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Von willebrands disease

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https://www.readbyqxmd.com/read/28226363/coagulation-profile-in-patients-with-different-etiologies-for-cushing-syndrome-a-prospective-observational-study
#1
Amit Tirosh, Maya Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Richard A Feelders, Constantine A Stratakis
Previous studies reported a higher prevalence of venous-thromboembolic events among patients with Cushing disease (CD) compared to those with ACTH-independent Cushing syndrome (CS) from adrenal sources. The objective of the current study was to evaluate the coagulation profile of patients with CS from different etiologies. A prospective observational study was conducted at a clinical research center. The study included adult patients admitted for evaluation of suspected CS (n=85), that were divided into 3 groups: CD (n=22), ACTH-independent CS from an adrenal tumor/hyperplasia (adrenal CS, n=21), and a control group consisting of subjects with negative screening for CS (rule-out CS, n=42)...
February 22, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28220580/molecular-diagnosis-of-von-willebrand-disease
#2
REVIEW
L Baronciani, A Goodeve, F Peyvandi
The role of molecular characterization in the diagnosis of von Willebrand disease (VWD) is not essential if the patients have been extensively investigated using phenotypic analysis. On the other hand, if some of these phenotype assays are not available, the identification of the mutation causing the disease could be crucial for an accurate diagnosis. Nevertheless, there are several reasons for performing molecular analysis in patients phenotypically well characterized, e.g. to identify the mutation causing VWD can be useful for patients and their family members when prenatal diagnosis is required (type 3 or severe type 2)...
February 20, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28217887/gastric-antral-vascular-ectasia-in-systemic-sclerosis-where-do-we-stand
#3
REVIEW
Hala El-Gendy, Kyrillus S Shohdy, Gehad G Maghraby, Kerolos Abadeer, Moustafa Mahmoud
Gastric antral vascular ectasia (GAVE) continues to be a challenge in both diagnosis and treatment. GAVE has a diverse group of associations and presumed causes, including cirrhosis, chronic renal failure and autoimmune connective tissue diseases. However, in most occasions, the management plan of GAVE itself is the same whatever the underlying disease by using Argon plasma coagulation (APC). Herein, we will discuss three cases of systemic sclerosis-associated GAVE presenting with either acute or chronic gastrointestinal bleeding showing variable responses to APC...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28209329/prognostic-factors-of-hemorrhagic-complications-after-oxaliplatin-based-hyperthermic-intraperitoneal-chemotherapy-toward-routine-preoperative-dosage-of-von-willebrand-factor
#4
J-B Delhorme, L Sattler, F Severac, E Triki, I Gross, B Romain, S Rohr, L Grunebaum, C Brigand
BACKGROUND: Oxaliplatin-based hyperthermic intraperitoneal chemotherapy (HIPEC-ox) induces specific morbidity with hemorrhagic complications (HC). The aim of this study was to identify preoperative, intraoperative and postoperative HC predictive factors after HIPEC-ox. METHODS: A prospective single center study that included all consecutive patients treated with curative-intent HIPEC-ox, whatever the origin of peritoneal disease, was conducted. All patients underwent systematic blood tests exploring primary hemostasis and endothelial activation before surgical incision (D0) and on postoperative days 2 (POD2) and 5 (POD5)...
February 2, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28196380/bleeding-scores-for-the-diagnosis-of-von-willebrand-disease
#5
Mackenzie L Bowman, Paula D James
Obtaining a personal history of bleeding is a critical component to the diagnosis of von Willebrand disease (VWD). The collection of this information can be challenging for physicians, however, as the reporting and interpretation of bleeding symptoms is subjective. The need for more precise quantification of bleeding symptoms was recognized and the Vicenza Bleeding Questionnaire was developed in 2005. This questionnaire collects data regarding the presence and severity of bleeding symptoms and generates a bleeding score by summing the severity of all symptoms reported by a patient...
