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Topic: Factor VIII

In the News (Tue 2 Dec 08)

Damian Green

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Wake Forest

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	s030406b - Factor VIII Inhibitors - Acquired or Induced
		Elective surgery on factor VIII inhibitor patients using continuous infusion of recombinant activated factor VII: plasma factor VII activity of 10 IU/ml is associated with an increased incidence of bleeding.[comment].
		Factor VIII level was <5% in 30 (90%) patients; factor VIII antibodies were elevated (>10 Bethesda units) in 23 (69%) patients.
		Abstract: A strategy is described for the initial detection, management and elimination of factor VIII inhibitors arising in patients with congenital and acquired haemophilia A. It is suggested that children with severe haemophilia A should be screened every 3 months up to the age of 10 years for inhibitors using the Bethesda method.
	www.emory.edu /WHSCL/grady/amreport/litsrch02/s030406b.html (3406 words)

	CBER - Transcript - Workshop on Factor VIII Inhibitors
		Factor VIII inhibitors are interesting for many reasons but one of the interesting points is complement fixation and immune-complex disease and anaphylaxis are relatively rare in contrast to the Factor IX inhibitors.
		The antibody response to Factor VIII is characterized by the titer at any point in time of antibody as measured in vitro and also as measured dynamically in the patient by the nature of the anamnestic response.
		This is the influence of von Willebrand factor on the immunogenicity of Factor VIII concentrate.
	www.fda.gov /cber/minutes/fctrviii112103t.htm (20343 words)

	Factor VIII deficiency (hemophilia A) - MayoClinic.com (Site not responding. Last check: 2007-11-03)
		Factor VIII deficiency, also known as hemophilia A, is an inherited bleeding disorder caused by inadequate levels of factor VIII.
		Factor VIII is a key clotting factor needed for normal blood clotting to occur.
		Factor VIII also may be reduced in other blood clotting disorders, such as von Willebrand's disease.
	www.mayoclinic.com /health/factor-viii-deficiency/AN01036 (192 words)

	NDI Terminology - factor (Site not responding. Last check: 2007-11-03)
		Often, use of the term "factor" indicates that the chemical nature of the substance or its mechanism of action is unknown, as in endocrinology, where "factors" are renamed as "hormones" when their chemical nature is determined.
		Coagulation Factor II, prothrombin - A plasma protein that is converted to the active form thrombin (factor IIa) by cleavage by activated factor X (Xa) in the common pathway of blood coagulation; thrombin then cleaves fibrinogen to its active form fibrin.
		Deficiency of this factor results in a tendency toward thrombotic disorders, due to lack of activation of the fibrinolytic pathway.
	www.ndif.org /Terms/factor.html (623 words)

	Factor VIIa (Recombinant)
		Factor VIIa (recombinant) is a vitamin K-dependent glycoprotein made up of 406 amino acid residues, and is structurally similar to human plasma-derived factor VIIa.
		Factor VIIa is used for the prevention and control of hemorrhagic episodes in certain individuals with hemophilia A (i.e., factor VIII deficiency) or hemophilia B (i.e., factor IX deficiency) who have developed inhibitors to factor VIII or factor IX.
		Factor VIIa for hemophilia A (i.e., antihemophilic factor [factor VIII] deficiency/classic hemophilia) or hemophilia B (i.e., factor IX deficiency/Christmas disease) is considered medically appropriate for the control of hemorrhagic episodes in individuals that have developed inhibitors (alloantibodies) to factor VIII or factor IX.
	www.bcbst.com /MPManual/Factor_VIIa_Recombinant.htm (1026 words)

