11,624 results match your criteria von Willebrand Disease


Migraine and subclinical atherosclerosis: endothelial dysfunction biomarkers and carotid intima-media thickness: a case-control study.

Neurol Sci 2019 Jan 15. Epub 2019 Jan 15.

Department of Biostatistics, Selcuk University Faculty of Veterinary Medicine, Konya, Turkey.

Background: Migraine is a common neurovascular disease associated with vascular risks, especially in young adult females, but the mechanism underlying these associations remains unknown. This study evaluated the relationships between plasma endothelial dysfunction biomarkers and carotid intima-media thickness (IMT) in young adult females with migraine.

Methods: This case-control study included 148 female patients (age range: 18-50 years). Read More

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http://dx.doi.org/10.1007/s10072-019-3710-5DOI Listing
January 2019

Advancing multimer analysis of von Willebrand factor by single-molecule AFM imaging.

PLoS One 2019 15;14(1):e0210963. Epub 2019 Jan 15.

Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

The formation of hemostatic plugs at sites of vascular injury crucially involves the multimeric glycoprotein von Willebrand factor (VWF). VWF multimers are linear chains of N-terminally linked dimers. The latter are formed from monomers via formation of the C-terminal disulfide bonds Cys2771-Cys2773', Cys2773-Cys2771', and Cys2811-Cys2811'. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210963PLOS
January 2019

The von Willebrand factor D'D3 assembly and structural principles for factor VIII binding and concatemer biogenesis.

Blood 2019 Jan 14. Epub 2019 Jan 14.

Children's Hospital Boston and Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA, United States;

D assemblies comprise half of von Willebrand factor yet are of unknown structure. D1 and D2 in the prodomain and D'D3 in mature VWF at Golgi pH form helical VWF tubules in Weibel Palade bodies and template dimerization of D3 through disulfides to form ultralong VWF concatemers. D'D3 forms the binding site for factor VIII. Read More

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http://dx.doi.org/10.1182/blood-2018-10-876300DOI Listing
January 2019
1 Read

[Correlation Research on Blood Glucose Fluctuation in Type 2 Diabetes Mellitus Patients and Vas- cular Endothelial Injury/Platelet Activation/PKCβ1 Expression].

Zhongguo Zhong Xi Yi Jie He Za Zhi 2016 10;36(10):1184-1190

Objective To observe the correlation between blood glucose fluctuation in type 2 dia- betes mellitus ( T2DM) patients and vascular endothelial injury/platelet activation/protein kinase Cβ1 (PKCpβ1). Methods Capillary blood was collected from finger tips of 38 T2DM patients at 7 time points, i.e. Read More

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October 2016
4 Reads

Closing the gap - detection of clinically relevant von Willebrand disease in emergency settings through an improved algorithm based on rotational Thromboelastometry.

BMC Anesthesiol 2019 Jan 10;19(1):10. Epub 2019 Jan 10.

Department of Pediatrics and Adolescent Medicine, University of Erlangen-Nurnberg, Loschgestr. 15, 91054, Erlangen, Germany.

Background: Hemorrhage and blood loss are still among the main causes of preventable death. Global hemostatic assays are useful point-of-care test (POCT) devices to rapidly detect cumulative effects of plasma factors and platelets on coagulation. Thromboelastography (TEG) and Thromboelastometry (ROTEM) are established methods in many anesthesiological departments for guided hemostatic treatment. Read More

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http://dx.doi.org/10.1186/s12871-018-0672-8DOI Listing
January 2019
1 Read

Blood inflammatory and endothelial markers in women with von Willebrand disease.

PLoS One 2019 10;14(1):e0210544. Epub 2019 Jan 10.

Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.

Introduction: VWD-affected females often experience menorrhagia. Periodical fluctuations of the sex steroids during the menstrual cycle cause changes both in the coagulation and immune system. The aim of the current study was to assess the changes in selected inflammatory and endothelial markers in women with VWD during two phases of the menstrual cycle (follicular and luteal) and to compare it with corresponding data from healthy controls. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210544PLOS
January 2019
1 Read

Recipient Single-Nucleotide Polymorphism Predicts Relapse after Unrelated Bone Marrow Transplantation for Hematologic Malignancy.

Int J Mol Sci 2019 Jan 8;20(1). Epub 2019 Jan 8.

Department of Clinical Laboratory Science, Kanazawa University School of Medical Sciences, Kanazawa 920-0942, Japan.

