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Showing 1-30 of 76 results for "H0920000" within Papers
Innovations in coagulation: improved options for treatment of hemophilia A and B.
Ingrid Pabinger-Fasching et al.
Thrombosis research, 131 Suppl 2, S1-S1 (2013-03-30)
Puneet Gaitonde et al.
The Journal of biological chemistry, 288(24), 17051-17056 (2013-05-08)
Administration of recombinant factor VIII (FVIII), an important co-factor in blood clotting cascade, elicits unwanted anti-FVIII antibodies in hemophilia A (HA) patients. Previously, FVIII associated with phosphatidylserine (PS) showed significant reduction in the anti-FVIII antibody response in HA mice. The
Jay V Karri et al.
The Journal of surgical research, 190(2), 655-661 (2014-03-08)
Fibrinogen is the first coagulation factor to reach critical levels during hemorrhage. Consequently, reestablishing normal fibrinogen levels is necessary to achieve adequate hemostasis. Fibrinogen is supplemented through administration of fresh frozen plasma, cryoprecipitate, or human fibrinogen concentrate, RiaSTAP. RiaSTAP is
Bei Jia et al.
Life sciences, 108(1), 22-29 (2014-05-20)
Hemophilia A (HA) is a severe, congenital bleeding disorder caused by the deficiency of clotting factor VIII (FVIII). For years, traditional laboratory animals have been used to study HA and its therapies, although animal models may not entirely mirror the
A F Chernousov et al.
Bulletin of experimental biology and medicine, 154(3), 396-398 (2013-03-14)
The stimulatory effect of cryoprecipitate on liver regeneration was studied in rabbits with cirrhosis of the liver induced by subcutaneous injection of tetrachloromethane. Cryoprecipitate stimulated the hepatocyte regeneration (appearance of binuclear and proliferating cells) in liver tissue in cirrhosis, but
Brian L Mejak et al.
The journal of extra-corporeal technology, 45(2), 107-111 (2013-08-13)
The timing of blood product administration after cardiopulmonary bypass (CPB) may influence the amount of postoperative transfusion and chest tube output. We performed a retrospective study of a novel technique of administering blood products during modified ultrafiltration (MUF) in congenital
Gerry A F Nicolaes et al.
Blood, 123(1), 113-120 (2013-11-15)
The C domains of coagulation factors V (FV) and VIII (FVIII) are structurally conserved domains and share a common and essential function in membrane binding. In vivo regulation of thrombin formation strongly depends on the expression and regulation of the
An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE).
Priyanka Kasatkar et al.
Annals of hematology, 93(1), 173-175 (2013-04-30)
G Inocêncio et al.
European review for medical and pharmacological sciences, 17(21), 2857-2859 (2013-11-21)
von Willebrand disease type 3, is an extremely rare condition. It can be severe and potentially life-threatening, particularly in pregnant women during labor and subsequently during early puerperium. Due to its rarity, there is no optimal treatment/management during pregnancy. We
Massimo Franchini et al.
British journal of haematology, 165(5), 600-608 (2014-03-19)
Rituximab is a humanized chimeric anti-CD20 monoclonal antibody initially developed for the treatment of some haematological malignancies. Thanks to its ability to rapidly and specifically deplete B cells, it has also been used in a variety of autoimmune disorders, haematological
John B Holcomb et al.
The journal of trauma and acute care surgery, 75(1 Suppl 1), S31-S39 (2013-08-15)
There are few clinical data to guide the use of cryoprecipitate in severely injured trauma patients. Cryoprecipitate is a rich source of fibrinogen and has been associated with improved survival in animal as well as limited human studies. Our objectives
Yan Zeng et al.
The Cochrane database of systematic reviews, 8(8), CD010761-CD010761 (2014-08-29)
Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). In most cases, bleeding episodes are spontaneous and severe at presentation. The optimal hemostatic therapy is controversial. To determine the efficacy of hemostatic therapies
David Lillicrap
Blood, 122(23), 3735-3740 (2013-09-26)
von Willebrand disease (VWD) is the most common autosomally inherited bleeding disorder. The disease represents a range of quantitative and qualitative pathologies of the adhesive glycoprotein von Willebrand factor (VWF). The pathogenic mechanisms responsible for the type 2 qualitative variants
Platelets and bleeding in the dental patient. It's not always from "blood thinners". Von Willebrand disease--clinical assessment and case report.
Harold V Cohen et al.
Journal of the New Jersey Dental Association, 84(2), 28-30 (2013-09-03)
M Osooli et al.
Journal of internal medicine, 277(1), 1-15 (2014-08-30)
Congenital haemophilia A and B are genetic disorders affecting factor VIII and factor IX production, respectively. Factor replacement is the only effective treatment for these deficiencies, but a patient's immune system can develop inhibitory antibodies which bind and interfere with
Gouri Shankar Pandey et al.