	The FactorVIIa Source
		Recombinant Factor VIIa (recombinant activated Factor VII) induces hemostasis at the site of injury independent of the presence of Factor VIII or Factor IX by forming complexes with exposed tissue factor (TF), and this distinct mechanism of action explains its potential for effective hemophilia treatment.
		Activated Factor VII (Factor VIIa) is found in the circulation at concentrations roughly corresponding to 1% of the total Factor VII protein mass.
		Factor IXa, Factor VIIIa and Factor Va bind efficiently to the surface of the activated platelet and further activation of Factor X into Factor Xa occurs via the complex between Factor IXa and Factor VIIIa.
	factorviia.com /questionsdisplay.asp?num=3 (401 words)

	Von Willebrand factor - Wikipedia, the free encyclopedia
		Factor VIII is bound to vWF whilst inactive in circulation; Factor VIII degrades rapidly when not bound to vWF.
		Factor VIII is released from vWF by the action of thrombin.
		In the 1950s, vWD was shown to be caused by a plasma factor deficiency (instead of being caused by platelet disorders), and, in the 1970s, the vWF protein was purified.
	en.wikipedia.org /wiki/Von_Willebrand_factor (871 words)

	Factor VIII & IX (Site not responding. Last check: 2007-11-03)
		Two candidate factor VIII concentrates had been circulated to several laboratories, which had all used their routine methods for determination of factor VIII:C by one-stage and chromogenic assay.
		Mikaelsson presented data on her studies on assay discrepancies in pharmacokinetic studies of recombinant factor VIII concentrates, by outlining the well-known difference detected when the factor VIII:C of some recombinant factor VIII concentrates was determined by a one-stage assay compared to the chromogenic assay.
		The aim of the double-blind cross-over study is to evaluate the efficacy of quite low doses of BDD recombinant factor VIII in knee-joint bleeds in adult patients, and various clinical end-points were proposed.
	www.med.unc.edu /isth/SSC/00sscminutes/00fviiiix.htm (1696 words)

	eMedicine - Factor VIII : Article by Robert A Schwartz, MD, MPH
		That the disorder was due to a deficiency of a factor (F) in the blood was proven in 1840 by correction of the bleeding defect by transfusion of whole blood; this was followed in 1911 by the demonstration that normal plasma could shorten the whole blood clotting time of hemophilic blood.
		Another factor that critically determines the length of survival of FVIIIa is activated protein C (APC), which, along with its cofactor, free protein S, is a potent anticoagulant.
		Modulation of the hemorrhagic disorder induced by deficiencies of intrinsic coagulation factors, by the co-inheritance of thrombophilic mutations, is now an accepted determinant of the extent of disruption of hemostasis in patients with a bleeding diathesis.
	www.emedicine.com /med/topic981.htm (14010 words)

	Factor VIII and Factor IX 2004 Minutes (Site not responding. Last check: 2007-11-03)
		Though there was no statistical difference in factor VIII half-life between patients with a more severe and patients with a milder phenotype, a one hour increase in factor VIII half-life was associated with 100 IU/kg/yr less annual clotting factor use.
		Apparently, hufVIII-R593C mice are tolerant to human factor VIII which is in agreement with the strongly reduced incidence of factor VIII inhibitors in patients with mild hemophilia A. However, anti-factor VIII antibody development was induced in hufVIII-R593C/E-16KO mice by multiple subcutaneous injections of factor VIII with an adjuvant.
		Classification of the severity of haemophilia is generally based on the levels of factor VIII or factor IX levels in the plasma.
	www.med.unc.edu /isth/SSC/04sscminutes/04factorviiifactorix.html (5472 words)

	TREATMENT OF ACQUIRED FACTOR VIII INHIBITOR ASSOCIATED WITH CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS
		Factor VIII:C was <2.0% (normal 50-200) and a strong inhibitor was assayed at 38 BU/ml (normal <0.6), using Bethesda assay and human Factor VIII.
		With resolution of her hemarthrosis, factor VIII infusion was stopped after two weeks of treatment, but she was maintained on prednisolone 1 mg/kg/day.
		Syndrome of an acquired inhibitor of factor VIII responsive to cyclophosphamide and prednisolone.
	www.kfshrc.edu.sa /annals/192/98-173.html (2324 words)