Relapse remains a major obstacle to the survival of patients with hematologic malignancies after allogeneic hematopoietic stem cell transplantation. A disintegrin-like and metalloprotease with a thrombospondin type 1 motif (ADMATS13), which cleaves von Willebrand factor multimers into less active fragments, is encoded by the gene and has a functional single-nucleotide polymorphism (SNP) rs2285489 (C > T). We retrospectively examined whether rs2285489 affected the transplant outcomes in a cohort of 281 patients who underwent unrelated human leukocyte antigen (HLA)-matched bone marrow transplantation for hematologic malignancies. Read More

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http://www.mdpi.com/1422-0067/20/1/214
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http://dx.doi.org/10.3390/ijms20010214DOI Listing
January 2019
1 Read

Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.

N Engl J Med 2019 Jan 9. Epub 2019 Jan 9.

From the Department of Haematology, University College London Hospitals, Cardiometabolic Program, National Institute for Health Research UCLH-UCL Biomedical Research Center, London (M.S.); the Division of Hematology, Department of Internal Medicine, Ohio State University, Columbus (S.R.C.); Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, and the Department of Pathophysiology and Transplantation, University of Milan, Milan (F.P.); the Department of Hematology, Saint-Antoine University Hospital, Paris (P.C.); the Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna (P.K.); the Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland (J.A.K.H.); the Division of Hematology, Duke University School of Medicine, Durham, NC (A.M.); the Hematology Department, Universidad Católica de Valencia Hospital Dr. Peset, Valencia, Spain (J.R.); the Departments of Medicine and Laboratory Medicine, St. Michael's Hospital and University of Toronto, Toronto (K.P.); and Clinical Development, Ablynx, Zwijnaarde, Belgium (F.C., D.B., H.D.W., R.K.Z.).

Background: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets.

Methods: In this double-blind, controlled trial, we randomly assigned 145 patients with TTP to receive caplacizumab (10-mg intravenous loading bolus, followed by 10 mg daily subcutaneously) or placebo during plasma exchange and for 30 days thereafter. Read More

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http://dx.doi.org/10.1056/NEJMoa1806311DOI Listing
January 2019

Cardiac implications of thrombotic thrombocytopenic purpura.

World J Cardiol 2018 Dec;10(12):254-266

Division of Cardiology, Lang Research Center, New York Presbyterian Medical Group - Queens Hospital, Flushing, NY 11355, United States.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that essentially can affect any organ in the human body. The hallmark of the pathogenesis in TTP is the large von Willebrand factor multimers on platelet-mediated micro-thrombi formation, leading to microvascular thrombosis. Autopsy studies showed that cardiac arrest and myocardial infarction are the most common immediate causes of death in these patients. Read More

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https://www.wjgnet.com/1949-8462/full/v10/i12/254.htm
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http://dx.doi.org/10.4330/wjc.v10.i12.254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314883PMC
December 2018
1 Read

Blood Disorders in Patients Undergoing Transcatheter Aortic Valve Replacement: A Review.

JACC Cardiovasc Interv 2019 Jan;12(1):1-11

Department of Cardiology, Quebec Heart and Lung Institute, Laval University, Quebec City, Quebec, Canada. Electronic address:

Transcatheter aortic valve replacement (TAVR) is well established for treating patients with severe aortic stenosis considered at intermediate to high surgical risk. Blood disorders such as anemia, thrombocytopenia, and acquired type 2A von Willebrand disease are relatively frequent in TAVR candidates, and multiple studies to date have highlighted their potential clinical association with mortality and/or bleeding complications post-TAVR. The present review provides an overview of various blood disorders observed pre- and post-TAVR, with special focus on their incidence, etiology, clinical association, and management. Read More

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http://dx.doi.org/10.1016/j.jcin.2018.09.041DOI Listing
January 2019

[Research progress of the relationship between von Willebrand factor and hepatic diseases].

Authors:
Q Wang Y M Zhao

Zhonghua Gan Zang Bing Za Zhi 2018 Nov;26(11):877-880

The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.

Hepatic diseases refer to acute and chronic liver disease linked to all kinds of hepatic pathological changes. Viral hepatitis, bacterial infections, endotoxins and other factors initiate the activation and injury of vascular endothelial cells in patients with liver diseases. Von Willebrand factor (vWF) represents a specific marker of endothelial dysfunction and plays a vital role in the occurrence and development of hepatic diseases. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1007-3418.2018.11.016DOI Listing
November 2018
1 Read

Mirroring preeclampsia: the molecular basis of Ballantyne syndrome.

J Matern Fetal Neonatal Med 2019 Jan 6:1-6. Epub 2019 Jan 6.

e Perinatal Research Centre , The Royal Women's Hospital , Melbourne , Australia.

Objectives: The purpose of this article was to further elucidate the pathophysiology of Mirror (Ballantyne) syndrome within the context of known biomarkers for preeclampsia.

