How to Trip Rapid Review

Step 1: Select articles relevant to your search (remember the system is only optimised for single intervention studies)

Step 2: press

Step 3: review the result, and maybe amend the or if you know better! If we're unsure of the overall sentiment of the trial we will display the conclusion under the article title. We then require you to tell us what the correct sentiment is.

1,214 results for

Factor IX Deficiency

by
...
Latest & greatest
Alerts

Export results

Use check boxes to select individual results below

SmartSearch available

Trip's SmartSearch engine has discovered connected searches & results. Click to show

81. Effects and Interferences of Emicizumab, a Humanised Bispecific Antibody Mimicking Activated Factor VIII Cofactor Function, on Coagulation Assays. (PubMed)

Effects and Interferences of Emicizumab, a Humanised Bispecific Antibody Mimicking Activated Factor VIII Cofactor Function, on Coagulation Assays. Emicizumab bridges activated factor IX (FIX) and FX to restore the tenase function mediated by activated FVIII (FVIIIa), which is deficient in people with haemophilia A (PwHA). Unlike FVIII, emicizumab does not require activation to function; thus, in coagulation assays, the behavior of emicizumab may differ from that of FVIII. The objective

Full Text available with Trip Pro

2019 Thrombosis and haemostasis

82. Coagulation factors

that are currently known, eg hepatitis, HIV and potential diseases, eg new variant Creutzfeld-Jacob Disease (nvCJD). Available in: 250, 500, 1000, 1500, 2000 & 3000 iu vials For use in children with Haemophilia A (Classical haemophilia/factor VIII deficiency) Recombinant Factor IX: BeneFix B-Long* Inspiration* PUP B Long* * = in clinical trials Available in: 250, 500, 1000 & 2000 iu vials For use in children with Haemophilia B (Christmas Disease/factor IX deficiency) Recombinant technology reduces exposure (...) to human blood borne infections, both those that are currently known, eg hepatitis, HIV and potential diseases, eg nvCJD Recombinant Factor VIIa Novoseven Available in: 1.2 & 5 mg vials For use in children who have developed antibodies to Factor VIII or IX, or those with severe platelet disorders, and rarely by those with intractable bleeding. It acts by bypassing the factor VIII or IX activation in the clotting cascade. Can also be used in congenital factor VII deficiency but has a very short half

2015 Great Ormond Street Hospital

83. MASAC Recommendation Regarding the Use of Recombinant Clotting Factor Products with Respect to Pathogen Transmission

transmission and should be considered the treatment of choice for individuals with hemophilia A. 2. The recombinant factor IX products Alprolix, BeneFIX, and Rixubis are potentially the safest factor IX products available with respect to pathogen transmission and should be considered the treatment of choice for individuals with hemophilia B. 3. The recombinant factor VIIa product NovoSeven is potentially the safest factor VII product available with respect to pathogen transmission and should be considered (...) the treatment of choice for individuals with congenital factor VII deficiency. 4. The recombinant factor XIII-A subunit product Tretten is potentially the safest factor XIII-A subunit product available with respect to pathogen transmission and should be considered the treatment of choice for individuals with congenital factor XIII-A subunit deficiency. 5. For hemophilia A and B patients with inhibitors, there are often overriding concerns about efficacy that supersede those of potentially increased safety

2014 National Hemophilia Foundation

84. Inhibition of Tissue Factor Pathway Inhibitor (TFPI) as a Treatment for Haemophilia: Rationale with Focus on Concizumab (PubMed)

Inhibition of Tissue Factor Pathway Inhibitor (TFPI) as a Treatment for Haemophilia: Rationale with Focus on Concizumab Replacement therapy with missing factor (F) VIII or IX in haemophilia patients for bleed management and preventative treatment or prophylaxis is standard of care. Restoration of thrombin generation through novel mechanisms has become the focus of innovation to overcome limitations imposed by protein replacement therapy. Tissue factor pathway inhibitor (TFPI) is a multivalent (...) Kunitz-type serine protease inhibitor that regulates tissue factor (TF)-induced coagulation through a FXa-dependent feedback inhibition of the TF.FVIIa complex in plasma and on endothelial surfaces. Concizumab is a monoclonal, humanised antibody, specific for the second Kunitz domain of TFPI that binds and inhibits FXa, abolishing the inhibitory effect of TFPI. Concizumab restored thrombin generation in FVIII and FIX deficient plasmas and decreased blood loss in a rabbit haemophilia model. Phase 1

