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Factor IX Deficiency

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81. Demonstrable Deficiency of Christmas Factor in Two Sisters Full Text available with Trip Pro

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

1960 British medical journal

82. Reactions of Blood with Nonbiologic Surfaces: Ultrastructural and Clotting Studies with Normal and Coagulation Factor Deficient Bloods Full Text available with Trip Pro

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

1972 The American journal of pathology

83. RISK FACTORS OF HEPATITIS B IN PATIENTS ON HEMODIALYSIS: A SYSTEMATIC REVIEW Full Text available with Trip Pro

RISK FACTORS OF HEPATITIS B IN PATIENTS ON HEMODIALYSIS: A SYSTEMATIC REVIEW ??? 4, ? 2 1 ??????????????? ? ??????????? ???????? ® (...) . Maleva, Olga A. Trubnikova, Olga L. Barbarash STATINS IN PREVENTION OF POSTOPERATIVE COGNITIVE DYSFUNCTION IN PATIENTS AFTER CARDIAC SURGERY (Kemerovo, Russian Federation) Daria V . Kvashnina, Olga V . Kovalishena, Nikolay V . Saperkin RISK FACTORS OF HEPATITIS B IN PATIENTS ON HEMODIALYSIS: A SYSTEMATIC REVIEW (Nizhniy Novgorod, Russian Federation; Utrecht, Netherlands) Natalia V . Tapeshkina, Larisa P. Pochueva, Oksana P. Vlasova ORGANIZING NUTRITION OF SCHOOLCHILDREN: PROBLEMS AND SOLUTIONS

2019 Fundamental and Clinical Medicine

84. MASAC Statement Regarding Use of Various Clotting Factor Assays to Monitor Factor Replacement Therapy

, magnifying limitations with the assays currently available for monitoring replacement therapy. It has been widely recognized that there is marked variability between factor VIII and factor IX one-stage assays performed in different laboratories, owing to the numerous combinations of aPTT reagents, instruments, calibration standards, and factor- deficient plasmas available. In addition, the variability among the aPTT-based assays is greater when attempting to measure factor levels near the lower limits (...) of the assay range. Moreover, in some test conditions the aPTT may not be sensitive enough to screen for mild factor VIII and factor IX deficiencies, and reliance on the one-stage aPTT assay alone may not allow accurate characterization of some forms of mild hemophilia. These problems are either minimized or not seen when chromogenic assays are performed. Chromogenic assays have become widely utilized in Europe, and the European Pharmacopoeia requires that potency of all factor products be assigned using

2014 National Hemophilia Foundation

85. Female factor IX deficiency due to maternally inherited X-inactivation. Full Text available with Trip Pro

Female factor IX deficiency due to maternally inherited X-inactivation. X-chromosome inactivation is normally a random event that is regulated by the X chromosome itself. Rarely, females are affected by X-linked disorders from extremely skewed X-chromosome inactivation. Here, we report a family with hemophilia B with female expression through inherited X skewing that appears to be independent of either X chromosome. This finding suggests the possibility of a dominant autosomal contribution

2012 Clinical Genetics

86. 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 Publication 1593

87. Recombinant factor VIIa and prothrombin complex concentrates to treat bleeding episodes in hemophilic patients with inhibitors: a Systematic Review Full Text available with Trip Pro

efficacy of the biotechnological rFVIIa manufactured in Russia, which appears to be much lower than that of rFVIIa and aPCC. Keywords: hemophilia A, hemophilia B, blood coagulation factor inhibitors, factor VIIa, generic drugs, blood coagulation Introduction Hemophilia is caused by a hereditary deficiency of factor VIII (FVIII) or IX (FIX), and it is classified as severe, moderate or mild, based on factor coagulant activity. , Treatment aim, prophylactic or on-demand, is to avoid bleedings (...) Recombinant factor VIIa and prothrombin complex concentrates to treat bleeding episodes in hemophilic patients with inhibitors: a Systematic Review Recombinant factor VIIa and prothrombin complex concentrates to treat bleeding episodes in hemophilic patients with inhibitors: a systematic review - MedCrave online Due to current COVID19 situation and as a measure of abundant precaution, our Member Services centre are operating with minimum staff eISSN: 2469-2778 Hematology & Transfusion

2018 Hematology & Transfusion International Journal

88. Systematic and meta-analysis of factors associated with preeclampsia and eclampsia in sub-Saharan Africa Full Text available with Trip Pro

among the individual factors. IX. Drinking alcohol during pregnancy. In this review, four studies investigated the relationship between maternal alcohol drinking during pregnancy and the risk of preeclampsia/eclampsia [ , , , ]. Based on this review, none of the included studies reported the quantifying level of alcohol consumption in their reports. Only one study (conducted in Ethiopia) showed a significant association between alcohol use in pregnancy and eclampsia or preeclampsia; women who drank (...) Systematic and meta-analysis of factors associated with preeclampsia and eclampsia in sub-Saharan Africa Systematic and meta-analysis of factors associated with preeclampsia and eclampsia in sub-Saharan Africa ') Advertisement Publish Submissions Policies Manuscript Review and Publication Submit Your Manuscript Discover a faster, simpler path to publishing in a high-quality journal. PLOS ONE promises fair, rigorous peer review, broad scope, and wide readership – a perfect fit for your research

