keyword
MENU ▼
Read by QxMD icon Read
search

Willebrand

keyword
https://www.readbyqxmd.com/read/28430802/the-quality-changes-in-fresh-frozen-plasma-of-the-blood-donors-at-high-altitude
#1
Zongkui Wang, Hua Liu, Miaomiao Dou, Xi Du, Jijun Hu, Na Su, Ya Wang, Rong Zhang, Changqing Li
OBJECTIVE: According to the international guidelines, fresh frozen plasma (FFP) is unanimously used to treat coagulation disorders. The quality of FFP is critical for the clinical transfusion. Till now, few studies have integratedly evaluated the differences of FFP from blood donors at between high altitude (HA) and low altitude (LA). Besides, there were no special quality standards for HA FFP in China. MATERIALS AND METHODS: Up to 41 HA (Lhasa, 3700 m) and 46 LA (Chengdu, 500 m) blood donors were included in our study to estimate the differences of FFP from HA and LA blood donors...
2017: PloS One
https://www.readbyqxmd.com/read/28428218/gain-of-function-mutation-in-filamin-a-potentiates-platelet-integrin-%C3%AE-iib%C3%AE-3-activation
#2
Eliane Berrou, Frédéric Adam, Marilyne Lebret, Virginie Planche, Patricia Fergelot, Odile Issertial, Isabelle Coupry, Jean-Claude Bordet, Paquita Nurden, Dominique Bonneau, Estelle Colin, Cyril Goizet, Jean-Philippe Rosa, Marijke Bryckaert
OBJECTIVE: Dominant mutations of the X-linked filamin A (FLNA) gene are responsible for filaminopathies A, which are rare disorders including brain periventricular nodular heterotopia, congenital intestinal pseudo-obstruction, cardiac valves or skeleton malformations, and often macrothrombocytopenia. APPROACH AND RESULTS: We studied a male patient with periventricular nodular heterotopia and congenital intestinal pseudo-obstruction, his unique X-linked FLNA allele carrying a stop codon mutation resulting in a 100-amino acid-long FLNa C-terminal extension (NP_001447...
April 20, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28420732/modulation-of-tmem16a-channel-activity-by-the-von-willebrand-factor-type-a-vwa-domain-of-the-calcium-activated-chloride-channel-regulator-1-clca1
#3
Monica Sala-Rabanal, Zeynep Yurtsever, Kayla N Berry, Colin G Nichols, Tom J Brett
Calcium-activated chloride channels (CaCCs) are key players in transepithelial ion transport and fluid secretion, smooth muscle constriction, neuronal excitability, and cell proliferation. The CaCC regulator 1 (CLCA1) modulates the activity of the CaCC TMEM16A/Anoctamin 1 (ANO1) by directly engaging the channel at the cell surface, but the exact mechanism is unknown. Here, we demonstrate that the von Willebrand factor type A (VWA) domain within the cleaved CLCA1 N-terminal fragment is necessary and sufficient for this interaction...
April 18, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#4
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28415932/the-effects-of-corrective-surgery-on-endothelial-biomarkers-and-anthropometric-data-in-children-with-congenital-heart-disease
#5
Hung-Tao Chung, Yu-Sheng Chang, Sui-Ling Liao, Shen-Hao Lai
Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Demographic, haemodynamic and cardiac ultrasonography data were collected. Blood samples were taken pre-surgery, 24-48 hours post-surgery and again 3-6 months later...
April 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28414126/characterization-of-the-novel-protein-kiaa0564-von-willebrand-domain-containing-protein-8
#6
Moulun Luo, April E Mengos, Wuqiong Ma, Jean Finlayson, Rocio Zapata Bustos, Yuan Xiao Zhu, Chang-Xin Shi, Tianna M Stubblefield, Wayne T Willis, Lawrence J Mandarino
The VWA8 gene was first identified by the Kazusa cDNA project and named KIAA0564. Based on the observation, by similarity, that the protein encoded by KIAA0564 contains a Von Willebrand Factor 8 domain, KIAA0564 was named Von Willebrand Domain-containing Protein 8 (VWA8). The function of VWA8 protein is almost unknown. The purpose of this study was to characterize the tissue distribution, cellular location, and function of VWA8. In mice VWA8 protein was mostly distributed in liver, kidney, heart, pancreas and skeletal muscle, and is present as a long isoform and a shorter splice variant (VWA8a and VWA8b)...