Full Text available with Trip Pro

2018 Drugs

85. Evaluation of Coagulation Factors and Point-of-care Devices During Veno-venous ECMO Therapy

. Condition or disease Intervention/treatment Extracorporeal Membrane Oxygenation Complication Coagulation Factor Deficiency Diagnostic Test: Detailed coagulation monitoring Study Design Go to Layout table for study information Study Type : Observational Actual Enrollment : 20 participants Observational Model: Cohort Time Perspective: Prospective Official Title: Analysis of the Activity of Different Coagulation Factors and Monitoring of Coagulation Using Point-of-care Devices During a Veno-venous ECMO (...) of the activity of coagulation factor VIII in % through standard coagulometric methods. Changes in the activity of coagulation factor IX [%] during Veno-venous ECMO [ Time Frame: Pre-canulation and 6 hours, 1 day, 3 days, 7 days, 11 days, 15 days and 21 days after canulation ] Repeated assessment of the activity of coagulation factor IX in % through standard coagulometric methods. Changes in the activity of coagulation factor X [%] during Veno-venous ECMO [ Time Frame: Pre-canulation and 6 hours, 1 day, 3

2018 Clinical Trials

86. Extended Half Life Factor (EHF) Products For Heavy Menstrual Bleeding in Hemophilia Carriers

study will provide information for an upcoming larger study. Condition or disease Intervention/treatment Phase Hemophilia Menstrual Flow Excessive Drug: Recombinant FVIII Fc fusion product Eloctate Drug: Recombinant FIX Fc fusion product Alprolix Device: Patient-operated diagnostic device for anemia AnemoCheck. Early Phase 1 Detailed Description: Hemophilia A or B is caused by defects in the factor VIII or IX gene, respectively, of which is located on the X chromosome. This disorder exhibits X (...) -linked inheritance, in which primarily males, with a single X chromosome, are affected and females, with two X chromosomes, are heterozygotes, or carriers. Hemophilia carriers show a wide distribution of factor VIII or IX levels with a mean of 50%, which overlaps the distribution of non-carrier women. Heavy menstrual bleeding is defined as menstrual bleeding that lasts more than 7 days or more specifically as the loss of more than 80cc of blood per cycle. Management is critical as it can lead to iron

2017 Clinical Trials

87. Role of extracytoplasmic function sigma factor PG1660 (RpoE) in the oxidative stress resistance regulatory network of Porphyromonas gingivalis (PubMed)

conditions 176 genes including genes involved in secondary metabolism were downregulated more than two-fold compared with the parental strain. The rPG1660 protein also showed the ability to bind to the promoters of the highly downregulated genes in the PG1660-deficient mutant. As the ECF sigma factor PG0162 has a 29% identity with PG1660 and can modulate its expression, the cross-talk between their regulatory networks was explored. The expression profile of the PG0162PG1660-deficient mutant (P (...) . gingivalis FLL356) revealed that the type IX secretion system genes and several virulence genes were downregulated under hydrogen peroxide stress conditions. Taken together, we have confirmed that PG1660 can function as a sigma factor, and plays an important regulatory role in the oxidative stress and virulence regulatory network of P. gingivalis.© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Full Text available with Trip Pro

2017 Molecular oral microbiology

88. CarF Mediates Signaling by Singlet Oxygen, Generated via Photoexcited Protoporphyrin IX, in Myxococcus xanthus Light-Induced Carotenogenesis (PubMed)

CarF Mediates Signaling by Singlet Oxygen, Generated via Photoexcited Protoporphyrin IX, in Myxococcus xanthus Light-Induced Carotenogenesis Blue light triggers carotenogenesis in the nonphototrophic bacterium Myxococcus xanthus by inducing inactivation of an anti-σ factor, CarR, and the consequent liberation of the cognate extracytoplasmic function (ECF) σ factor, CarQ. CarF, the protein implicated earliest in the response to light, does not resemble any known photoreceptor. It interacts (...) physically with CarR and is required for its light-driven inactivation, but the mechanism is unknown. Blue-light sensing in M. xanthus has been attributed to the heme precursor protoporphyrin IX (PPIX), which can generate the highly reactive singlet oxygen species ((1)O(2)) by energy transfer to oxygen. However, (1)O(2) involvement in M. xanthus light-induced carotenogenesis remains to be established. Here, we present genetic evidence of the involvement of PPIX as well as (1)O(2) in light-induced

