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Von willebrands disease

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https://www.readbyqxmd.com/read/28319833/aortic-calcified-particles-modulate-valvular-endothelial-and-interstitial-cells
#1
Nicole C A van Engeland, Sergio Bertazzo, Padmini Sarathchandra, Ann McCormack, Carlijn V C Bouten, Magdi H Yacoub, Adrian H Chester, Najma Latif
BACKGROUND: Normal and calcified human valve cusps, coronary arteries, and aortae harbor spherical calcium phosphate microparticles of identical composition and crystallinity, and their role remains unknown. OBJECTIVE: The objective was to examine the direct effects of isolated calcified particles on human valvular cells. METHOD AND RESULTS: Calcified particles were isolated from healthy and diseased aortae, characterized, quantitated, and applied to valvular endothelial cells (VECs) and interstitial cells (VICs)...
March 3, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28319521/warfarin-and-aspirin-versus-warfarin-alone-for-prevention-of-embolic-events-in-patients-with-a-heartmate-ii%C3%A2-left-ventricular-assist-device
#2
Joseph S Van Tuyl, Ian B Hollis, Khalid A Alburikan, Richard Tran, Brian P Murray, Jo E Rodgers, Jason N Katz, Brett C Sheridan
Acquired von Willebrand disease increases bleeding risk in patients implanted with a continuous-flow left ventricular assist device. Lower aspirin doses decrease the risk of bleeding without an increased risk of embolic events. No published studies in the U.S. have compared the incidence of bleeding and thrombotic events between antithrombotic regimens with and without aspirin. A single-center, retrospective analysis was conducted of adult patients implanted with a HM II. Patients received warfarin and aspirin 81 mg daily or warfarin alone...
March 17, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28306198/plasma-levels-of-plasminogen-activator-inhibitor-1-and-bleeding-phenotype-in-patients-with-von-willebrand-disease
#3
S Abdul, J Boender, J J M C Malfliet, J Eikenboom, K Fijn van Draat, E P Mauser-Bunschoten, K Meijer, J de Meris, B A P Laros-van Gorkom, J G van der Bom, F W G Leebeek, D C Rijken, S Uitte de Willige
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. AIM: To investigate whether plasminogen activator inhibitor-1 (PAI-1) level influences the variation in bleeding tendency in VWD patients...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28306170/impact-of-atrial-fibrillation-on-platelet-gene-expression
#4
Waldemar E Wysokinski, Alfonso Tafur, Naser Ammash, Samuel J Asirvatham, Yanhong Wu, Izabella Gosk-Bierska, Diane E Grill, Joshua P Slusser, Jozef Mruk, Robert D McBane
AIMS: Platelets retain cytoplasmic messenger RNA and are capable of protein biosynthesis. Several diseases are known to impact the platelet transcriptome but the effect of non-valvular atrial fibrillation (NVAF) on platelet RNA transcript is essentially unknown. The aim of this study was to evaluate the impact of NVAF on platelet RNA transcript by measuring platelet genes expression in consecutive NVAF patients before and 3-4 months after pulmonary vein isolation (PVI) and compared to normal sinus rhythm controls (NSR)...
March 17, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#5
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 15, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28280651/prehospital-systolic-hypertension-and-outcomes-in-patients-with-spontaneous-intracerebral-hemorrhage
#6
Stacy Hatcher, Connie Chen, Prasanthi Govindarajan
BACKGROUND: It is well known that hematoma volume and expansion is associated with poor outcomes in patients with spontaneous intracerebral hemorrhage (sICH). The factors associated with hematoma volume and possible expansion include the use of anticoagulant medications, autoimmune or bacterial diseases that reduce platelet production, and genetic defects of Von Willebrand factor causing inhibition or reduction of platelet aggregation. However, little is known about the role of elevated systolic blood pressure (SBP) on hematoma volume and its ultimate role on sICH when identified in the prehospital setting...
