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.

1,633 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

21. 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

22. Overestimation of N9-GP (N-glycoPEGylated FIX) in one-stage factor IX clotting method due to silica-mediated precocious conversion to factor IXa. (Abstract)

Overestimation of N9-GP (N-glycoPEGylated FIX) in one-stage factor IX clotting method due to silica-mediated precocious conversion to factor IXa. Essentials Nonacog beta pegol (N9-GP) activity is overestimated in clot method using silica-based reagents. Mimicking contact activation phase with silica reveals N9-GP activation before recalcification. Localization of N9-GP to silica facilitates activation by factor XIa and plasma kallikrein. Silica-based reagents to be used with caution when (...) monitoring N9-GP therapy using clot method.Background Clinical laboratories routinely quantify factor IX (FIX) activity by measurement of the activated partial thromboplastin time (APTT) in a one-stage (OS) clotting assay. This assay can be performed with any of a plethora of differently composed APTT reagents, giving variable recovery when applied to nonacog beta pegol (N9-GP), an N-glycoPEGylated recombinant FIX. Objective To identify the cause of observed overestimations of N9-GP activity in an OS FIX

2016 Journal of Thrombosis and Haemostasis

23. The transformation of hemostatic platelet plugs in normal and Factor IX deficient dogs. Full Text available with Trip Pro

The transformation of hemostatic platelet plugs in normal and Factor IX deficient dogs. 5675266 1968 10 28 2018 11 13 0002-9440 53 3 1968 Sep The American journal of pathology Am. J. Pathol. The transformation of hemostatic platelet plugs in normal and Factor IX deficient dogs. 355-73 Hovig T T Dodds W J WJ Rowsell H C HC Mustard J F JF eng Journal Article United States Am J Pathol 0370502 0002-9440 9001-31-4 Fibrin AIM IM Animals Blood Coagulation Blood Platelets Cell Membrane Permeability (...) Cytoplasm Dogs Fibrin Hemophilia A etiology pathology Hemophilia B pathology Microscopy, Electron Organoids Time Factors 1968 9 1 1968 9 1 0 1 1968 9 1 0 0 ppublish 5675266 PMC2013462 Blood. 1966 Feb;27(2):167-86 5901983 Am J Pathol. 1967 Nov;51(5):681-719 4168026 Blood. 1967 Nov;30(5):636-68 6073859 Acta Med Scand. 1958 Nov 27;162(5):361-74 13605613 Br J Haematol. 1960 Jul;6:259-66 13727144 Acta Pathol Microbiol Scand. 1964;60:55-82 14114329 Br J Exp Pathol. 1964 Oct;45:467-74 14213053 Acta Pathol

1968 The American journal of pathology

24. Thrombin generation assay using factor XIa to measure factors VIII and IX and their glycoPEGylated derivatives is robust and sensitive. (Abstract)

as a possible method for characterizing bleeding phenotypes in individuals with hemophilia.This study assessed the robustness and sensitivity of the TGA for measuring the activity of recombinant factor VIII (rFVIII), recombinant factor IX (rFIX) and their glycoPEGylated derivatives, N8-GP and N9-GP, in vitro.Factor-deficient plasma was spiked with 0.13-130 IU dL(-1) rFVIII or N8-GP (hemophilia A [HA] plasma), or rFIX or N9-GP (hemophilia B [HB] plasma). A calibrated automated thrombogram triggered (...) Thrombin generation assay using factor XIa to measure factors VIII and IX and their glycoPEGylated derivatives is robust and sensitive. Conventional coagulation factor assays are associated with certain limitations, as they do not always reflect the clinical heterogeneity of bleeding in hemophilic patients or correctly reflect the individual patient response to treatment with bypassing agents or novel factor concentrates. The thrombin generation assay (TGA) is currently being assessed

2015 Journal of Thrombosis and Haemostasis

25. Everolimus in Castrated Resistant Prostate Cancer(CRPC)Patients With PI3K-AKT-mTOR Signaling Pathway Deficiency

Everolimus in Castrated Resistant Prostate Cancer(CRPC)Patients With PI3K-AKT-mTOR Signaling Pathway Deficiency Everolimus in Castrated Resistant Prostate Cancer(CRPC)Patients With PI3K-AKT-mTOR Signaling Pathway Deficiency - 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. Everolimus in Castrated Resistant Prostate Cancer(CRPC)Patients With PI3K-AKT-mTOR Signaling Pathway Deficiency 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. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov

2018 Clinical Trials

26. Vitamin K deficiency: a case report and review of current guidelines Full Text available with Trip Pro

Vitamin K deficiency: a case report and review of current guidelines Vitamin K, a fat soluble vitamin, is a necessary cofactor for the activation of coagulation factors II, VII, IX, X, and protein C and S. In neonatal period, vitamin K deficiency may lead to Vitamin K Deficiency Bleeding (VKDB).We present the case of a 2 months and 20 days Caucasian male, presented for bleeding from the injections sites of vaccines. At birth oral vitamin K prophylaxis was administered. Neonatal period (...) was normal. He was exclusively breastfed and received a daily oral supplementation with 25 μg of vitamin K. A late onset vitamin K deficiency bleeding was suspected. Intravenous Vitamin K was administered with complete recovery.Nevertheless the oral prophylaxis, our case developed a VKDB: it is necessary to revise the current guidelines in order to standardize timing and dosage in different clinical conditions.