Full Text available with Trip Pro

2012 Journal of bacteriology

89. Role of Rac1 in Glycoprotein Ib-IX Mediated Signal Transduction and Integrin Activation. (PubMed)

Role of Rac1 in Glycoprotein Ib-IX Mediated Signal Transduction and Integrin Activation. The platelet receptor for von Willebrand factor, the glycoprotein Ib-IX (GPIb-IX) complex, mediates platelet adhesion at sites of vascular injury and transmits signals leading to platelet activation. von Willebrand factor/GPIb-IX interaction sequentially activates the Src family kinase Lyn (SFK), phosphoinositide 3-kinase (PI3K), and Akt, leading to activation of integrin α(IIb)β(3) and integrin-dependent (...) stable platelet adhesion and aggregation. It remains unclear how Lyn activates the PI3K/Akt pathway after ligand binding to GPIb-IX.Using platelet-specific Rac1(-/-) mice and the Rac1 inhibitor NSC23766, we examined the role of Rac1 in GPIb-IX-dependent platelet activation. Rac1(-/-) mouse platelets and NSC23766-treated human platelets were defective in GPIb-dependent stable adhesion to von Willebrand factor under shear stress, integrin activation, thromboxane A(2) synthesis, and platelet aggregation

Full Text available with Trip Pro

2012 Thrombosis and Vascular Biology

90. RNA-based therapeutic approaches for coagulation factor deficiencies. (PubMed)

RNA-based therapeutic approaches for coagulation factor deficiencies. Substitutive therapy has significantly ameliorated the quality of life of patients with coagulation factor deficiencies. However, there are some limitations that support research towards alternative therapeutic approaches. Here we focus on the rescue of coagulation factor biosynthesis by targeting the RNA processing and translation, which would permit restoration of the altered gene expression while maintaining the gene (...) and secretion of functional factor (F)VII. (ii) Spliceosome-mediated RNA trans-splicing (SMaRT) between mutated and engineered pre-mRNAs produces normal FVIII mRNA and secretion of functional protein. (iii) Aminoglycoside drugs induce ribosome readthrough and suppress premature translation termination caused by nonsense mutations in FVII, VIII and IX. The rescued expression levels ranged from very low (aminoglycosides) to moderate (U1 snRNA and SMaRT), which could result in amelioration of the disease

Full Text available with Trip Pro

2011 Journal of Thrombosis and Haemostasis

91. Diagnosis and management of acquired coagulation factor inhibitor

(VWD) guideline is in preparation (Laffan et al , ; Pasi et al , ). Methods The writing group reviewed publications known to them, supplemented with papers identified through Pubmed, using index terms H(a)emophilia, acquired h(a)emophilia, factors VIII, II, V, VII, IX, X, XI, XIII, fibrinogen, fibrin, inhibitors, autoantibodies, rFVIIa, Novoseven, FEIBA, aPCC, rituximab, management. The writing group produced the draft guideline, which was reviewed and revised by members of the United Kingdom (...) , correction to within the reference range may not occur with a moderate/severe factor deficiency. Conversely, a low titre inhibitor may be diluted by addition of normal plasma to give partial correction in mixing studies. 2It is possible for LA to coexist with a factor deficiency. 3Patients on oral direct inhibitors may also develop acquired inhibitors – attention to the pattern of screening test results (e.g., a prolongation of the aPTT out of proportion to the PT in a patient on Coumadin therapy

2013 British Committee for Standards in Haematology

92. Diagnosis of haemophilia: use of an artificial factor-VIII-deficient human plasma system (PubMed)

Diagnosis of haemophilia: use of an artificial factor-VIII-deficient human plasma system An artificial clotting system deficient in factor VIII has been made from normal human plasma. Factors XII and XI are supplied as ;activation product'. An eluate from Al(OH)(3), which has been incubated with normal plasma, supplies factors X and IX in their ;plasma' (unactivated) form with II. Factor V is provided as the supernatant after the Al(OH)(3)- treated plasma has been precipitated at one-third (...) saturation with (NH(4))(2)SO(4). Fibrinogen is freed of factor VIII by freezing and thawing a lyophylized preparation and then added. Of these, activation product and the fibrinogen may be prepared in advance and stored frozen, and the eluate and supernatant may be made on the day of testing. A phospholipid source and CaCl(2)-solution are also required. In use, a patient's and a control plasma are first diluted in a mixture of the eluate, supernatant, and fibrinogen solution, and clotting times