Methods: This novel insight from clinical practice involved a case of post-twin-to-twin transfusion syndrome-laser hydrops in an ex-donor twin, corroborated by histopathologic placental territory edema and maternal sequelae of Mirror syndrome. We serially measured the levels of activin A, follistatin, endothelin-1 (ET-1), intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), soluble fms-like tyrosine kinase 1 (sFlt), and von Willebrand factor (vWF) in the maternal serum from disease evolution through to recovery. Read More

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http://dx.doi.org/10.1080/14767058.2018.1500550DOI Listing
January 2019

Blood Diathesis in a Patient of Rare Blood Group 'Bombay Phenotype'.

Cureus 2018 Oct 23;10(10):e3488. Epub 2018 Oct 23.

Hematology, Shifa International Hospital, Islamabad, PAK.

Bombay blood group or Oh phenotype is a rare autosomal recessive phenotype within the ABO blood grouping system. It occurs due to a mutation in the H gene that produces H antigen on red blood cells (RBCs). Individuals with two mutant H genes lack H antigen on RBCs and have anti-H antibodies in serum. Read More

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http://dx.doi.org/10.7759/cureus.3488DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314794PMC
October 2018
1 Read

Haemostatic Profiles are Similar across All Aetiologies of Cirrhosis.

Thromb Haemost 2019 Jan 4. Epub 2019 Jan 4.

Surgical Research Laboratory and Section of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Background And Aim:  Patients with cirrhosis may acquire profound changes in haemostasis. Although haemostatic changes in cirrhosis have been extensively studied, most studies were performed in groups of patients with mixed aetiology. As thrombotic events appear more common in some aetiologies of disease, notably non-alcoholic steatohepatitis (NASH) and cholestatic disease, we hypothesized that haemostatic changes might be different across aetiologies. Read More

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http://dx.doi.org/10.1055/s-0038-1676954DOI Listing
January 2019

A Non-woven Path: Electrospun Poly(lactic acid) Scaffolds for Kidney Tissue Engineering.

Tissue Eng Regen Med 2018 Jun 14;15(3):301-310. Epub 2018 Feb 14.

Institute of Bioengineering, School of Engineering, The University of Edinburgh, Faraday Building, The King's Buildings, Mayfield Road, Edinburgh, EH9 3JL UK.

Chronic kidney disease is a major global health problem affecting millions of people; kidney tissue engineering provides an opportunity to better understand this disease, and has the capacity to provide a cure. Two-dimensional cell culture and decellularised tissue have been the main focus of this research thus far, but despite promising results these methods are not without their shortcomings. Polymer fabrication techniques such as electrospinning have the potential to provide a non-woven path for kidney tissue engineering. Read More

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http://link.springer.com/10.1007/s13770-017-0107-5
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http://dx.doi.org/10.1007/s13770-017-0107-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171675PMC
June 2018
1 Read

Management of von Willebrand disease in patients undergoing total hip and knee arthroplasty.

J Perioper Pract 2019 01 2:1750458918820793. Epub 2019 Jan 2.

Division of Trauma & Orthopaedics Surgery, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK.

Von Willebrand disease is the commonest bleeding disorder encountered in the medical and surgical field. As a result of the repetitive bleeding into the joints many patients suffer from arthropathy that can cause a significant amount of pain and functional limitation. Total joint arthroplasty is being utilised as the last resort in managing these patients; however, the presence of von Willebrand disease raises a number of dilemmas. Read More

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http://dx.doi.org/10.1177/1750458918820793DOI Listing
January 2019

Von Willebrand Factor/ADAMTS-13 Interactions at Birth: Implications for Thrombosis in the Neonatal Period.

J Thromb Haemost 2018 Dec 29. Epub 2018 Dec 29.

Hemostasis Branch, Division of Plasma Protein Therapeutics, Office of Tissues and Advanced Therapies, Center for Biologics Evaluation and Research, US FDA, Silver Spring, MD, 20993.

Von Willebrand Factor (VWF) and its cleaving protease ADAMTS-13 (A Disintegrin and Metalloproteinase with Thrombospondin type 1 motif, member 13) are essential components to hemostasis. These plasma proteins have also been implicated in number of disease states, including those affecting children. The best described abnormality is the congenital form of Thrombotic Thrombocytopenic Purpura (TTP) resulting from germline mutations in the ADAMTS-13 gene. Read More

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http://doi.wiley.com/10.1111/jth.14374
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http://dx.doi.org/10.1111/jth.14374DOI Listing
December 2018
5 Reads

Automated assays for von Willebrand factor activity.

Am J Hematol 2018 Dec 28. Epub 2018 Dec 28.