January 26, 2017: Curēus
https://www.readbyqxmd.com/read/28276537/recombinant-von-willebrand-factor-a-first-of-its-kind-product-for-von-willebrand-disease
#7
M Singal, P A Kouides
von Willebrand disease (VWD) is caused by quantitative or qualitative defects in von Willebrand factor (VWF). The mainstay of therapy is desmopressin, which is, however, not useful in certain forms of VWD notwithstanding adverse events. For these patients, plasma-derived factor VIII (pdFVIII)/VWF concentrates have been available for close to three decades but have a theoretical risk of disease transmission, hypersensitivity/allergic reactions, inhibitors and thrombosis. A recombinant VWF (vonicog alfa, Vonvendi™; manufactured by Baxalta, now part of Shire) was approved by the U...
December 2016: Drugs of Today
https://www.readbyqxmd.com/read/28256747/elbasvir-grazoprevir-for-patients-with-hepatitis-c-virus-infection-and-inherited-blood-disorders-a-phase-iii-study
#8
Christophe Hézode, Massimo Colombo, Marc Bourlière, Ulrich Spengler, Ziv Ben-Ari, Simone I Strasser, William M Lee, Leslie Morgan, Jingjun Qiu, Peggy Hwang, Michael Robertson, Bach-Yen Nguyen, Eliav Barr, Janice Wahl, Barbara Haber, Robert Chase, Rohit Talwani, Vito Di Marco
Direct-acting antiviral agents have not been studied exclusively in patients with inherited blood disorders and hepatitis C virus (HCV) infection. The objective of the randomized, placebo-controlled, phase III C-EDGE IBLD study was to assess the safety and efficacy of elbasvir/grazoprevir (EBR/GZR) in patients with inherited bleeding disorders and HCV infection. One hundred fifty-nine adults with HCV infection and sickle cell anemia, thalassemia, or hemophilia A/B or von Willebrand disease were enrolled at 31 study sites in the United States, Europe, Australia, Canada, Israel, and Thailand...
March 3, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28249049/platelet-independent-adhesion-of-calcium-loaded-erythrocytes-to-von-willebrand-factor
#9
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage. Adhesion of erythrocytes to vascular endothelial cells has been described in multiple hemolytic disorders, especially in sickle cell disease, but the adhesion of normal erythrocytes to endothelial cells has hardly been described. It was shown that calcium-loaded erythrocytes can adhere to endothelial cells. Because sickle erythrocyte adhesion to ECs can be enhanced by ultra-large von Willebrand factor multimers, we investigated whether calcium loading of erythrocytes could promote binding to endothelial cells via ultra-large von Willebrand factor multimers...
2017: PloS One
https://www.readbyqxmd.com/read/28246490/cystic-adrenal-lesions-focus-on-pediatric-population-a-review
#10
REVIEW
Mara Carsote, Adina Ghemigian, Dana Terzea, Ancuta Augustina Gheorghisan-Galateanu, Ana Valea
BACKGROUND AND AIM: The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. GENERAL DATA: Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/28236847/is-von-willebrand-disease-linked-to-cholesteatoma-aetiology
#11
 Reis Rego, M Santos, M Coutinho, T Feliciano, C Almeida E Sousa
Cholesteatoma is a common clinical picture seen by otolaryngologists. The disease is characterised by an abnormal grow "of skin in the wrong place". Specifically, in acquired cholesteatoma, the main causative issue is associated with ventilatory deficits within the middle ear and results in chronic infection. Molecular science has associated the presence of specific molecules with its development, and moreover recent research suggests that deregulated angiogenesis is a crucial process in the development of cholesteatoma and its recurrence...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28226363/coagulation-profile-in-patients-with-different-etiologies-for-cushing-syndrome-a-prospective-observational-study
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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-elution-and-bare-metal-stent-implantation-in-patients-with-stable-coronary-artery-disease
#18
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 monitored markers of platelet activation after the implantation of sirolimus-eluting stents (SES) in patients with stable coronary artery disease (CAD). Additionally, we compared these findings with those after bare-metal stent (BMS) implantation. METHODS: A cohort of 47 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...
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
#19
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
#20
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
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