April 13, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28412143/thermo-sensitive-assembly-of-the-biomaterial-rep-reduces-hematoma-volume-following-collagenase-induced-intracerebral-hemorrhage-in-rats
#7
Joohyun Park, Jong Youl Kim, Seong-Kyoon Choi, Jae Young Kim, Jae Hwan Kim, Won Bae Jeon, Jong Eun Lee
Intracerebral hemorrhage (ICH) frequently results in severe disabilities and high mortality. RGD-containing Elastin-Like Polypeptide (REP), a modified Elastin-Like Polypeptide (ELP), is a thermally responsive biopolymer. REP has high affinity for cells and is known to show non-immunotoxicity, -cytotoxicity, and -inflammatory responses. Here we found that administration of REP in the acute phase of the ICH rat model reduced the hematoma volume, decreased the number of activated microglia, attenuated the expression of von Willebrand Factor (vWF), and prevented the leakage of Immunoglobulin G (IgG) into the cerebral parenchyma without any occlusion of intact microvessels...
April 12, 2017: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/28409234/clinical-role-of-von-willebrand-factor-in-acute-ischemic-stroke
#8
Marija Menih, Miljenko Križmarić, Tanja Hojs Fabjan
BACKGROUND: An elevated level of von Willebrand factor (VWF) is associated with an increased risk for coronary heart disease and ischemic stroke. The objective of the study was to determine whether the level of VWF is associated with the cardioembolic subtype of ischemic stroke, stroke severity, and clinical outcome. PATIENTS AND METHODS: In this study 108 patients suffering from acute ischemic stroke (AIS) were included. According to the etiology of the stroke, patients were classified into the subtype of cardioembolic (CE) stroke and the group with non-CE stroke...
April 13, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28405326/von-willebrand-factor-and-alkaline-phosphatase-predict-re-transplantation-free-survival-after-the-first-liver-transplantation
#9
Andreas Wannhoff, Conrad Rauber, Kilian Friedrich, Christian Rupp, Wolfgang Stremmel, Karl Heinz Weiss, Peter Schemmer, Daniel N Gotthardt
BACKGROUND: After liver transplantation (LT), there are liver-related, infectious and cardiovascular complications that contribute to reduced graft survival. These conditions are associated with an increase in the Von Willebrand factor antigen (VWF-Ag), which was previously correlated with survival in cirrhotic patients. OBJECTIVE: Evaluate VWF-Ag as a predictive marker of re-transplantation-free survival in patients after LT. METHODS: We measured VWF-Ag in patients after first LT and then followed them prospectively with regard to the primary endpoint, namely re-transplantation-free survival...
February 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28403644/the-effect-of-basal-insulin-glargine-on-the-fibrinolytic-system-and-von-willebrand-factor-in-people-with-dysglycaemia-and-high-risk-for-cardiovascular-events-swedish-substudy-of-the-outcome-reduction-with-an-initial-glargine-intervention-trial
#10
Aslak Rautio, Kurt Boman, Hertzel C Gerstein, Jenny Hernestål-Boman, Shun Fu Lee, Mona Olofsson, Linda Garcia Mellbin
INTRODUCTION: Fibrinolytic factors, plasminogen activator inhibitor-1, tissue plasminogen activator, tissue plasminogen activator/plasminogen activator-complex and the haemostatic factor von Willebrand factor are known markers of cardiovascular disease. Their plasma levels are adversely affected in patients with dysglycaemia, and glucose normalization with insulin glargine might improve the levels of these factors. METHODS: Prespecified Swedish substudy of the Outcome Reduction with an Initial Glargine Intervention trial (ClinicalTrials...
April 1, 2017: Diabetes & Vascular Disease Research
https://www.readbyqxmd.com/read/28402755/distribution-of-fcrn-across-species-and-tissues
#11
Sari Latvala, Bjoern Jacobsen, Michael B Otteneder, Annika Herrmann, Sven Kronenberg
The neonatal Fc receptor (FcRn) is a major histocompatibility complex class I type molecule that binds to, transports, and recycles immunoglobulin G (IgG) and albumin, thereby protecting them from lysosomal degradation. Therefore, besides the knowledge of FcRn affinity, FcRn protein expression is critical in understanding the pharmacokinetic behavior of Fc-containing biotherapeutics such as monoclonal antibodies. The goal of this investigation was to achieve for the first time a comparative assessment of FcRn distribution across a variety of tissues and species...
April 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28395735/induced-pluripotent-stem-cells-derived-from-bernard-soulier-syndrome-patient-s-peripheral-blood-cells-with-a-p-phe55ser-mutation-in-the-gpix-gene
#12
Lourdes Lopez-Onieva, Mar Lamolda, Rosa Montes, Maria Luisa Lozano, Vicente Vicente, José Rivera, Verónica Ramos-Mejía, Pedro J Real
Bernard Soulier Syndrome (BSS) is a rare autosomal platelet disorder characterized by mutations in the von Willebrand factor platelet receptor complex GPIb-V-IX. In this work we have generated an induced pluripotent stem cell (BSS3-PBMC-iPS4F8) from peripheral blood mononuclear cells of a BSS patient with a p.Phe55Ser mutation in the GPIX gene. Characterization of BSS3-PBMC-iPS4F8 showed that these cells maintained the original mutation present in the BSS patient, expressed pluripotent stem cell markers and were able to differentiate into the three germline layers...