Nature medicine, 19(10), 1318-1324 (2013-09-17)
Neutralizing antibodies (inhibitors) to replacement factor VIII (FVIII, either plasma derived or recombinant) impair the effective management of hemophilia A. Individuals with hemophilia A due to major deletions of the FVIII gene (F8) lack antigenically cross-reactive material in their plasma
Joseph Philip et al.
Laboratory medicine, 45(2), 111-115 (2014-05-30)
To compare the coagulation-factor profile of cryoprecipitate produced from fresh frozen plasma from whole blood (WB) stored for 24 hours at room temperature (24CP) with that of standard cryoprecipitate (CP). We collected 80 units of WB from healthy volunteers, of
Anna Aronovich et al.
Blood, 122(15), 2562-2571 (2013-08-29)
Analysis of hematopoietic stem cells (HSCs) in factor VIII knockout (FVIIIKO) mice revealed a novel regulatory role for the coagulation cascade in hematopoiesis. Thus, HSCs in FVIIIKO mice had reduced proportions of CD34(low) cells within Lin(-)Sca(+)Kit(+) progenitors, and exhibited reduced
Jonathan J Morrison et al.
JAMA surgery, 148(3), 218-225 (2013-05-15)
To quantify the impact of fibrinogen-containing cryoprecipitate in addition to the antifibrinolytic tranexamic acid on survival in combat injured. Retrospective observational study comparing the mortality of 4 groups: tranexamic acid only, cryoprecipitate only, tranexamic acid and cryoprecipitate, and neither tranexamic
Mohammed Quader et al.
The Annals of thoracic surgery, 96(6), 2245-2247 (2013-12-04)
We report here a hemophilia patient who was bridged with a left ventricle assist device and later received heart transplantation. Preparation for surgery with factor VIII supplementation, intraoperative conduct of surgery, and challenges of postoperative course are described with a
Von Willebrand disease.
Tonya Shearin-Patterson et al.
JAAPA : official journal of the American Academy of Physician Assistants, 26(4), 46-46 (2013-04-25)
Zuben E Sauna et al.
Blood, 125(2), 223-228 (2014-11-20)
Intron-22-inversion patients express the entire Factor VIII (FVIII)-amino-acid sequence intracellularly as 2 non-secreted polypeptides and have a positive "intracellular (I)-FVIII-CRM" status. Mutations conferring a positive I-FVIII-CRM status are associated with low inhibitor risk and are pharmacogenetically relevant because inhibitor risk
Hironao Wakabayashi et al.
Biochemistry, 52(22), 3921-3929 (2013-05-11)
Factor (F)VIII consists of a heavy chain [A1(a1)A2(a2)B domains] and a light chain [(a3)A3C1C2 domains]. Several reports have shown significant changes in FVIII stability and/or activity following selected mutations at the A1-A2, A1-A3, A2-A3, and A1-C2 domain interfaces. In this
Amanda B Payne et al.
Ethnicity & disease, 24(2), 169-174 (2014-05-09)
Venous thromboembolism (VTE) affects more than 300,000 people in the United States each year. However, it has been estimated that current diagnostic testing fails to identify prothrombotic risk in 50% of VTE patients. This article examines the relationship between levels
Holly Lindsay et al.
Journal of pediatric hematology/oncology, 36(5), e333-e334 (2014-03-26)
Pyogenic granulomas (PGs) are benign vascular lesions occurring in skin and mucous membranes, often secondary to trauma or chronic inflammation. Oral PGs have never been described previously in hemophilia. We describe 2 pediatric patients with hemophilia A, who developed PGs
Esther Bloem et al.
The Journal of biological chemistry, 288(41), 29670-29679 (2013-09-07)
The C1 domain of factor VIII (FVIII) has been implicated in binding to multiple constituents, including phospholipids, von Willebrand factor, and low-density lipoprotein receptor-related protein (LRP). We have previously described a human monoclonal antibody called KM33 that blocks these interactions
Samantha C Gouw et al.
Blood, 121(20), 4046-4055 (2013-04-05)
The objective of this study was to examine the association of the intensity of treatment, ranging from high-dose intensive factor VIII (FVIII) treatment to prophylactic treatment, with the inhibitor incidence among previously untreated patients with severe hemophilia A. This cohort
Flora Peyvandi et al.
American journal of hematology, 88(10), 895-898 (2013-07-03)
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy syndrome caused by a congenital or acquired deficiency of ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor (VWF) and thus prevents the formation of platelet-rich thrombi in the microcirculation. TTP can be
Stefan Schulte
Thrombosis research, 131 Suppl 2, S2-S6 (2013-03-30)
Albumin fusion technology has been used to enhance the pharmacokinetic properties of recombinant coagulation factors. The goal of linking albumin to coagulation factors is to extend the half-life of the coagulation factor, thereby allowing for less frequent dosing for patients
Natalia Rydz et al.
American journal of hematology, 88(12), 1030-1034 (2013-08-06)
A reference genotyping laboratory was established in 2000 at Queen's University, Kingston, to provide genetic testing for Hemophilia A (HA) and B (HB) and create a Canadian mutation database. Canadian hemophilia treatment centers and genetics clinics provided DNA and clinical
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