|1.||Hemophilia A (Haemophilia)
|5.||Liver Diseases (Liver Disease)
|1.||Favaloro, Emmanuel J: 24 articles (12/2014 - 04/2002)|
|2.||Federici, Augusto B: 22 articles (08/2011 - 02/2002)|
|3.||Castaman, G: 21 articles (04/2015 - 01/2000)|
|4.||Budde, U: 21 articles (07/2014 - 04/2001)|
|5.||Federici, A B: 19 articles (01/2013 - 03/2000)|
|6.||Mannucci, P M: 18 articles (11/2014 - 01/2000)|
|7.||Franchini, Massimo: 18 articles (10/2012 - 06/2002)|
|8.||Schneppenheim, R: 17 articles (07/2014 - 04/2001)|
|9.||Rodeghiero, F: 15 articles (05/2012 - 01/2000)|
|10.||Favaloro, E J: 14 articles (04/2013 - 01/2000)|
|1.||von Willebrand FactorIBA
01/01/2010 - "The efficacy of highly purified VWF/FVIII concentrates with standardized ristocetin cofactor content (VWF:RCo) has been already proven in patients with von Willebrand's disease (VWD). "
07/01/2007 - "While plasma-derived concentrates containing large amounts of von Willebrand factor (VWF) are effective in treating von Willebrand disease (VWD), optimal dosing remains to be fully characterized. "
01/01/1995 - "The definition of the molecular basis of von Willebrand's disease has helped clarify the structure of von Willebrand factor as well as its essential role in platelet function, particularly under haemodynamic conditions of high shear stress. "
04/01/2011 - "Accelerated clearance of von Willebrand factor (VWF) has been recently identified as a major pathophysiologic mechanism inducing low VWF in some patients with von Willebrand disease (VWD). "
02/01/2006 - "Increased clearance of von Willebrand factor (VWF) is one of the main features of type Vicenza von Willebrand disease (VWD), a variant with plasma and platelet VWF level discrepancies and unusually large VWF multimers. "
|2.||Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric
03/01/2009 - "Until the mid-80s, cryoprecipitate has been the mainstay of treatment of patients with von Willebrand disease who were unresponsive to desmopressin. "
04/01/2005 - "Until the mid 1980s, cryoprecipitate had been the mainstay of treatment of patients with von Willebrand disease (VWD) who were unresponsive to desmopressin. "
12/01/1994 - "Re-evaluation of the therapeutic efficacy of DDAVP in type IIB von Willebrand's disease."
03/01/2002 - "Efficacy of desmopressin as surgical prophylaxis in patients with acquired von Willebrand disease undergoing thyroid surgery."
11/01/2005 - "The aim of the study was to analyze the effectiveness of the application of DDAVP (desmopressin) and Hemate P with cryoprecipitate pre- and postpartum in patients with von Willebrand disease. "
|3.||Factor VIII (Coagulation Factor VIII)IBA
07/15/1983 - "The efficacy of high purity preparations of factor VIII complex are usually lacking effect on the bleeding time in von Willebrand's disease. "
03/01/1989 - "The authors describe a patient with von Willebrand's disease in whom pregnancy itself improved factor VIII activity, enabling performance of an epidural block for labor and delivery."
09/01/1983 - "Recent advances in characterizing and measuring the factor VIII complex have greatly improved the diagnosis of the variant forms of von Willebrand's disease. "
08/01/2008 - "Genetic studies were performed in a patient with a phenotype of von Willebrand disease (VWD) characterized by very low plasma factor VIII and VWF levels and a VWF consisting of only a dimeric band and total absence of all multimers in plasma. "
02/01/1998 - "[Ultrapure factor VIII concentrate with double virus inactivation: study of pharmacokinetics in von Willebrand's disease patients]."
|4.||von Willebrand factor drug combination factor VIIIIBA
11/01/2002 - "Clinical efficacy of highly purified, doubly virus-inactivated factor VIII/von Willebrand factor concentrate (Fanhdi) in the treatment of von Willebrand disease: a retrospective clinical study."
02/01/1999 - "Safety and efficacy of continuous infusion of a combined factor VIII-von Willebrand factor (vWF) concentrate (Haemate-P) in patients with von Willebrand disease."
05/01/2012 - " (Fanhdi and Alphanate Italian Retrospective Study in Willebrand disease) study, 120 von Willebrand disease (VWD) patients treated with Fanhdi(®) or Alphanate(®) were retrospectively analysed. "
07/01/2010 - "Clinical efficacy and safety of the factor VIII/von Willebrand factor concentrate BIOSTATE in patients with von Willebrand's disease: a prospective multi-centre study."
