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Factor IX Deficiency

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121. Individual clotting factor contributions to mortality following trauma Full Text available with Trip Pro

presenting to San Francisco General Hospital between February 2005 and February 2015. Factors II, V, VII, VIII, IX, and X and protein C activity on admission and mortality status at 28 days were assessed. Predictors of 28-day mortality in univariate analysis were included in multiple logistic regression controlling for traumatic brain injury (TBI), acidosis, age, and mechanism of injury. Principal component analysis was utilized to identify phenotypic coagulation.Complete coagulation factor data were (...) available for 876 (61%) of 1,429 patients. In multiple logistic regression, factors V (odds ratio [OR], 0.86; 95% confidence interval [CI], 0.76-0.97), VIII (OR, 0.97; 95% CI, 0.95-0.99), and X (OR, 0.79; 95% CI, 0.68-0.92) and protein C (OR, 1.17; 95% CI, 1.05-1.30) significantly predicted 28-day mortality after controlling for age, base deficit, mechanism of injury, and TBI. Principal component analysis identified two significant principal components (Phenotypes 1 and 2) that accounted for 66.3

2017 The journal of trauma and acute care surgery

122. 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

123. 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

124. 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

125. [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.

126. Shoulder Pain and Mobility Deficits: Adhesive Capsulitis

of Orthopaedic & Sports Physical Therapy. CLASSIFICATION The terms adhesive capsulitis, frozen shoulder, and periar- thritis have been used for patients with shoulder pain and mobility deficits. Adhesive capsulitis will be used in these guidelines to describe both primary idiopathic adhesive cap- sulitis and secondary adhesive capsulitis related to systemic disease, such as diabetes mellitus and thyroid disorders, as well as extrinsic or intrinsic factors, including cerebral vas- cular accident, proximal (...) Shoulder Pain and Mobility Deficits: Adhesive Capsulitis Clinical Practice Guidelines MARTIN J. KELLEY, DPT • MICHAEL A. SHAFFER, MSPT • JOHN E. KUHN, MD • LORI A. MICHENER, PT , PhD AMEE L. SEITZ, PT , PhD • TIMOTHY L. UHL, PT , PhD • JOSEPH J. GODGES, DPT , MA • PHILIP W. MCCLURE, PT , PhD Shoulder Pain and Mobility Deficits: Adhesive Capsulitis Clinical Practice Guidelines Linked to the International Classification of Functioning, Disability, and Health From the Orthopaedic Section

2013 The Orthopaedic Section of the American Physical Therapy Association (APTA), Inc.

127. A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B

A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save (...) this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details

2011 Clinical Trials

128. Nonredundant Roles of IL-10 and TGF-β in Suppression of Immune Responses to Hepatic AAV-Factor IX Gene Transfer Full Text available with Trip Pro

growth factor-β (TGF-β) in the development of tolerance to human coagulation factor IX (hF.IX). Interestingly, IL-10 deficient C57BL/6 mice receiving gene transfer remained tolerant to hF.IX and generated Treg that suppressed anti-hF.IX formation. Effects of TGF-β blockade were also minor in this strain. In contrast, in C3H/HeJ mice, a strain known to have stronger T-cell responses against hF.IX, IL-10 was specifically required for the suppression of CD8(+) T-cell infiltration of the liver (...) Nonredundant Roles of IL-10 and TGF-β in Suppression of Immune Responses to Hepatic AAV-Factor IX Gene Transfer Hepatic gene transfer using adeno-associated viral (AAV) vectors has been shown to efficiently induce immunological tolerance to a variety of proteins. Regulatory T-cells (Treg) induced by this route suppress humoral and cellular immune responses against the transgene product. In this study, we examined the roles of immune suppressive cytokines interleukin-10 (IL-10) and transforming

2011 Molecular Therapy

129. Prevention and Reversal of Antibody Responses Against Factor IX in Gene Therapy for Hemophilia B Full Text available with Trip Pro

Prevention and Reversal of Antibody Responses Against Factor IX in Gene Therapy for Hemophilia B Intramuscular (IM) administration of an adeno-associated viral (AAV) vector represents a simple and safe method of gene transfer for treatment of the X-linked bleeding disorder hemophilia B (factor IX, F.IX, deficiency). However, the approach is hampered by an increased risk of immune responses against F.IX. Previously, we demonstrated that the drug cocktail of immune suppressants rapamycin, IL-10