Department of Pathology and Laboratory Medicine, Larner College of Medicine, University of Vermont, Burlington, Vermont.

von Willebrand factor (VWF) ristocetin cofactor activity (VWF:RCo) by platelet aggregometry has been considered the gold standard for evaluating the ability of VWF to bind platelets for over 40 years. Many automated systems no longer require platelets and rather rely on agglutination of latex particles. Automated methods of measuring VWF activity have improved performance characteristics and are performed on the same coagulation instruments used for routine testing via immunoturbidimetric methodology. Read More

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http://doi.wiley.com/10.1002/ajh.25393
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http://dx.doi.org/10.1002/ajh.25393DOI Listing
December 2018
2 Reads

Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.

Eur J Cardiothorac Surg 2018 Dec 22. Epub 2018 Dec 22.

Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Objectives: Bleeding signs can become life-threatening complications in patients on mechanical circulatory support (MCS). Clinical phenotyping and comprehensive analyses of the cause of bleeding are, therefore, essential, especially when risk-stratifying patients during MCS workup. We conducted coagulation analyses and determined von Willebrand factor (VWF) parameters in a paediatric cohort on temporary extracorporeal life support, extracorporeal membrane oxygenation or long-term ventricular assist device support. Read More

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http://dx.doi.org/10.1093/ejcts/ezy408DOI Listing
December 2018

Identification and molecular characterization of a novel Babesia orientalis thrombospondin-related anonymous protein (BoTRAP1).

Parasit Vectors 2018 Dec 27;11(1):667. Epub 2018 Dec 27.

State Key Laboratory of Agricultural Microbiology, College of Veterinary Medicine, Huazhong Agricultural University, Wuhan, 430070, Hubei, China.

Background: The thrombospondin-related anonymous protein (TRAP) family, a kind of transmembrane protein, is widely distributed with a conserved feature of structure in all apicomplexan parasites and plays a crucial role in the gliding motility and survival of parasites.

Methods: The Babesia orientalis TRAP1 gene (BoTRAP1) was truncated and cloned into a pET-42b expression vector and expressed as a GST-tag fusion protein with a TEV protease site. Rabbit anti-rBoTRAP1 antibody was produced and purified using a protein A chromatography column. Read More

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http://dx.doi.org/10.1186/s13071-018-3245-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307320PMC
December 2018

Genome-Wide Association Trans-Ethnic Meta-Analyses Identifies Novel Associations Regulating Coagulation Factor VIII and von Willebrand Factor Plasma Levels.

Circulation 2018 Nov 20. Epub 2018 Nov 20.

Epidemiology, University of Washington, Seattle, UNITED STATES.

Background: Factor VIII (FVIII) and its carrier protein von Willebrand factor (VWF) are associated with risk of arterial and venous thrombosis and with hemorrhagic disorders. We aimed to identify and functionally test novel genetic associations regulating plasma FVIII and VWF.

Methods: We meta-analyzed genome-wide association results from 46,354 individuals of European, African, East Asian, and Hispanic ancestry. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.034532DOI Listing
November 2018
2 Reads
14.430 Impact Factor

The influence of hypoxia on platelet function and plasmatic coagulation during systemic inflammation in humans in vivo.

Platelets 2018 Dec 25:1-4. Epub 2018 Dec 25.

a Department of Intensive Care Medicine , Radboud University Medical Center , Nijmegen , The Netherlands.

Systemic inflammation and hypoxia frequently occur simultaneously in critically ill patients, and are both associated with platelet activation and coagulopathy. However, human in vivo data on the effects of hypoxia on platelet function and plasmatic coagulation under systemic inflammatory conditions are lacking. In the present study, 20 healthy male volunteers were randomized to either 3. Read More

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http://dx.doi.org/10.1080/09537104.2018.1557617DOI Listing
December 2018
1 Read

No independent association found between von Willebrand factor and plaque ulceration in carotid artery atherosclerosis.

Thromb Res 2018 Dec 4;174:95-97. Epub 2018 Dec 4.

Department of Radiology, Erasmus MC University Medical Center, Rotterdam, the Netherlands. Electronic address:

Angiography Computerized Tomography (CT) Cerebrovascular disease/stroke Ischemic stroke Atherosclerosis Stenosis Thrombosis. Read More

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http://dx.doi.org/10.1016/j.thromres.2018.12.002DOI Listing
December 2018

How I treat low von Willebrand factor levels.

Blood 2018 Dec 21. Epub 2018 Dec 21.

Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland.

Partial quantitative deficiency of plasma von Willebrand factor (VWF) is responsible for the majority of cases of von Willebrand disease, the commonest inherited human bleeding disorder. International consensus guidelines recommend that patients with reduced plasma VWF:Ag levels and bleeding phenotypes should be considered in two distinct subsets. First, patients with marked reductions in plasma VWF levels (<30 IU/dL) usually have significant bleeding phenotypes and should be classified with 'Type 1 VWD. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-10
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http://dx.doi.org/10.1182/blood-2018-10-844936DOI Listing
December 2018
7 Reads

Successful management of multiple pregnancies in a family with varying severity of Von Willebrand disease.

