disease-risk-factors-measured-in-older-men-results-from-the-british-regional-heart-study
#3
Claudio Sartini, Peter H Whincup, S Goya Wannamethee, Barbara J Jefferis, Lucy Lennon, Gordon DO Lowe, Paul Welsh, Naveed Sattar, Richard W Morris
OBJECTIVE: We estimated associations of time of day with cardiovascular disease (CVD) risk factors measured in older men. METHODS: CVD risk factors (markers of inflammation and haemostasis, and cardiac markers) were measured on one occasion between 08:00 and 19:00 hours in 4252 men aged 60-79 years from the British Regional Heart Study. Linear models were used to estimate associations between time of day and risk factors. When an association was found, we examined whether the relationship between risk factors and cardiovascular mortality was affected by the adjustment for time of day using survival analyses...
November 12, 2017: BMJ Open
https://www.readbyqxmd.com/read/29130325/the-plasma-levels-of-adamts-13-von-willebrand-factor-vwfpp-and-fibrin-related-markers-in-patients-with-systemic-sclerosis-having-thrombosis
#4
Koji Habe, Hideo Wada, Ayaka Higashiyama, Tomoko Akeda, Kenshiro Tsuda, Ryoko Mori, Masato Kakeda, Takeshi Matsumoto, Kohshi Ohishi, Keiichi Yamanaka, Naoyuki Katayama, Hitoshi Mizutani
This study aimed to examine the hemostatic abnormalities in patients with systemic sclerosis (SSc) and the relationship between these abnormalities and thrombotic events (THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp), d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. The relationship between their levels and organ involvement, including THEs and interstitial lung disease (ILD), was evaluated...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29119855/two-novel-variants-of-uncertain-significance-in-gp9-associated-with-bernard-soulier-syndrome-are-they-true-mutations
#5
P Boisseau, C Debord, M Eveillard, A Quéméner, M Sigaud, M Giraud, P Talarmain, C Thomas, G Landeau, S Bezieau, B Pan Petesch, M C Béné, M Fouassier
Bernard-Soulier syndrome (BSS) is an autosomal recessive major thrombocytopathy, the symptoms of which are mainly marked by mucocutaneous bleeding. This rare disease, initially described in the 1970s, is the result of an abnormal formation of the glycoprotein complex Ib-IX-V (GP Ib-IX-V), a platelet receptor of von Willebrand factor. A large number of mutations, sometimes involving the GP9 gene, have been described as possibly responsible for the disease. We report here the case of a BSS patient who presented with persistent thrombocytopenia (31x10(9)/L) and decreased surface expression of GPIb-IX-V on large platelets with anisocytosis...
November 9, 2017: Platelets
https://www.readbyqxmd.com/read/29115727/bleeding-score-in-type-1-von-willebrand-disease-patients-using-the-isth-bat-questionnaire
#6
A Pathare, S Al Omrani, F Al Hajri, N Al Obaidani, B Al Balushi, K Al Falahi
BACKGROUND: Bleeding assessment tools have evolved in the last decade to standardize the assessment of the severity of bleeding symptom in a consistent way. In 2010, the International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) was developed and validated. AIMS: Our aim was to administer ISTH-BAT questionnaire to the Omani patients with type 1 VWD and obtain the bleeding score (BS). We also studied the severity of their bleeding symptoms and correlated it with the BS as well as with the laboratory parameters...
November 8, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29115440/aspirin-ameliorates-cerebral-infarction-through-regulation-of-tlr4-nf%C3%A2-%C3%AE%C2%BAb%C3%A2-mediated-endoplasmic-reticulum-stress-in-mouse-model
#7
Xin Wang, Bin Shen, Dezhou Sun, Xiangyu Cui
Cerebral infarction is a cerebrovascular disease caused by local brain ischemic necrosis or softening, which is associated with diabetes, obesity, hypertension and rheumatic heart arrhythmia. Previous studies have indicated that aspirin is a potential oral anticoagulant in the treatment of cerebral ischemic stroke. However, the potential mechanism mediated by aspirin in cerebral infarction therapy is not well understood. The present study analyzed the therapeutic effects of aspirin on cerebral infarction and investigated the underlying molecular mechanism of aspirin‑ameliorated benefits for thrombolysis...