	Factor VIII / Antihemophilic Factor (AHF) Lawsuit Information - Find Trial Lawyers and Attorneys with Experience in ...
		Factor VIII, also known as antihemophilic factor or AHF, is indicated for the treatment of patients suffering from hemophilia A, an inherited disorder in which the blood clotting protein Factor VIII is deficient or abnormal.
		Replacement therapy with Factor VIII corrects the defect temporarily but must be given by intravenous infusion, in many cases daily or more often.
		Side effects of Factor VIII include, but may not be limited to, headache, sore throat, upset stomach, nausea, dizziness, fatigue, fever, unusual bleeding and increased pulse rate.
	www.injuryboard.com /view.cfm/Topic=1004 (466 words)

	Hemophilia: Combined Factor V and Factor VIII Deficiency
		With this disorder, the levels of Factor V and Factor VIII in the blood are lower than normal.
		Instead of a single defective Factor V-VIII gene on chromosome 18, a child could inherit a defective Factor V gene from both parents and a defective Factor VIII gene from one parent.
		The chances of inheriting Factor V Deficiency are 1 in 1,000,000 and the chances of inheriting Factor VIII are 1 in 10,000.
	www.hemophilia.ca /en/2.3.4.php (581 words)

	- Guild Regulations
		VIII is the tag for all Factor VIII members.
		The Factor VIII Contingency as a whole is known as a "Guild".
		Factor VIII Contingency was founded with a strong belief that everyone from the Round Table, to our newest members have a voice.
	www.factor8guild.com /index.php?page=regulations (1306 words)

	NHF \| Bleeding Disorders Information Center \| Hemophilia A
		Hemophilia is a bleeding disorder caused by a deficiency in one of the blood clotting factors.
		It is a deficiency in clotting factor VIII.
		Depending on the severity of the disease, DDAVP or factor VIII concentrate may be given prior to dental extractions and surgery to prevent bleeding.
	www.hemophilia.org /bdi/bdi_types1.htm (1358 words)

	August / September1998 - Acquired Inhibitors to Factor VIII
		In contrast to alloantibodies, which are directed against foreign, infused factor VIII and occur in about 15% of children with hemophilia A, autoantibodies to factor VIII are rare and directed against endogenous factor VIII and primarily occur in the elderly, with no prior bleeding history.
		Typically, the patient with an autoantibody to factor VIII presents with a severe, spontaneous, usually massive hemorrhage, such as a retroperitoneal hematoma, hematuria, epistaxis, or postpartum hemorrhage.
		Autoantibodies to factor VIII are suspected when the APTT is prolonged and does not correct in a 1:1 mix with normal plasma, associated with a very low factor VIII level, usually <0.01 U/ml.
	www.itxm.org /TMU1998/tmu8-98.htm (932 words)

	Factor VIII assay
		Factor VIII assay is a blood test to measure the activity of factor VIII (one of the proteins important for coagulation).
		For example, Factor I is also called fibrinogen, Factor II is prothrombin, and Factor XII is Hageman factor.
		Substances that inhibit or "turn off" the action of the coagulation factors are activated along with the coagulation factors.
	www.pennhealth.com /ency/article/003678.htm (696 words)

	NEJM -- Altered factor VIII in acute respiratory failure
		Since endothelial cells synthesize or release factor VIII, we studied factor VIII:antigen, factor VIII:von Willebrand's factor, and factor VIII:coagulant activity in 100 patients with acute respiratory failure, in 29 critically ill patients without evidence of lung disease, and in 60 normal subjects.
		In patients with respiratory failure, both slowly and rapidly migrating components of factor VIII:antigen had abnormal electrophoretic patterns; in moderate and severe cases, there was a marked increase in the fast component.
		Factor VIII:antigen may be a sensitive circulating indicator of pulmonary endothelial injury and repair.
	content.nejm.org /cgi/content/abstract/307/18/1113 (296 words)