Obstet Med 2018 Dec 23;11(4):192-194. Epub 2018 Mar 23.

Department of Haematology, Guys & St Thomas' NHS Foundation Trust, London, UK.

We present the obstetric history of a family of three sisters with Von Willebrand disease, managed in our centre over the course of nine successful pregnancies. The abnormalities result from inheritance of an exon 50 skipping mutation in the Von Willebrand factor gene, resulting from consanguinity. Two of the sisters were identified as having a severe phenotype with a Von Willebrand factor level of less than 5 IU/dl, with the other having a mild phenotype. Read More

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http://dx.doi.org/10.1177/1753495X17754150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295762PMC
December 2018

[Retrospective analysis of risk factors for postoperative bleeding undergoing adenotonsillectomy].

Laryngorhinootologie 2018 Dec 20. Epub 2018 Dec 20.

Department of ENT surgery, Linköping university, Linköping, Sweden (Verksamhetschef: T. Jaklovska).

Objective: Adenotonsillectomy is one of the most common surgical procedures in Germany and the most critical postoperative complication is postoperative hemorrhage, particularly for children. The medical history has been considered superior to laboratory tests.

Material And Methods: In a retrospective anonymous examination over a 4 - year period the data of children and adolescent patients subject to surgery of the Waldeyer's tonsillar ring were recorded. Read More

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http://dx.doi.org/10.1055/a-0806-7578DOI Listing
December 2018

Critical role of C5a in sickle cell disease.

Am J Hematol 2018 Dec 19. Epub 2018 Dec 19.

Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota, Minneapolis, Minnesota.

Innate immune complement activation may contribute to sickle cell disease (SCD) pathogenesis. Ischemia-reperfusion physiology is a key component of the inflammatory and vaso-occlusive milieu in SCD and is associated with complement activation. C5a is an anaphylatoxin, a potent pro-inflammatory mediator that can activate leukocytes, platelets, and endothelial cells, all of which play a role in vaso-occlusion. Read More

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http://doi.wiley.com/10.1002/ajh.25384
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http://dx.doi.org/10.1002/ajh.25384DOI Listing
December 2018
12 Reads

Defective collagen binding and increased bleeding in a murine model of von Willebrand disease affecting collagen IV binding.

J Thromb Haemost 2019 Jan 18;17(1):63-71. Epub 2018 Dec 18.

Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, WI, USA.

Essentials Defective binding to collagen IV has been seen in von Willebrand factor (VWF) A1 domain variants. We developed a murine model of defective VWF-collagen IV interactions with VWF variant p.R1399H. Read More

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http://doi.wiley.com/10.1111/jth.14341
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http://dx.doi.org/10.1111/jth.14341DOI Listing
January 2019
3 Reads

Diagnostic challenges of inherited mild bleeding disorders: a bait for poorly explored clinical and basic research.

Authors:
D Mezzano T Quiroga

J Thromb Haemost 2018 Dec 18. Epub 2018 Dec 18.

Clinical Laboratory, School of Medicine, Pontificia Universidad Católica de Chile, Chile.

The best known inherited mild bleeding disorders (MBD), type 1 von Willebrand disease, platelet function disorders (PFD) and mild/moderate clotting factor deficiencies, are characterized clinically by mucocutaneous bleeding (MCB) and although highly prevalent, still pose difficult diagnostic problems. These include establishing the pathological nature of bleeding, as well as the uncertainties surrounding the clinical relevance of laboratory results. Furthermore, the high frequency of bleeding symptoms in the normal population and the subjective appraisal of symptoms by patients or parents makes elucidating the pathologic nature of bleeding difficult. Read More

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http://dx.doi.org/10.1111/jth.14363DOI Listing
December 2018

A single-centre study of management of pregnant women with von Willebrand disease.

Haemophilia 2018 Dec 18. Epub 2018 Dec 18.

Hospices Civils de Lyon, Unité d'Hémostase Clinique/Centre Régional de Traitement de l'Hémophilie, Hôpital Louis Pradel, Bron Cedex, France.

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http://dx.doi.org/10.1111/hae.13653DOI Listing
December 2018

Treatment of rare factor deficiencies other than hemophilia.

Blood 2018 Dec 17. Epub 2018 Dec 17.

Universita degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy

The deficiency of fibrinogen, prothrombin, factor V, VII, VIII, IX, X, XI and XIII, called rare coagulation disorders (RCDs), may result in coagulopathies leading to spontaneous or post-trauma and post-surgery hemorrhages. RCDs are characterized by a wide variety of symptoms, from mild to severe, which can vary significantly from one disease to another and from one patient to another. The most typical symptoms of all RCDs are mucosal bleedings and bleeding at the time of invasive procedures, while other life-threatening symptoms such as central nervous system bleeding and hemarthroses are mostly present only in some deficiencies (afibrinogenemia, FX and FXIII). Read More

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http://dx.doi.org/10.1182/blood-2018-06-820738DOI Listing
December 2018
1 Read

Missed at first Glanz: Glanzmann thrombasthenia initially misdiagnosed as Von Willebrand Disease.