12/01/2007 - "Recently, three multicentre prospective international studies have been carried out to evaluate the clinical efficacy and safety of Fanhdi [high-purity, double-inactivated plasma-derived factor VIII/von Willebrand factor (VWF) concentrate] in patients with von Willebrand's disease (VWD). "
04/02/2015 - "The interactions of VWF and collagen 4 were further studied using plasma samples from a large study of both healthy controls and subjects with different types of von Willebrand disease (VWD). "
08/01/2015 - "The von Willebrand disease (VWD) phenotype of the patient featured low plasma and platelet VWF, multimers with smears extending over the highest normal oligomers in plasma, but not platelets, and an impaired collagen-binding capacity. "
07/01/2015 - "Contribution of the collagen binding activity (VWF:CB) in the range of tests for the diagnosis and classification of von Willebrand disease."
04/01/2013 - "Collagen binding provides a sensitive screen for variant von Willebrand disease."
07/01/2012 - "Collagen binding assays (VWF:CB), however, are not part of the routine work-up for von Willebrand disease (VWD). "
04/15/1992 - "During a study period from 1985 through 1988, plasma von Willebrand's factor antigen (vWF:Ag) concentration was measured as a marker for prevalence of the von Willebrand's disease (vWD) trait in Doberman Pinschers (doberman, n = 5,554), Scottish Terriers (scottie, n = 1,363), and Shetland Sheepdogs (sheltie, n = 4,279). "
06/01/1978 - "This study reports a second von Willebrand's disease antigen (vWagII), distinct from VIIIag, that is also deficient in the platelets and plasma of patients with severe von Willebrand's disease. "
03/01/2010 - "In addition, platelet VWF antigen and activity levels vary markedly between patients with different types of von Willebrand disease (VWD). "
10/01/2008 - "Since the FVIII activity is frequently normal in patients with mild type 1 or 2 von Willebrand disease (VWD), there is controversy whether FVIII testing should accompany VWF Antigen (VWF:Ag) assay. "
01/01/2003 - "Results obtained with the flow cytometric method on samples from two patients with von Willebrand disease 2B were lower than those obtained with the antigen method in accordance with the diagnosis. "
|7.||human F8 proteinIBA
11/01/2011 - "Thrombelastograph platelet mapping in a patient with von Willebrand disease who was treated with Humate-P."
05/01/1990 - "Correction of prolonged bleeding time in von Willebrand's disease with Humate-P."
09/01/1988 - "Humate-P for treatment of von Willebrand disease."
02/01/1977 - "The plasma from swine with von Willebrand's disease late after infusion contained high levels of antihemophilic factor without other detectable activities of the complex. "
05/01/1969 - "Similar fractions of plasma prepared from the blood of patients with classic hemophilia, von Willebrand's disease, or a circulating anticoagulant directed against antihemophilic factor contained, on the average, somewhat less protein than normal plasma; whether this difference was significant is not yet known. "
|8.||Fibrinogen (Factor I)FDA Link
11/01/2014 - "Hematologic laboratory tests performed included complete blood cell count in 62.3%, prothrombin time (PT) in 55.0%, and activated partial thromboplastin time (aPTT) in 53.6%; fibrinogen in 27.6%; factor activity in 17.1%; von Willebrand disease evaluation in 14.5%; and platelet function analyzer in 11.7%. "
01/01/2000 - "In countries lacking the economic resources to obtain virally inactivated concentrates, MB-cryoprecipitates could be useful in von Willebrand's disease and fibrinogen and FXIII deficiencies. "
02/01/1985 - "The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. "
07/01/2012 - "THE PAPWORTH PLUG - successful use of high dose fibrinogen concentrate and platelet concentrate in potential life-threatening complication after cardiopulmonary bypass surgery in a patient with Type 2M Vicenza von Willebrand Disease."
09/01/2002 - "It will be of particular interest to determine the efficacy of rVIIa in conditions such as severe thrombocytopenia, severe von Willebrand disease, severe defects in platelet activation, and severe deficiencies of factors V, X, II, and fibrinogen in which effectiveness would seem to be unlikely based on our current understanding of mechanisms of action of rVIIa."
02/01/2011 - "Use of 1-desamino-8-D-arginine vasopressin in microsurgical reconstruction in a patient with von Willebrand's disease."