April 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28394285/diagnosis-and-treatment-of-von-willebrand-disease-and-rare-bleeding-disorders
#13
REVIEW
Giancarlo Castaman, Silvia Linari
Along with haemophilia A and B, von Willebrand disease (VWD) and rare bleeding disorders (RBDs) cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF), represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF...
April 10, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28392945/pitfalls-in-interventional-pain-medicine-hyponatremia-after-ddavp-for-a-patient-with-von-willebrand-disease-undergoing-an-epidural-steroid-injection
#14
Talal W Khan, Abdulraheem Yacoub
Desmopressin (DDAVP), a synthetic analog of vasopressin, has been used in patients with von Willebrand disease (VWD), mild hemophilia A, and platelet dysfunction to reduce the risk of bleeding associated with surgical and interventional procedures. We report the case of a patient with VWD presenting with a bulging disc and radicular pain that underwent transforaminal epidural steroid injections. Her course was complicated with the interval development of headaches and dizziness symptomatic of moderate hyponatremia, likely due to excessive fluid intake...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28391888/inflammatory-markers-modify-the-risk-of-recurrent-coronary-events-associated-with-apolipoprotein-a-i-in-postinfarction-patients
#15
Meng Wang, James Corsetti, Scott McNitt, David Q Rich, Charles E Sparks, Arthur J Moss, Wojciech Zareba
BACKGROUND: Laboratory findings have suggested that systemic and vascular inflammation can impair the antiatherogenic function of high-density lipoproteins (HDLs). However, evidence from population studies is sparse. OBJECTIVE: The objective of the study was to assess if blood inflammatory markers modify the risk of recurrent coronary events associated with apolipoprotein A-I (apoA-I) and HDL cholesterol (HDL-C) among postinfarction patients. METHODS: ApoA-I, HDL-C, and inflammatory markers (C-reactive protein [CRP], serum amyloid A (SAA), fibrinogen, von Willebrand factor [vWF], and D-dimer) were measured from blood samples of 1028 patients drawn 2 months after an index myocardial infarction (MI)...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28388959/autosomal-recessive-inherited-bleeding-disorders-in-pakistan-a-cross-sectional-study-from-selected-regions
#16
Arshi Naz, Muhammad Younus Jamal, Samina Amanat, Ikram Din Ujjan, Akber Najmuddin, Humayun Patel, Fazle Raziq, Nisar Ahmed, Ayisha Imran, Tahir Sultan Shamsi
BACKGROUND: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured...
April 7, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28388906/predictors-of-quality-of-life-among-adolescents-and-young-adults-with-a-bleeding-disorder
#17
John M McLaughlin, James E Munn, Terry L Anderson, Angela Lambing, Bartholomew Tortella, Michelle L Witkop
BACKGROUND: Health-related quality of life (HRQoL) in adolescents and young adults with bleeding disorders is under-researched. We aimed to describe factors related to HRQoL in adolescents and young adults with hemophilia A or B or von Willebrand disease. METHODS: A convenience sample of volunteers aged 13 to 25 years with hemophilia or von Willebrand disease completed a cross-sectional survey that assessed Physical (PCS) and Mental (MCS) Component Summary scores on the SF-36 questionnaire...
April 7, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28384313/variation-in-home-range-size-of-red-foxes-vulpes-vulpes-along-a-gradient-of-productivity-and-human-landscape-alteration
#18
Zea Walton, Gustaf Samelius, Morten Odden, Tomas Willebrand
Home range size is a fundamental concept for understanding animal dispersion and ecological needs, and it is one of the most commonly reported ecological attributes of free-ranging mammals. Previous studies indicate that red foxes Vulpes vulpes display great variability in home range size. Yet, there has been little consensus regarding the reasons why home range sizes of red foxes vary so extensively. In this study, we examine possible causes of variation in red fox home range sizes using data from 52 GPS collared red foxes from four study areas representing a gradient of landscape productivity and human landscape alteration in Norway and Sweden...
2017: PloS One
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#19
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28382367/von-willebrand-factor-deficiency-leads-to-impaired-blood-flow-recovery-after-ischaemia-in-mice
#20
Margreet R de Vries, Erna A B Peters, Paul H A Quax, A Yaël Nossent
Neovascularisation, i. e. arteriogenesis and angiogenesis, is an inflammatory process. Therefore attraction and extravasation of leukocytes is essential for effective blood flow recovery after ischaemia. Previous studies have shown that von Willebrand factor (VWF) is a negative regulator of angiogenesis. However, it has also been shown that VWF facilitates leukocyte attraction and extravasation. We aimed to investigate the role of VWF in arteriogenesis and angiogenesis during post-ischaemic neovascularisation...
April 6, 2017: Thrombosis and Haemostasis
keyword
keyword
81953
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"