Transfus Apher Sci 2018 Dec 5. Epub 2018 Dec 5.

National Coagulation Centre, St James's Hospital, Dublin 8, Ireland; Irish Centre for Vascular Biology, Molecular & Cellular Therapeutics, Royal College of Surgeons in Ireland, Dublin 2, Ireland. Electronic address:

Glanzmann thrombasthenia (GT) is an autosomal recessive bleeding disorder caused by a defect in platelet integrin αIIbβ3. Given the rarity of the condition (1/1,000,000), assessment and diagnosis should be undertaken in a specialist centre. We report the case of a 34 year old woman with severe menorrhagia and a childhood diagnosis from another centre of Von Willebrand Disease. Read More

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http://dx.doi.org/10.1016/j.transci.2018.11.008DOI Listing
December 2018

Combination of acquired von Willebrand syndrome (AVWS) and Glanzmann thrombasthenia in monoclonal gammopathy of uncertain significance (MGUS), a case report.

Thromb J 2018 26;16:30. Epub 2018 Nov 26.

Department of Molecular Medicine and Haematology, Faculty of Health Sciences, University of the Witwatersr and National Health Laboratory Services, 7 York Road, Parktown, Johannesburg, 2196 South Africa.

Background: Autoimmune paraphenomena, are associated with B-cell lymphoproliferative disorders, including monoclonal gammopathy of uncertain significance. These paraphenomena can rarely include acquired bleeding disorders.

Case Presentation: This case study reports an unusual clinical presentation of 2 acquired bleeding disorders, Acquired von Willebrand syndrome (disease) and Acquired Glanzmann's thrombasthenia, in an elderly patient with monoclonal gammopathy of uncertain significance. Read More

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http://dx.doi.org/10.1186/s12959-018-0184-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260759PMC
November 2018
2 Reads

The effects of vitamin D supplementation on endothelial activation among patients with metabolic syndrome and related disorders: a systematic review and meta-analysis of randomized controlled trials.

Nutr Metab (Lond) 2018 29;15:85. Epub 2018 Nov 29.

4Research Center for Biochemistry and Nutrition in Metabolic Diseases, Kashan University of Medical Sciences, Kashan, IR Iran.

Background And Objective: The current systematic review and meta-analysis of randomized controlled trials (RCTs) was conducted to summarize the effect of vitamin D supplementation on endothelial activation among patients with metabolic syndrome and related disorders.

Methods: Cochrane library, Embase, PubMed, and Web of Science database were searched to identify related RCTs published before 30th April 2018. The heterogeneity among the included studies was assessed using Cochran's Q test and I-square (I) statistic. Read More

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http://dx.doi.org/10.1186/s12986-018-0320-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6267828PMC
November 2018
1 Read

Thrombin generation during a regular menstrual cycle in women with von Willebrand disease.

Sci Rep 2018 Nov 30;8(1):17467. Epub 2018 Nov 30.

Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.

Fluctuations of the sex steroids during the menstrual cycle might significantly influence hemostasis. This association, derived from a number of the observations on healthy women, is yet to be described in females affected by bleeding disorders. The aim of the current study was to assess the changes in hemostatic variables in women with vWD during two phases of the menstrual cycle (follicular and luteal) and to compare it with healthy controls. Read More

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http://dx.doi.org/10.1038/s41598-018-35897-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6269516PMC
November 2018
1 Read

Beyond plasma exchange: novel therapies for thrombotic thrombocytopenic purpura.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):539-547

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246029PMC
November 2018
7 Reads

Clinical and laboratory diagnosis of TTP: an integrated approach.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):530-538

Department of Medicine and Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Thrombotic thrombocytopenia purpura (TTP) is a rare, life-threatening disease with an incidence of approximately 2 persons per million per year. It is characterized by severe deficiency of the von Willebrand cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), leading to formation of platelet-rich thrombi in the microvasculature. Prompt initiation of appropriate therapy, particularly plasma exchange, may be life-saving. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.530DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246034PMC
November 2018
8 Reads

Adult and pediatric mechanical circulation: a guide for the hematologist.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):507-515

Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.

Mechanical circulatory support (MCS) is the overarching term that encompasses the temporary and durable devices used in patients with severe heart failure. MCS disturbs the hematologic and coagulation system, leading to platelet activation, activation of the contact pathway of coagulation, and acquired von Willebrand syndrome. Ischemic stroke and major hemorrhage occur in up to 30% of patients. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245997PMC
November 2018
1 Read

FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders.