02/01/2005 - "Cerebral arterial thrombosis in a young woman following vasopressin for von Willebrand's disease."
06/01/1984 - "The subcutaneous administration of the vasopressin analogue 1-desamino-8-D-arginine vasopressin in patients with von Willebrand's disease and hemophilia."
11/26/1980 - "The use of 1 deamino-8-D-arginine vasopressin (DDVAP), is reported in seven patients with von Willebrand's disease and mild haemophilia undergoing elective surgery. "
06/01/1982 - "Studies of the blood plasminogen activator induced by 1-desamino-8-D-arginine vasopressin with observations in von Willebrand's disease."
06/01/2005 - "Recently, the hematologic literature has reported continuous infusion of factor concentrates and intravenous immunoglobulin as an improved therapy for active bleeding and prophylaxis in patients who are anticipating surgery with congenital von Willebrand disease. "
10/01/2007 - "To describe a patient with common variable immunodeficiency (CVID) and von Willebrand disease who tolerated immunoglobulin replacement via the subcutaneous route. "
05/01/1995 - "High-dose intravenous immunoglobulin delays clearance of von Willebrand factor in acquired von Willebrand disease."
06/01/2005 - "Acquired von Willebrand disease: management of labor and delivery with intravenous dexamethasone, continuous factor concentrate, and immunoglobulin infusion."
05/01/2002 - "Acquired von Willebrand disease--hemostatic management of major orthopedic surgery with high-dose immunoglobulin, desmopressin, and continuous factor concentrate infusion."
05/01/1987 - "The efficacy of intensive plasma exchange in acquired von Willebrand's disease."
10/01/1989 - "Treatment of von Willebrand's disease and hypofibrinogenemia with single donor cryoprecipitate from plasma exchange donation."
05/01/1987 - "The current case demonstrates that the clinical and laboratory abnormalities in a patient with acquired von Willebrand's disease can be corrected completely by plasma exchange; it is recommended therefore that plasma exchange be considered as a mode of therapy in symptomatic patients with this disorder."
05/01/1987 - "This study describes the response to therapeutic plasma exchange in a 60-year-old man with Waldenström's macroglobulinemia who developed a clinically severe bleeding disorder with laboratory features characteristic of acquired von Willebrand's disease. "
11/22/1980 - "A sixth patient with von Willebrand's disease and a factor VIII clotting activity inhibitor was successfully prepared for operation using plasma exchange. "
01/01/1990 - "[Hemostatic therapy in a patient with acquired von Willebrand disease and Waldenström disease with plasmapheresis and DDAVP]."
01/01/2013 - "Hyperviscosity syndrome with pulmonary arterial hypertension presentation in a patient with acquired von Willebrand disease in an autoimmune context is a rare condition that can be treated successfully with plasmapheresis and critical care support."
01/01/1992 - "Donor exposure can be strikingly reduced for patients with classical hemophilia A and von Willebrand's disease when large volumes of potent cryoprecipitated AHF are prepared from donors following DDAVP (1-deamino-8-D-arginine vasopressin) stimulation and automated plasmapheresis--a procedure called "plasma exchange donation." Although this procedure has been reported to be relatively safe for donors, data are limited. "
04/09/1987 - "In 26 patients with hemophilia A or von Willebrand's disease who had not been treated with blood or any blood product and hence were highly susceptible to the development of post-transfusion hepatitis, we infused 32 batches of a factor VIII concentrate that had been produced from large pools of human plasma (collected from paid plasmapheresis donors) and then heated in solution at 60 degrees C for 10 hours before final lyophilization. "
05/01/2002 - "The increasing clinical experience with CI used in haemophilia A and B and von Willebrand disease has proven the advantages of this mode of replacement therapy, providing improved margins of efficacy and safety in various clinical settings requiring the maintenance of haemostatic levels over prolonged periods, as well as reduction in treatment cost compared with the traditional therapy via intermittent injections of coagulation factors. "
06/01/2007 - "[Von Willebrand's disease and anesthesia.]."
06/01/2003 - "[Anesthesia for septoplasty and turbinectomy in von Willebrand disease patient: case report]."
05/01/1981 - "[Anesthesia for a patient with von Willebrand's disease (author's transl)]."
07/01/2009 - "No consensus exists about the use of neuraxial analgesia or anesthesia in patients with von Willebrand disease. "
05/01/2009 - "von Willebrand disease, pregnancy and neuraxial anesthesia: a multi-disciplinary approach for successful regional anesthesia."