February 14, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28187737/evaluation-of-a-web-based-registry-of-inherited-bleeding-disorders-a-descriptive-study-of-the-brazilian-experience-with-hemovidaweb-coagulopatias
#6
Suely Meireles Rezende, Silvia Helena Lacerda Rodrigues, Kelly Neves Pinheiro Brito, Diego Lima Quintino da Silva, Marcos Lázaro Santo, Bárbara de Jesus Simões, Guilherme Genovez, Helder Teixeira Melo, João Paulo Baccara Araújo, Danila Augusta Accioly Varella Barca
BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28185978/long-term-serial-changes-in-platelet-activation-indices-following-sirolimus-eluting-and-bare-metal-stents-implantation-in-patients-with-stable-coronary-artery-disease
#7
Maria Marketou, George E Kochiadakis, Aikaterini Giaouzaki, Katerini Sfiridaki, Stelios Petousis, Fragiskos Maragoudakis, Konstantinos Roufas, Despoina Vougia, John Logakis, Gregory Chlouverakis, Panos E Vardas
BACKGROUND: Platelet activation is crucial in the development of stent thrombosis following percutaneous coronary intervention (PCI). We carried out a long-term assessment of multiple factors implicated in the thrombotic process and markers of platelet activation, after implantation of sirolimus-eluting stents (SES) in patients with stable coronary artery disease (CAD), and we compared the results with those after bare-metal stent (BMS) implantation. METHODS: Forty-seven consecutive patients, aged <70 years, with severe stenosis (>70% narrowing of the lumen) of one major epicardial coronary artery and stable CAD, underwent successful elective PCI and were randomly allocated to SES (n=25) or BMS (n=22)...
February 6, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28182756/correction-postpartum-hemorrhage-in-women-with-von-willebrand-disease-a-retrospective-observational-study
#8
Igor Govorov, Signe Löfgren, Roza Chaireti, Margareta Holmström, Katarina Bremme, Miriam Mints
[This corrects the article DOI: 10.1371/journal.pone.0164683.].
2017: PloS One
https://www.readbyqxmd.com/read/28181360/screening-of-female-family-members-of-von-willebrand-disease-patients-utility-of-a-modified-screening-tool-in-a-high-risk-population
#9
A S Faiz, A Kaveney, S Guo, S Murphy, C S Philipp
INTRODUCTION: Family members of Von Willebrand disease (VWD) patients may have low levels of VWF without major bleeding episodes and often remain undiagnosed. AIM: The purpose of this study was to assess the utility of a modified Screening Tool in identifying previously untested reproductive age female family members of VWD patients for haemostatic evaluation. METHODS: Ninety-four reproductive age women including 41 previously untested family members of VWD patients, 26 previously diagnosed VWD patients and 27 healthy controls were administered a modified Screening Tool and had blood drawn for CBC, ferritin, and VWF testing...
February 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28155617/von-willebrand-factor-and-cardiovascular-disease-from-a-biochemical-marker-to-an-attractive-therapeutic-target
#10
Felice Gragnano, Enrica Golia, Francesco Natale, Renatomaria Bianchi, Ivana Pariggiano, Mario Crisci, Vincenzo Diana, Fabio Fimiani, Giuseppe Limongelli, Mariagiovanna Russo, Plinio Cirillo, Paolo Calabrò
Despite recent advances, there is still an unmet need in antithrombotic therapy. New drugs have to overcome old targets, looking for new complementary strategies to counteract thrombus formation and propagation. Since its initial recognition in the 50's, von Willebrand Factor (VWF) has proved to be a contributor in clot formation. The contribution of VWF to platelet adhesion and aggregation is pivotal at high shear rates (i.e. microcirculation and critical artery stenosis), where globular-inactive-VWF elongates in a long chain-active conformation...
February 1, 2017: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/28151804/pregnancy-loss-in-women-with-von-willebrand-disease-a-single-center-pilot-study
#11
Leslie Skeith, Natalia Rydz, Maeve O'Beirne, Dawn Goodyear, Haocheng Li, Man-Chiu Poon
The risk of pregnancy loss in von Willebrand disease (VWD) has been inconsistently reported. Von Willebrand factor (VWF) is a known regulator of angiogenesis, so has the potential to affect placental function. We sought to determine the risk of pregnancy loss and placenta-mediated pregnancy complications in women with VWD, compared with women without VWD. Women with VWD followed in the Southern Alberta Rare Blood and Bleeding Disorders Clinic were invited to participate in a questionnaire (February-June 2014)...
February 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28151041/differing-associations-between-a%C3%AE-accumulation-hypoperfusion-blood-brain-barrier-dysfunction-and-loss-of-pdgfrb-pericyte-marker-in-the-precuneus-and-parietal-white-matter-in-alzheimer-s-disease
#12
J Scott Miners, Isabel Schulz, Seth Love
Recent studies implicate loss of pericytes in hypoperfusion and blood-brain barrier (BBB) leakage in Alzheimer's disease (AD). In this study, we have measured levels of the pericyte marker, platelet-derived growth factor receptor-β (PDGFRB), and fibrinogen (to assess blood-brain barrier leakage), and analyzed their relationship to indicators of microvessel density (von Willebrand factor level), ante-mortem oxygenation (myelin-associated glycoprotein:proteolipid protein-1 ratio and vascular endothelial growth factor level), Aβ level and plaque load, in precuneus and underlying white matter from 49 AD to 37 control brains...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28126959/von-willebrand-disease-type-2b
#13
Eric McGinnis, Suzanne M Vercauteren
No abstract text is available yet for this article.