Full Text available with Trip Pro

1967 Journal of Clinical Pathology

93. Combined Antihaemophilic Globulin and Christmas Factor Deficiency in Haemophilia (PubMed)

Combined Antihaemophilic Globulin and Christmas Factor Deficiency in Haemophilia 13269910 2003 05 01 2018 12 01 0007-1447 2 4955 1955 Dec 24 British medical journal Br Med J Combined antihaemophilic globulin and Christmas factor deficiency in haemophilia. 1533-5 VERSTRAETE M M VANDENBROUCKE J J eng Journal Article England Br Med J 0372673 0007-1447 0 Serum Globulins 9001-27-8 Factor VIII 9001-28-9 Factor IX OM Factor IX Factor VIII Hemophilia A blood Hemophilia B Humans Serum Globulins von

Full Text available with Trip Pro

1955 British medical journal

94. Demonstrable Deficiency of Christmas Factor in Two Sisters (PubMed)

Demonstrable Deficiency of Christmas Factor in Two Sisters 13811697 1998 11 01 2018 12 02 0007-1447 1 5171 1960 Feb 13 British medical journal Br Med J Demonstrable deficiency of Christmas factor in two sisters. 479-82 COOK I A IA DOUGLAS A S AS eng Journal Article England Br Med J 0372673 0007-1447 9001-28-9 Factor IX OM Factor IX Female Hemophilia A genetics Humans Medicine Siblings HEMOPHILIA/genetics 1960 2 13 1960 2 13 0 1 1960 2 13 0 0 ppublish 13811697 PMC1967005 J Clin Pathol. 1958 May

Full Text available with Trip Pro

1960 British medical journal

95. Reactions of Blood with Nonbiologic Surfaces: Ultrastructural and Clotting Studies with Normal and Coagulation Factor Deficient Bloods (PubMed)

test, were influenced by both the type of surface to which blood was exposed and the deficiencies of coagulation Factors I, VIII, IX, or XII. Deficiency of fibrinogen appears to enhance, minimally, activation of the coagulation sequences by test materials. However, deficiency of fibrinogen markedly reduces adhesion of platelets to foreign surfaces. Deficiency of Factor XII, but not of Factors VIII or IX, decreases platelet adhesion to nonbiologic surfaces but to a lesser extent than does deficiency (...) Reactions of Blood with Nonbiologic Surfaces: Ultrastructural and Clotting Studies with Normal and Coagulation Factor Deficient Bloods Interaction of normal and coagulation factor deficient bloods with glass, Teflon and silicone-coated glass surfaces have been studied. The morphology of the blood-surface interaction was observed by scanning electron microscopy. Activation of the intrinsic coagulation system and progression of these changes, monitored by use of the partial thromboplastin time

Full Text available with Trip Pro

1972 The American journal of pathology

96. [Biology of haemostasis disorders: detection and titration of antihaemophilic factor (AHF) inhibitor]

, factor VIII (haemophilia A) or factor IX (haemophilia B). The prevalence of haemophilia is estimated to be about 5000 patients, almost 80% of whom have haemophilia A, i.e. about 4000 patients. The clinical haemorrhagic manifestations depend not on the form (A or B) but on the severity of the haemophilia, defined according to the deficiency in AHF. Treatment is based on the use of concentrates of AHF (factor VIII for haemophilia A, factor IX for haemophilia B). The commonest and most dreaded (...) ) asked HAS to assess the value of the different laboratory tests for haemostasis abnormalities with a view to updating the section in the Nomenclature of Procedures in Laboratory Medicine (NABM) containing the procedures in laboratory medicine for measuring abnormalities of haemostasis (subsection 5-02). One of those procedures is testing for and titration of antihaemophilic factor (AHF) inhibitor. Congenital haemophilia is a haemorrhagic disorder linked to a deficiency in an antihaemophilic factor