Eur J Epidemiol 2018 Dec 5. Epub 2018 Dec 5.

Haemophilia Treatment Centre, APHM, Children Hospital La Timone, Aix Marseille University, INSERM, INRA, C2VN, Marseille, France.

FranceCoag is an ongoing open prospective multicentre cohort project aimed at improving epidemiological knowledge about inherited bleeding disorders in France. The main objective of this article was to evaluate the project's progress as of the 30th December 2016. Between 1994 and this date, of the 10,047 patients included in the study, 384 (3. Read More

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http://link.springer.com/10.1007/s10654-018-0468-7
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http://dx.doi.org/10.1007/s10654-018-0468-7DOI Listing
December 2018
4 Reads

Asparaginyl endopeptidase may promote liver sinusoidal endothelial cell angiogenesis via PI3K/Akt pathway.

Rev Esp Enferm Dig 2018 Dec 3;111. Epub 2018 Dec 3.

Department of Gastroenterology, Zhongshan Hospital, Fudan University, China.

Background And Aims: pathological angiogenesis plays an important role in the progression of chronic liver diseases. Asparaginyl endopeptidase (AEP) participates in tumor angiogenesis and was recently shown to be associated with liver fibrosis. This study aimed to explore the effect of AEP on liver sinusoidal endothelial cell (LSECs) angiogenesis and determine the underlying mechanism. Read More

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http://dx.doi.org/10.17235/reed.2018.5709/2018DOI Listing
December 2018
1.317 Impact Factor

Characterization of VWF gene conversions causing von Willebrand disease.

Br J Haematol 2018 Nov 29. Epub 2018 Nov 29.

Department of Pediatric Haematology and Oncology, University Medical Centre, Eppendorf, Hamburg, Germany.

We previously reported that von Willebrand Factor gene (VWF) conversions are a relatively frequent cause of von Willebrand disease (VWD), however, their molecular pathomechanisms resulting in variant phenotypes is largely unknown. Here, we characterized VWF conversions harbouring missense and synonymous mutations, through generating a series of mutant constructs followed by transient expression in 293 cells, and qualitative and quantitative analysis of recombinant VWF (rVWF). The characterization of mutant rVWF showed the critical roles of synonymous variants in the pathogenicity of VWF conversions. Read More

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http://doi.wiley.com/10.1111/bjh.15709
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http://dx.doi.org/10.1111/bjh.15709DOI Listing
November 2018
10 Reads
4.711 Impact Factor

Multiple biomarker panel to screen for severe aortic stenosis: results from the CASABLANCA study.

Open Heart 2018 1;5(2):e000916. Epub 2018 Nov 1.

Cardiology Division, Department of Medicine, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts, USA.

Objective: Severe aortic valve stenosis (AS) develops via insidious processes and can be challenging to correctly diagnose. We sought to develop a circulating biomarker panel to identify patients with severe AS.

Methods: We enrolled study participants undergoing coronary or peripheral angiography for a variety of cardiovascular diseases at a single academic medical centre. Read More

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http://dx.doi.org/10.1136/openhrt-2018-000916DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6242008PMC
November 2018
1 Read

Altered plasma proteins released from platelets and endothelial cells are associated with human patent ductus arteriosus.

J Cell Physiol 2018 Nov 27. Epub 2018 Nov 27.

Department of Cardiovascular Surgery & Center for Basic Medical Research, TEDA International Cardiovascular Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China.

Patent ductus arteriosus is the third most common congenital heart disease and resulted from the persistence of ductal patency after birth. Ductus arteriosus closure involves functional and structural remodeling, controlled by many factors. The changes in plasma protein levels associated with PDA closure are not known. Read More

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http://doi.wiley.com/10.1002/jcp.27433
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http://dx.doi.org/10.1002/jcp.27433DOI Listing
November 2018
5 Reads

Pharmacokinetics of plasma infusion in congenital thrombotic thrombocytopenic purpura.

J Thromb Haemost 2019 Jan 31;17(1):88-98. Epub 2018 Dec 31.

Department of Haematology, UCLH and Cardiometabolic Programme-NIHR UCLH/UC BRC London, London, UK.

Essentials Congenital thrombotic thrombocytopenic purpura (TTP) is primarily treated with plasma infusion. We present a pharmacokinetic analysis of ADAMTS-13 in six patients following plasma infusion. A median half-life of 130 h was demonstrated, ranging between 82. Read More

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http://dx.doi.org/10.1111/jth.14345DOI Listing
January 2019
1 Read

Bleeding Disorders in Adolescents with Heavy Menstrual Bleeding: The Queensland Statewide Paediatric and Adolescent Gynaecology Service.