January 26, 2017: Blood
https://www.readbyqxmd.com/read/28122514/indoxyl-sulfate-the-uremic-toxin-linking-hemostatic-system-disturbances-with-the-prevalence-of-cardiovascular-disease-in-patients-with-chronic-kidney-disease
#14
Tomasz W Kamiński, Krystyna Pawlak, Małgorzata Karbowska, Michał Myśliwiec, Dariusz Pawlak
BACKGROUND: During chronic kidney disease progression, kidney-specific risk factors for cardiovascular disease come into play. The present study investigated the impact of indoxyl sulfate, dietary tryptophan-derived uremic toxin, accumulated in the blood of patients with chronic kidney disease on hemostatic parameters, markers of inflammation, oxidative stress and monocyte to macrophage transition. METHODS: Fifty-one CKD patients not undergoing hemodialysis were enrolled in the study...
January 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28120508/validation-of-automated-latex-von-willebrand-factor-activity-assay-in-a-new-prospective-study-including-post-treatment-patient-samples
#15
M Ettel, M A Nardi, L McVoy
INTRODUCTION: Von Willebrand disease (VWD) is the most prevalent inherited bleeding disorder. Diagnosis requires measurement of VWF-platelet binding function, for which VWF ristocetin cofactor activity (VWF:RCo) is the reference method. Recently, an automated latex particle-enhanced immunoturbidimetric von Willebrand factor activity assay (VWF:Ab) has been validated showing superior characteristics. We further validate VWF:Ab in a prospective study including post-treatment patient samples...
January 25, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28118618/management-of-type-2b-von-willebrand-disease-during-pregnancy
#16
David McLaughlin, Ron Kerr
Type 2B von Willebrand disease is a rare bleeding condition resulting in thrombocytopenia and a reduction in large VWF multimers. It usually has an autosomal dominant pattern of inheritance. We report the management of a patient with type 2B von Willebrand disease, whose diagnosis was confirmed by demonstration of a R1306W mutation, through her first pregnancy. The patient's von Willebrand factor (VWF) antigen and VWF ristocetin cofactor levels rose throughout pregnancy, with an associated drop in the platelet count...
January 25, 2017: Acta Haematologica
https://www.readbyqxmd.com/read/28111980/reduced-endothelial-activation-after-exercise-is-associated-with-improved-hba1c-in-patients-with-type-2-diabetes-and-coronary-artery-disease
#17
Rune Byrkjeland, Ida U Njerve, Harald Arnesen, Ingebjørg Seljeflot, Svein Solheim
OBJECTIVE: We have previously reported insignificant changes in HbA1c after exercise in patients with both type 2 diabetes and coronary artery disease. In this study, we investigated the effect of exercise on endothelial function and possible associations between changes in endothelial function and HbA1c. METHODS: Patients with type 2 diabetes and coronary artery disease ( n = 137) were randomised to 12 months exercise or standard follow-up. Endothelial function was assessed by circulating biomarkers (E-selectin, intercellular adhesion molecule-1, vascular cell adhesion molecule-1, von Willebrand factor, tissue plasminogen activator antigen, asymmetric dimethylarginine and L-arginine/asymmetric dimethylarginine ratio)...
January 1, 2017: Diabetes & Vascular Disease Research
https://www.readbyqxmd.com/read/28111840/laboratory-monitoring-of-replacement-therapy-for-major-surgery-in-von-willebrand-disease
#18
REVIEW
P M Mannucci, M Franchini
Von Willebrand disease (VWD) is an inherited haemorrhagic disorder caused by a quantitative or qualitative defect of von Willebrand factor (VWF), a multimeric plasma glycoprotein that plays a key role in platelet adhesion to the subendothelium and acts as a carrier of factor VIII (FVIII) in blood. Patients with VWD experience bleeding symptoms that are mainly localized in mucous membranes and soft tissues, and their severity depends on the degree of the primary reduction in VWF and the secondary deficiency of FVIII in plasma...
January 22, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#19
REVIEW
Paul Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
December 30, 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28103720/crohn-s-disease-and-acquired-von-willebrand-syndrome-a-rare-dangerous-affair
#20
Federico Pasin, Sophie Testa, Pietro Capone, Federico Buffoli, Antonio Cuzzoli, Giovanni Paolo Coppeta, Roberto Grassia
No abstract text is available yet for this article.
January 20, 2017: Scandinavian Journal of Gastroenterology
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