2011 Health Technology Assessment (HTA) Database.

97. Thromboembolism Risk Factors

) increase after acute therapy completed (2.2) Persistent risk factors - see above (2) Idiopathic VTE (2) Protein C,Protein S and deficiency (1.8) Prothrombin mutation - G20210A (1.7) for (1.6) Second VTE (1.5) Mild (0.9) Distal VTE (0.5) Transient risk factors (0.5) VII. Risk Factors: Venous Stasis Prolonged immobility Long leg or other limb immobilization Paralysis (CVA) Spinal cord injury s High risk for DVT in surgery without Cardiac Disease VIII. Risk Factors: Hypercoagulable state See Inherited (...) cause found in up to one third of DVT cases Prior (DVT) Medications Increased or Pregnancy (Nolvadex) Phenothiazines Major Recent Surgery (e.g. ) Cancer Consider evaluation for occult cancer in DVT Polycythemia History of thromboembolic disease Deep Venous Thrombosis Type A Blood IX. Risk Factors: Intimal damage Local Surgery (Especially ral and Orthopedic Surgery) ral anesthesia is an independent risk factor Consider Penetrating vessel injury Especially femoral X. References Images: Related links

2018 FP Notebook

98. Von Willebrand Factor

Factor Aka: Von Willebrand Factor , VWF , Von Willebrand Factor Antigen , Von Willebrand Factor Ristocetin Cofactor Activity , VWF:Ag , VWF:RCo From Related Chapters II. Physiology Von Willebrand Factor synthesis Vascular endothelium s Von Willebrand Factor release Platelet activation Endothelial cells Von Willebrand Factor activity Binds factor VIII in circulation (prolongs Factor VIII half-life) Releases factor VIII in response to bleeding Factor VIII in turn is a or in the conversion of Factor IX (...) to IXa in the initrinsic to form thrombin Bridges exposed collagen and platelets when vascular injury occurs Von Willebrand Factor mediates platelet adhesion Von Willebrand Factor is a large protein that binds damaged vasculature and traps, binds platelets to form a platelet plug Analogous to 6-pack plastic ring holder that traps wildlife Von Willebrand Factor deficiency Results in delayed platelet plug formation Results in mucocutaneous bleeding III. Labs Normal VWF range: 50-200 IU/dl IV. Causes

2018 FP Notebook

99. Does the Thrombin Generation Test Performed During the Pharmacokinetic Profile of the Substitutive Factor VIII Bring Benefits to the Personalized Treatment of Pediatric Patients and Adult Hemophilia A Patients Under Prophylaxis ?

topics: available for: resources: Arms and Interventions Go to Arm Intervention/treatment Experimental: hemophilia Patients with severe hemophilia (or moderate hemophilia if presence of hemorrhages) under prophylaxy and subjected to a pharmacokinetic profile of factor VIII Device: Chronometric method Chronometric method of FVIII dosage on STA-R PLC automate (reactive Cephascreen STAGO, Unicalibrateur STAGO, plasma deficient Cryopep, Controls STAGO) Device: Chromogenic method Chromogenic FVIII assay (...) method ( "BIOPHEN FVIII: C" and "BIOPHEN Factor IX" of the firm Hyphen BioMed) Device: Thrombin generation test (TGT) Briefly: the measurement of thrombin generation is performed by the technique of calibrated and automated Thrombinography (CAT) developed by Hemker HC. This technique allows the simultaneous analysis of multiple samples using a fluorometer (Fluoroscan Ascent, ThermoLabsystems OY, Helsinki, Finland) and a Thrombinoscope® software that converts the fluorescence intensity obtained

2016 Clinical Trials

100. Integration-deficient Lentiviral Vectors Expressing Codon-optimized R338L Human FIX Restore Normal Hemostasis in Hemophilia B Mice (PubMed)

. In this article, we describe for the first time a complete correction of factor IX (FIX) deficiency in hemophilia B mice by IDLVs carrying a novel, highly potent human FIX cDNA. A 50-fold increase in human FIX cDNA potency was achieved by combining two mechanistically independent yet synergistic strategies: (i) optimization of the human FIX cDNA codon usage to increase human FIX protein production per vector genome and (ii) generation of a highly catalytic mutant human FIX protein in which the arginine (...) Integration-deficient Lentiviral Vectors Expressing Codon-optimized R338L Human FIX Restore Normal Hemostasis in Hemophilia B Mice Integration-deficient lentiviral vectors (IDLVs) have been shown to transduce a wide spectrum of target cells and organs in vitro and in vivo and to maintain long-term transgene expression in nondividing cells. However, epigenetic silencing of episomal vector genomes reduces IDLV transgene expression levels and renders these safe vectors less efficient

Full Text available with Trip Pro

2014 Molecular Therapy

To help you find the content you need quickly, you can filter your results via the categories on the right-hand side >>>>