2011 Frontiers in microbiology

130. A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B

A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more (...) studies before adding more. A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT01361126 Recruitment Status : Completed First Posted : May 26, 2011 Results First Posted : May 9, 2016 Last Update Posted : May 9, 2016

2011 Clinical Trials

131. RNA-based therapeutic approaches for coagulation factor deficiencies. (Abstract)

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

2011 Journal of Thrombosis and Haemostasis

132. 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

133. Clotting Factor Product Administration and Same-Day Occurrence of Thrombotic Events, as Recorded in Large Healthcare Database During 2008-2013. (Abstract)

products were identified by procedure codes, and TEs were ascertained via diagnosis codes. Crude same-day TE rates (per 1000 persons exposed) were estimated overall and by congenital factor deficiency (CFD) status, CF products, age and gender. Multivariable logistic regression analyses were used to control for confounding. Laboratory analysis was used to compare the procoagulant activities of FIX products.Of 3801 individuals exposed to CFs, 117 (30.8 per 1000) had same-day TEs recorded. The crude same (...) -day TE rate was higher for CF users without CFD, 70.2 (102 of 1452), as compared with those with CFD, 6.4 (15 of 2349) (RR, 11.0; 95% CI, 6.4-18.9). For individuals without CFD, a significantly increased same-day TE risk was identified for factor IX complex (OR, 6.92; 95% CI, 3.11-15.40), factor VIIa (OR, 9.42; 95% CI, 4.99-17.78) and other products when compared with fibrin sealant. An increased risk of a TE was found with older age (≥ 45 years), history of TEs and underlying health conditions

2015 Journal of Thrombosis and Haemostasis

134. A Study Comparing Factor Level and Inhibitor Titer Testing Results Drawn From Central Venous Lines and Venipuncture

line (CVL) instead of from a peripheral stick. Condition or disease Intervention/treatment Hemophilia A Factor VIII Deficiency Hemophilia Hemophilia B Factor IX Deficiency Procedure: Peripheral Vein Blood draw Detailed Description: Patients with hemophilia A and B sometimes require the placement of a central venous line (CVL). A CVL is a medical device that is placed into a vein that gives easier access to a vein either for a blood draw or to give factor replacement product. Patients (...) A Study Comparing Factor Level and Inhibitor Titer Testing Results Drawn From Central Venous Lines and Venipuncture A Study Comparing Factor Level and Inhibitor Titer Testing Results Drawn From Central Venous Lines and Venipuncture - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number

2015 Clinical Trials

135. Tissue Factor Pathway Inhibitor (TFPI) and Haemorrhagic Manifestations in Haemophilia A and B Patients

to a factor VIII (FVIII) deficiency and Haemophilia B (15 % of cases) due to factor IX (FIX) deficiency. According to the intensity of the defect, there are three forms of haemophilia: severe (FVIII or FIX lower than 1 %), moderate (factor level between 1 and 5 %), minor (factor level between 5 and 40 %). For a same level of factor VIII or IX, hemorrhagic manifestations are variable from one patient to the other. Moreover, several studies showed that haemophilic B patients bleed less and consume fewer (...) by the amplification pathway being strongly altered because of factor VIII or IX deficiency, thrombin generation (via Xa) comes exclusively from TFPI regulated tissue factor pathway. We can thus say that if haemophilic patients bleed, it is also because of the presence of TFPI that inhibits at the same time Xa and the complex TF-VIIa as soon as factor Xa is generated. Condition or disease Intervention/treatment Hemophilia Other: blood specimen Study Design Go to Layout table for study information Study Type

2015 Clinical Trials

136. The carboxyl-terminal region is NOT essential for secreted and functional levels of coagulation factor X. (Abstract)

The carboxyl-terminal region is NOT essential for secreted and functional levels of coagulation factor X. The homologous coagulation factor X (FX), VII (FVII), IX (FIX) and protein C (PC) display striking differences in the carboxyl-terminus, with that of FX being the most extended. This region is essential for FVII, FIX and PC secretion.To provide experimental evidence for the role of the FX carboxyl-terminus.Recombinant FX (rFX) variants were expressed in multiple eukaryotic cell systems (...) contribution to pro-coagulant properties. These findings, which might suggest an involvement of the carboxyl-terminal region in the divergence of the homologous FX, FVII, FIX and PC, help to interpret the mutational pattern of FX deficiency.© 2015 International Society on Thrombosis and Haemostasis.