October 25, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29115006/type-2n-von-willebrand-disease-characterization-and-diagnostic-difficulties
#8
A Casonato, E Galletta, L Sarolo, V Daidone
INTRODUCTION: An abnormal factor VIII (FVIII) binding capacity of von Willebrand factor (VWF) identifies type 2N von Willebrand disease (VWD). Type 2N VWD patients are identified by means of the VWF FVIII binding (VWF:FVIIIB) assay, and especially their VWF:FVIIIB/VWF:Ag ratio (VWF:FVIIIB ratio). AIM: We report on our 15-year experience of diagnosing type 2N VWD. METHODS: We have performed 2178 VWF:FVIIIB assays in bleeders and normal subjects...
November 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29114091/circulating-endothelial-markers-in-retinal-vasculopathy-with-cerebral-leukoencephalopathy-and-systemic-manifestations
#9
Nadine Pelzer, Roel Bijkerk, Marlies E J Reinders, Anton Jan van Zonneveld, Michel D Ferrari, Arn M J M van den Maagdenberg, Jeroen Eikenboom, Gisela M Terwindt
BACKGROUND AND PURPOSE-: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a monogenic small vessel disease, caused by C-terminal truncating TREX1 mutations, that can be considered a model for stroke and vascular dementia. The pathophysiology of RVCL-S is largely unknown, but systemic endothelial involvement has been suggested, leading to pathology in the brain and other highly vascularized organs. Here, we investigated circulating endothelial markers to confirm endothelial involvement and identify biomarkers for disease activity...
November 7, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/29108103/adamts13-and-von-willebrand-factor-a-dynamic-duo
#10
REVIEW
Kieron South, David A Lane
VWF is a key player in haemostasis, acting as a carrier for FVIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase, ADAMTS13. Recently, it has been shown that ADAMTS13 undergoes a conformational change upon interaction with VWF and that this enhances its activity towards its substrate. This review summarises recent work on these conformational transitions, describing how they are controlled...
November 6, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29099367/dental-management-of-patients-with-inherited-bleeding-disorders-a-multidisciplinary-approach
#11
Hassan Abed, Abdalrahman Ainousa
Bleeding disorders can be inherited or acquired and demonstrate different levels of severity. Dentists may be called on to treat patients who have bleeding disorders such as hemophilia A and von Willebrand disease (vWD). Dental extraction in any patient with clotting factor defects can result in a delayed bleeding episode. Local hemostatic measures provide effective results in a majority of cases but are insufficient in patients with severe hemophilia A and vWD. Therefore, consultation with the patient's hematologist is required to ensure preoperative prophylactic coverage...
November 2017: General Dentistry
https://www.readbyqxmd.com/read/29098718/outgrowing-the-laboratory-diagnosis-of-type-1-von-willebrand-disease-a-two-decade-study
#12
Mouhamed Yazan Abou-Ismail, Gbolahan O Ogunbayo, Michelle Secic, Peter A Kouides
Von Willebrand Factor (VWF) levels are known to increase with age in the general population, but that effect is unclear in von Willebrand's disease (VWD) patients. Thus, it is important to assess the trends of VWF levels with age, and the extent and rate of their normalization in patients with VWD. In a retrospective cohort study, we reviewed the medical records of 126 patients between 1996 and 2016 who met the NHLBI diagnostic criteria for Type 1 VWD or "Low VWF" (LVWF). We followed all their historically documented VWF antigen (VWF:Ag), VWF activity (VWF:RCo), and Factor VIII (FVIII) levels longitudinally over time, correlating data with clinical setting at time of testing...
November 3, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29093073/midlife-systemic-inflammatory-markers-are-associated-with-late-life-brain-volume-the-aric-study
#13
Keenan A Walker, Ron C Hoogeveen, Aaron R Folsom, Christie M Ballantyne, David S Knopman, B Gwen Windham, Clifford R Jack, Rebecca F Gottesman
OBJECTIVE: To clarify the temporal relationship between systemic inflammation and neurodegeneration, we examined whether a higher level of circulating inflammatory markers during midlife was associated with smaller brain volumes in late life using a large biracial prospective cohort study. METHODS: Plasma levels of systemic inflammatory markers (fibrinogen, albumin, white blood cell count, von Willebrand factor, and Factor VIII) were assessed at baseline in 1,633 participants (mean age 53 [5] years, 60% female, 27% African American) enrolled in the Atherosclerosis Risk in Communities Study...
November 1, 2017: Neurology
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#14
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/29079767/margination-and-stretching-of-von-willebrand-factor-in-the-blood-stream-enable-adhesion
#15
Kathrin Rack, Volker Huck, Masoud Hoore, Dmitry A Fedosov, Stefan W Schneider, Gerhard Gompper
The protein von Willebrand factor (VWF) is essential in primary hemostasis, as it mediates platelet adhesion to vessel walls. VWF retains its compact (globule-like) shape in equilibrium due to internal molecular associations, but is able to stretch when a high enough shear stress is applied. Even though the shear-flow sensitivity of VWF conformation is well accepted, the behavior of VWF under realistic blood flow conditions remains poorly understood. We perform mesoscopic numerical simulations together with microfluidic experiments in order to characterize VWF behavior in blood flow for a wide range of flow-rate and hematocrit conditions...