J Pediatr Adolesc Gynecol 2018 Nov 22. Epub 2018 Nov 22.

Statewide Paediatric and Adolescent Gynaecology Service, Royal Brisbane and Women's Hospital and Lady Cilento Children's Hospital, Brisbane, Queensland, Australia; University of Queensland, Faculty of Medicine, Brisbane, Queensland, Australia.

Study Objective: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.11.005DOI Listing
November 2018
1 Read

α-galactosidase A deficiency promotes von Willebrand factor secretion in models of Fabry disease.

Kidney Int 2019 Jan 22;95(1):149-159. Epub 2018 Nov 22.

Division of Nephrology, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Michigan, USA. Electronic address:

Fabry disease results from loss of activity of the lysosomal enzyme α-galactosidase A (GLA), leading to the accumulation of globoseries glycosphingolipids in vascular endothelial cells. Thrombosis and stroke are life-threatening complications of Fabry disease; however, the mechanism of the vasculopathy remains unclear. We explored the relationship between GLA deficiency and endothelial cell von Willebrand factor (VWF) secretion in in vivo and in vitro models of Fabry disease. Read More

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http://dx.doi.org/10.1016/j.kint.2018.08.033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320285PMC
January 2019
7 Reads

Cardiovascular disease-related hospitalization and mortality among persons with von Willebrand disease: A nationwide register study in Sweden.

Haemophilia 2018 Nov 23. Epub 2018 Nov 23.

Department of Translational Medicine, Skåne University Hospital, Lund University, Malmö, Sweden.

Introduction: It has been hypothesized that persons with von Willebrand disease (VWD) may be protected against arterial thrombosis despite having atherosclerosis.

Aim: To calculate a nationwide estimate of the absolute and comparative burden of cardiovascular disease (CVD) hospitalization and mortality among persons with VWD using birthdate and sex-matched comparisons from the general population in Sweden.

Methods: Persons with VWD regardless of the type and severity, diagnosed by a medical doctor, who lived in Sweden for some time during the observation period 1987 through 2008 were included. Read More

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http://dx.doi.org/10.1111/hae.13642DOI Listing
November 2018
5 Reads
2.603 Impact Factor

Evaluation of bleeding disorders in patients with Noonan syndrome: a systematic review.

J Blood Med 2018 23;9:185-192. Epub 2018 Oct 23.

Medical and Regulatory Affairs, Novo Nordisk Inc, Plainsboro, NJ, USA.

Background: Noonan syndrome (NS) is an autosomal dominant genetic condition that has a number of clinical features, including bleeding diathesis and a number of hematological abnormalities including clotting factor deficiencies, von Willebrand disease and abnormal platelet count/function.

Methods: We evaluated the frequency/types of bleeding disorders, and associated hematological laboratory findings, in patients with NS, using published data from 1965 to 2014.

Results: Of 45 studies identified, 31 included data for 428 patients with NS. Read More

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http://dx.doi.org/10.2147/JBM.S164474DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6208935PMC
October 2018
7 Reads

Association of sleep problems with neuroendocrine hormones and coagulation factors in patients with acute myocardial infarction.

BMC Cardiovasc Disord 2018 Nov 21;18(1):213. Epub 2018 Nov 21.

Department of Psychiatry, Clienia Schlössli AG, Oetwil am See, Switzerland.

Background: Obstructive sleep apnea (OSA) and insomnia are frequent sleep problems that are associated with poor prognosis in patients with coronary heart disease. The mechanisms linking poor sleep with an increased cardiovascular risk are incompletely understood. We examined whether a high risk of OSA as well as insomnia symptoms are associated with neuroendocrine hormones and coagulation factors in patients admitted with acute myocardial infarction. Read More

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http://dx.doi.org/10.1186/s12872-018-0947-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6249741PMC
November 2018
7 Reads
1.500 Impact Factor

ADAMTS13 protects mice against renal ischemia-reperfusion injury by reducing inflammation and improving endothelial function.

Am J Physiol Renal Physiol 2019 Jan 21;316(1):F134-F145. Epub 2018 Nov 21.

Kidney Disease Center of First Affiliated Hospital, and Department of Physiology, School of Basic Medical Sciences, Zhejiang University School of Medicine , Hangzhou , China.

Acute kidney injury (AKI) is a serious condition without efficient therapeutic options. Recent studies have indicated that recombinant human a disintegrin and metalloprotease with thrombospondin motifs 13 (rhADAMTS13) provides protection against inflammation. Therefore, we hypothesized that ADAMTS13 might protect against AKI by reducing inflammation. Read More

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https://www.physiology.org/doi/10.1152/ajprenal.00405.2018
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http://dx.doi.org/10.1152/ajprenal.00405.2018DOI Listing
January 2019
3 Reads