2015 Journal of Thrombosis and Haemostasis

137. Preparation of factor VII concentrate using CNBr-activated Sepharose 4B immunoaffinity chromatography Full Text available with Trip Pro

Preparation of factor VII concentrate using CNBr-activated Sepharose 4B immunoaffinity chromatography Factor VII concentrates are used in patients with congenital or acquired factor VII deficiency or treatment of hemophilia patients with inhibitors. In this research, immunoaffinity chromatography was used to purify factor VII from prothrombin complex (Prothrombin- Proconvertin-Stuart Factor-Antihemophilic Factor B or PPSB) which contains coagulation factors II, VII, IX and X. The aim (...) of this study was to improve purity, safety and tolerability as a highly purified factor VII concentrate.PPSB was prepared using DEAE-Sephadex and was used as the starting material for purification of coagulation factor VII. Prothrombin complex was treated by solvent/detergent at 24°C for 6 h with constant stirring. The mixture of PPSB in the PBS buffer was filtered and then chromatographed using CNBr-activated Sepharose 4B coupled with specific antibody. Factors II, IX, VII, X and VIIa were assayed

2015 Medical journal of the Islamic Republic of Iran

138. Attention Deficit Disorder

Attention Deficit Disorder Attention Deficit Disorder Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Attention Deficit Disorder (...) Attention Deficit Disorder Aka: Attention Deficit Disorder , Attention Deficit Hyperactivity Disorder , ADHD , Attentional Disorders , Attention Deficit Disorder in Children From Related Chapters II. Epidemiology Diagnosed at one point in up to 11% of school children ages 4 to 17 years old (1-2 per classroom) At a given time 8.8% of school children have the diagnosis and 6.1% are on medication for Attention Deficit More commonly diagnosed in boys than girls (4 - 8:1) However girls are more often

2015 FP Notebook

139. Protein C Deficiency

SOCIAL MEDIA Add to Any Platform Loading , MD, Baylor College of Medicine Click here for Patient Education NOTE: This is the Professional Version. CONSUMERS: Because activated protein C degrades coagulation factors Va and VIIIa, deficiency of protein C predisposes to venous thrombosis. (See also .) Protein C is a vitamin K–dependent protein, as are coagulation factors VII, IX, and X; prothrombin; and proteins S and Z. Because activated protein C (APC) degrades factors Va and VIIIa, APC is a natural (...) Protein C Deficiency Protein C Deficiency - Hematology and Oncology - MSD Manual Professional Edition Brought to you by The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases / / / / IN THIS TOPIC OTHER TOPICS IN THIS CHAPTER Test your knowledge Multiple Myeloma

2013 Merck Manual (19th Edition)

140. Vitamin Deficiency, Dependency, and Toxicity - Vitamin K

K, containing about 2.5 mcg/L (cow’s milk contains 5000 mcg/L). The neonatal gut is sterile during the first few days of life. In adults , vitamin K deficiency can result from Fat malabsorption (eg, due to , , , or resection of the small intestine) Use of coumarin anticoagulants Coumarin anticoagulants interfere with the synthesis of vitamin–K dependent coagulation proteins (factors II, VII, IX, and X) in the liver. Certain antibiotics (particularly some cephalosporins and other broad-spectrum (...) supplemental vitamin K. Vitamin K 2 refers to a group of compounds (menaquinones) synthesized by bacteria in the intestinal tract; the amount synthesized does not satisfy the vitamin K requirement. Vitamin K controls the formation of coagulation factors II (prothrombin), VII, IX, and X in the liver (see Table: ). Other coagulation factors dependent on vitamin K are protein C, protein S, and protein Z; proteins C and S are anticoagulants. Metabolic pathways conserve vitamin K. Once vitamin K has

2013 Merck Manual (19th Edition)

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