October 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29078976/abnormal-uterine-bleeding-including-coagulopathies-and-other-menstrual-disorders
#16
REVIEW
Efthimios Deligeoroglou, Vasileios Karountzos
Abnormal Uterine Bleeding (AUB) is a frequent cause of visits to the emergency department and a major reason for concern among adolescents and their families. The most common cause of AUB, in otherwise healthy adolescents, is ovulatory dysfunction, although 5-36% of adolescents who present with heavy menstrual bleeding, have an underlying bleeding disorder (BD). The most common form of BDs is von Willebrand Disease, reflecting 13% of adolescents with AUB. Management of AUB depends on the underlying etiology, the bleeding severity, as well as the need for hospitalization...
October 9, 2017: Best Practice & Research. Clinical Obstetrics & Gynaecology
https://www.readbyqxmd.com/read/29073918/a-first-description-of-the-colombian-national-registry-for-rare-diseases
#17
Heidi Eliana Mateus, Ana María Pérez, Martha Lucía Mesa, Germán Escobar, Jubby Marcela Gálvez, José Ivo Montaño, Martha Lucía Ospina, Paul Laissue
OBJECTIVE: Orphan diseases must be considered a public health concern, underlying country-specific challenges for their accurate and opportune diagnosis, classification and management. Orphan disease registries have not yet been created in South America, a continent having a population of ~ 415 million inhabitants. In Colombia ~ 3 million of patients are affected by rare diseases. The aim of the present study was to establish the first Colombian national registry for rare diseases. The registry was created after the establishment of laws promoting the development of clinical guidelines for diagnosis, management, census and registry of patients suffering rare diseases...
October 26, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29071228/assay-of-desmopressin-acetate-in-nasal-spray-development-of-validated-pre-column-hplc-fluorescence-method
#18
Neeraj Upmanyu, Pawan Kumar Porwal
Purpose: Desmopressin acetate (DDAPV), a synthetic analogue of vasopressin, has been recommended to be used in diabetes insipidus, mild forms of hemophilia and Von Willebrand disease. The DDAPV is available for adminstration via different routes viz. oral, parenteral and nasal, however its dose is very less in case of nasal sprays (20 µg) and parenteral route (4 µg) compared to oral route (0.1 to 0.3 mg in tablet). A sensitive and selective method is needed to be developed and validated for assay of low concentrations of DDAPV in its pharmaceutical dosage form i...
September 2017: Advanced Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/29064615/evaluation-of-a-microfluidic-flow-assay-to-screen-for-von-willebrand-disease-and-low-von-willebrand-factor-levels
#19
Marcus Lehmann, Katrina Ashworth, Marilyn Manco-Johnson, Jorge Di Paola, Keith B Neeves, Christopher J Ng
BACKGROUND: Von Willebrand disease (VWD) is a mucocutaneous bleeding disorder with a reported prevalence of 1 in 10,000. VWF function and platelet adhesion are regulated by hemodynamic forces that are not integrated into most current clinical assays. OBJECTIVE: We evaluated whether a custom microfluidic flow assay (MFA) can screen for deficiencies in VWF in patients presenting with mucocutaneous bleeding. METHODS: Whole blood from individuals with mucocutaneous bleeding was assayed in a custom MFA...
October 24, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29061482/irc2-bf-a-yeast-and-borrelia-responsive-component-of-the-complement-system-from-the-hard-tick-ixodes-ricinus
#20
Veronika Urbanová, Ondřej Hajdušek, Radek Šíma, Zdeněk Franta, Helena Hönig-Mondeková, Lenka Grunclová, Pavla Bartošová-Sojková, Marie Jalovecká, Petr Kopáček
Ticks possess components of a primordial complement system that presumably play a role in the interaction of the tick immune system with tick-borne pathogens and affect their transmission. Here we characterized a novel complement component, tagged as IrC2/Bf, from the hard tick Ixodes ricinus, the principal vector of Lyme disease in Europe. IrC2/Bf is a multi-domain molecule composed of 5-7 CCP modules, varied by alternative splicing, followed by a von Willebrand factor A domain and a C-terminal trypsin-like domain...
October 20, 2017: Developmental and Comparative Immunology
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