	antihemophilic factor (factor VIII) (injectable)
		Some forms of antihemophilic factor (factor VIII) are made from human plasma (part of the blood) and may contain infectious agents (e.g., viruses) that can cause disease.
		A deficiency of factor VIII is the cause of hemophilia A. Antihemophilic factor (factor VIII) is used to treat or prevent bleeding in people with hemophilia A. Antihemophilic factor (factor VIII) may also be used for purposes other than those listed here.
		Antihemophilic factor (factor VIII) is in the FDA pregnancy category C. This means that it is not known whether it will be harmful to an unborn baby.
	hw.healthdialog.com /kbase/multum/d00525a1.htm (1505 words)

	National Hemophilia Foundation for All Bleeding Disorders \| Hemophilia, von Willebrand Disease, Thrombophilia
		Clotting factor concentrates (also called “factor”) is a dried powder form of the missing clotting factor; it is mixed with water to become a liquid again before it is given.
		To prevent a bleeding crisis, people with hemophilia and their families can be taught to administer factor VIII concentrates at home at the first signs of bleeding.
		If antibodies are detected in the blood samples, the dosage of the factor may be increased, or different types of clotting factor or drugs to reduce the antibody levels may be used.
	www.hemophilia.org /bleeding_disorders/hemophilia_a.htm (1166 words)

	Haematologic Technologies - von Willebrand Factor (vWF)
		The formation of a 1.8 MDa von Willebrand factor (vWF) multimer with bound factor VIII is illustrated.
		Studies indicate that factor VIII binds to the NH2-terminal portion of the mature vWF subunit with a stoichiometry of one factor VIII molecule per vWF monomer (16,17).
		A factor VIII free vWF preparation, further purified to ensure removal of factor VIII procoagulant activity, is also available.
	www.haemtech.com /Cofactors/vWF.htm (507 words)

	Factor VIII Lawyer - About Factor VIII - Factor VIII Attorney - Factor VIII Law Firm
		Factor VIII, also known as IX concentrate, is indicated for the treatment of patients suffering from hemophilia A, an inherited disorder in which the blood clotting protein Factor VIII is deficient or abnormal.
		If you have been injured by the drug Factor VIII or IX concentrate, then you may be entitled to compensation.
		The law firm of Kelley/Uustal is handling Factor VIII cases.
	www.clottingfactors.com (285 words)

	Recombinant Human Factor VIII - PRO-318
		Interaction of dicaproyl phosphatidylserine with recombinant factor VIII and its impact on immunogenicity.
		Successful use of recombinant factor VIII devoid of von Willebrand factor during multiple teeth extractions in a patient with type 3 von Willebrand disease.
		Successful induction of immune tolerance by continuous infusion of recombinant factor VIII in a haemophilia A patient with high-inhibitor titres.
	www.prospec.co.il /~prospec/cart/catalog/rHuFacVIII.html (318 words)

	MedlinePlus Medical Encyclopedia: Factor VIII assay
		Plasma factor VIII is usually measured to diagnose or monitor treatment for hemophilia.
		Because of the link between vitamin K and prothrombin, people who take warfarin need to have consistent levels of vitamin K in their diet, as instructed by their doctor.
		This allows a clot to form, stopping the bleeding, then, after enough time has passed for the tissue to heal, the clot is dissolved and blood flow restored.
	www.nlm.nih.gov /medlineplus/ency/article/003678.htm (765 words)

	Bayer Biological Products EU - The Development of the Factor VIII producing Cell Line
		One alternative to antihaemophilic factor VIII isolated from plasma, is the Recombinant Factor VIII (rFactor VIII) manufactured by biotechnological methods.
		The factor VIII protein need no longer be isolated from numerous donations of blood or plasma, the heterogeneous initial material, but can be produced from the cells of an individual and precisely characterized marnmalian cell line.
		In 1984 the human factor VIII gene was first characterized (Gitschier, 1984).
	www.biological.com /rd_safety_development.cfm (388 words)

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