2020 PloS one

89. Membrane carbonic anhydrase IX expression and relapse risk in resected stage I-II non-small-cell lung cancer. Full Text available with Trip Pro

Membrane carbonic anhydrase IX expression and relapse risk in resected stage I-II non-small-cell lung cancer. Adjuvant chemotherapy reduces recurrences of non-small-cell lung cancer (NSCLC). To determine which patients need adjuvant chemotherapy, we assessed factors associated with time to relapse (TTR).In 230 resected stage I-II NSCLCs, we correlated immunohistochemistry scores for factors associated with cell growth rate, growth regulation, hypoxia, cell survival, and cell death with TTR.With (...) a median follow-up of 82 months (1-158) for those alive and relapse free at last follow-up, median time to recurrence was not reached. The 2- and 5-year probabilities of maintaining freedom from recurrence were 80.7% (95% confidence interval, 75.3%, 86.4%) and 74.6% (95% confidence interval, 68.6%, 81.2%), respectively. TTR curves flattened at an apparent cure rate of 70%. In multicovariate Cox models, factors correlating with shorter TTR were membranous carbonic anhydrase IX (mCAIX) staining (any

2014 Journal of Thoracic Oncology

90. How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors. (Abstract)

in this pathway, most typically factors VIII, IX, and XI. The APTT is also sensitive to contact factor deficiencies, including factor XII, prekallikrein, and high-molecular-weight kininogen. The APTT may also be elevated in a variety of conditions, including liver disease, vitamin K deficiency, and disseminated intravascular coagulation. The APTT can also be used for monitoring unfractionated heparin (UFH) therapy, as well as for screening lupus anticoagulant (LA) or for assessing thrombosis risk. Which (...) How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors. The activated partial thromboplastin time (APTT) assay is a very common coagulation test, used for several reasons. The test is conventionally used for assessing the contact factor (intrinsic) pathway of blood coagulation, and thus for screening deficiencies

2019 Seminars In Thrombosis And Hemostasis

91. Effects and Interferences of Emicizumab, a Humanised Bispecific Antibody Mimicking Activated Factor VIII Cofactor Function, on Coagulation Assays. Full Text available with Trip Pro

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

2019 Thrombosis and haemostasis

92. A mutated factor X activatable by thrombin corrects bleedings in vivo in a rabbit model of antibody-induced hemophilia A. Full Text available with Trip Pro

A mutated factor X activatable by thrombin corrects bleedings in vivo in a rabbit model of antibody-induced hemophilia A. Rendering coagulation factor X sensitive to thrombin was proposed as a strategy that can bypass the need for factor VIII. In this paper, this non-replacement strategy was evaluated in vitro and in vivo in its ability to correct factor VIII but also factor IX, X and XI deficiencies. A novel modified factor X, named Actiten, was generated and produced in the HEK293F cell line (...) . The molecule possesses the required post-translational modifications, partially keeps its ability to be activated by RVV-X, factor VIIa/tissue factor, factor VIIIa/factor IXa and acquires the ability to be activated by thrombin. The potency of the molecule was evaluated in respective deficient plasmas or hemophilia A plasmas, for some with inhibitors. Actiten corrects dose dependently all the assayed deficient plasmas. It is able to normalize the thrombin generation at 20 μg/mL showing however an increased

2019 Haematologica

93. Integration-deficient Lentiviral Vectors Expressing Codon-optimized R338L Human FIX Restore Normal Hemostasis in Hemophilia B Mice Full Text available with Trip Pro

. 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

2014 Molecular Therapy

94. 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

2018 FP Notebook

95. Recombinant Factor VIIa addition to hemophilic blood perfused over collagen/tissue factor can sufficiently bypass the Factor IXa/VIIIa defect to rescue fibrin generation Full Text available with Trip Pro

Recombinant Factor VIIa addition to hemophilic blood perfused over collagen/tissue factor can sufficiently bypass the Factor IXa/VIIIa defect to rescue fibrin generation Factor VIII (FVIII) or factor IX (FIX)-deficient haemophilic patients display deficits in platelet and fibrin deposition under flow detectable in microfluidics. Compared to fibrin generation, decreased platelet deposition in haemophilic blood flow is more easily rescued with recombinant factor VIIa (rFVIIa), whereas rFVIIa (...) of 100 s-1 .With WB from healthy controls, platelet deposition and fibrin accumulation increased as TF increased. Factor-deficient WB (1-3% of normal) displayed striking deficits in platelet deposition and fibrin formation at either TFlow or TFhigh . In contrast, mildly factor-deficient WB (14-32%) supported fibrin formation under flow on TFhigh /collagen. With either TFlow or TFhigh , exogenously added rFVIIa (20 nm) increased platelet deposition and fibrin accumulation in WB from factor-deficient

2017 Haemophilia : the official journal of the World Federation of Hemophilia

96. Vitamin K Deficiency (Overview)

with the carboxylase is not fully understood. Vitamin K is required in the synthesis of 4 clotting factors in the liver: factors II,VII, IX, and X. It is also essential in the production of protein C and S, which are anticoagulant proteins. [ ] Bone matrix proteins, specifically osteocalcin, undergo gamma carboxylation with calcium much the way coagulation factors do; this process also requires VK. Previous Next: Etiology In infants, the low transmission of vitamin K (VK) across the placenta, liver prematurity (...) Parenchymal liver disease Cystic fibrosis Inflammatory bowel disease Drugs - Antibiotics (cephalosporin), cholestyramines, warfarin, salicylates, anticonvulsants, and certain sulfa drugs) are some of the common causes of VK deficiency Massive transfusion Disseminated intravascular coagulation (DIC) - Severe Chronic kidney disease/hemodialysis [ ] The synthesis of VK-dependent factors are decreased by parenchymal liver diseases, such as cirrhosis secondary to viral hepatitis, alcohol intake, and other

2014 eMedicine.com

97. Proximal Femoral Focal Deficiency (Diagnosis)

knee arthroscopy to identify changes in cruciate ligaments and their relation to the different types of PFFD in patients with Pappas type III, IV, VII, VIII, or IX deficiency, Chomiak et al found variable changes of the cruciate ligaments in all but one patient. Although these changes were not clinically relevant in most of the patients and were not related to the Pappas classification, the authors recommended imaging of cruciate ligaments before lengthening of the extremity in order to avoid knee (...) age, whereas others will be more inclined to attempt to preserve the natural limb no matter what. Treatment must be individualized on the basis of the following factors [ ] : Limb-length discrepancy Presence of associated malformations Adequacy of musculature Proximal joint stability Previous References Oppenheim WL, Setoguchi Y, Fowler E. Overview and comparison of Syme's amputation and knee fusion with the van Nes rotationplasty procedure in proximal femoral focal deficiency. Herring JA, Birch J

2014 eMedicine Surgery

98. IgA and IgG Subclass Deficiencies (Overview)

immunoglobulin (Ig), or both to lacunar deficits that involve specific antibody responses to polysaccharides. The spectrum of antibody deficiency is broad, ranging from decreased total IgG levels to normal IgG levels and from primary B-cell defects to combined immunodeficiencies with antibody abnormalities associated with other immune and often nonimmune abnormalities. Although this article discusses and hypogammaglobulinemia, the emphasis is on selective Ig deficiencies, including the decreased production (...) vaccine in children with recurrent infections who are unresponsive to the polysaccharide vaccine. Pediatr Infect Dis J . 1998 Aug. 17(8):685-91. . Meyts I, Bossuyt X, Proesmans M, De B. Isolated IgG3 deficiency in children: to treat or not to treat? Case presentation and review of the literature. Pediatr Allergy Immunol . 2006 Nov. 17(7):544-50. . Garcia-Lloret M, McGhee S, Chatila TA. Immunoglobulin replacement therapy in children. Immunol Allergy Clin North Am . 2008 Nov. 28(4):833-49, ix

2014 eMedicine Pediatrics

99. Glucuronyl Transferase Deficiency (Overview)

Glucuronyl Transferase Deficiency (Overview) Unconjugated Hyperbilirubinemia: Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTc4ODQxLW92ZXJ2aWV3 processing (...) % is derived from the hepatic turnover of heme proteins, such as myoglobin, cytochromes, and catalase. A small portion of daily bilirubin is derived from the destruction of young or developing erythroid cells. Bilirubin is poorly soluble in water at physiologic pH because of internal hydrogen bonding that engages all polar groups and gives the molecule an involuted structure. The fully hydrogen-bonded structure of bilirubin is designated bilirubin IX-alpha-ZZ. The intramolecular hydrogen bonding shields

2014 eMedicine Pediatrics

100. Vitamin K Deficiency (Diagnosis)

functions as cofactor with the carboxylase is not fully understood. Vitamin K is required in the synthesis of 4 clotting factors in the liver: factors II,VII, IX, and X. It is also essential in the production of protein C and S, which are anticoagulant proteins. [ ] Bone matrix proteins, specifically osteocalcin, undergo gamma carboxylation with calcium much the way coagulation factors do; this process also requires VK. Previous Next: Etiology In infants, the low transmission of vitamin K (VK) across (...) Infectious diarrhea Cholestatic disease Parenchymal liver disease Cystic fibrosis Inflammatory bowel disease Drugs - Antibiotics (cephalosporin), cholestyramines, warfarin, salicylates, anticonvulsants, and certain sulfa drugs) are some of the common causes of VK deficiency Massive transfusion Disseminated intravascular coagulation (DIC) - Severe Chronic kidney disease/hemodialysis [ ] The synthesis of VK-dependent factors are decreased by parenchymal liver diseases, such as cirrhosis secondary to viral

2014 eMedicine.com

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