2018 Italian journal of pediatrics

27. Alloimmunization in Congenital Deficiencies of Platelet Surface Glycoproteins: Focus on Glanzmann's Thrombasthenia and Bernard-Soulier's Syndrome. (Abstract)

Alloimmunization in Congenital Deficiencies of Platelet Surface Glycoproteins: Focus on Glanzmann's Thrombasthenia and Bernard-Soulier's Syndrome. Glanzmann's thrombasthenia (GT) and Bernard-Soulier's syndrome (BSS) are well-understood congenital bleeding disorders, showing defect/deficiency of platelet glycoprotein (GP) IIb/IIIa (integrin αIIbβ3) and GPIb-IX-V complexes respectively, with relevant clinical, laboratory, biochemical, and genetic features. Following platelet transfusion, affected (...) patients may develop antiplatelet antibodies (to human leukocyte antigen [HLA], and/or αIIbβ3 in GT or GPIb-IX in BSS), which may render future platelet transfusion ineffective. Anti-αIIbβ3 and anti-GPIb-IX may also cross the placenta during pregnancy to cause thrombocytopenia and bleeding in the fetus/neonate. This review will focus particularly on the better studied GT to illustrate the natural history and complications of platelet alloimmunization. BSS will be more briefly discussed. Platelet

2018 Seminars In Thrombosis And Hemostasis

28. Attention Deficit Hyperactivity Disorder: Diagnosis and Treatment in Children and Adolescents

Attention Deficit Hyperactivity Disorder: Diagnosis and Treatment in Children and Adolescents Comparative Effectiveness Review Number 203 Attention Deficit Hyperactivity Disorder: Diagnosis and Treatment in Children and Adolescents eComparative Effectiveness Review Number 203 Attention Deficit Hyperactivity Disorder: Diagnosis and Treatment in Children and Adolescents Prepared for: Agency for Healthcare Research and Quality U.S. Department of Health and Human Services 5600 Fishers Lane (...) , Ph.D. AHRQ Publication No. 18-EHC005-EF January 2018 ii Key Messages Purpose of Review To update a previous review by comparing strategies to diagnose, treat, and monitor children and adolescents with attention deficit hyperactivity disorder (ADHD). Key Messages • Evidence was insufficient on imaging or electroencephalogram to diagnose ADHD in children 7–17 years of age. • Little evidence adds to the 2011 report that found that methylphenidate is effective for children under age 6 with ADHD

2018 Effective Health Care Program (AHRQ)

29. Baboon envelope pseudotyped lentiviral vectors transduce efficiently human B cells and allow active factor IX B cell secretion in vivo in NOD/SCID mice. Full Text available with Trip Pro

transfer of BaEV-LV-transduced mature B cells into NOD/SCID/γc-/- (NSG) [non-obese diabetic (NOD), severe combined immuno-deficiency (SCID)] mice allowed differentiation into plasmablasts and plasma B cells, confirming a sustained high-level gene marking in vivo. As proof of principle, we assessed BaEV-LV for transfer of human factor IX (hFIX) into B cells. BaEV-LVs encoding FIX efficiently transduced hB cells and their transfer into NSG mice demonstrated for the first time secretion of functional hFIX (...) Baboon envelope pseudotyped lentiviral vectors transduce efficiently human B cells and allow active factor IX B cell secretion in vivo in NOD/SCID mice. Essentials B cells are attractive targets for gene therapy and particularly interesting for immunotherapy. A baboon envelope pseudotyped lentiviral vector (BaEV-LV) was tested for B-cell transduction. BaEV-LVs transduced mature and plasma human B cells with very high efficacy. BaEV-LVs allowed secretion of functional factor IX from B cells

2016 Journal of Thrombosis and Haemostasis

30. CRISPR/Cas9‐mediated somatic correction of a novel coagulator factor IX gene mutation ameliorates hemophilia in mouse Full Text available with Trip Pro

CRISPR/Cas9‐mediated somatic correction of a novel coagulator factor IX gene mutation ameliorates hemophilia in mouse The X-linked genetic bleeding disorder caused by deficiency of coagulator factor IX, hemophilia B, is a disease ideally suited for gene therapy with genome editing technology. Here, we identify a family with hemophilia B carrying a novel mutation, Y371D, in the human F9 gene. The CRISPR/Cas9 system was used to generate distinct genetically modified mouse models and confirmed

2016 EMBO molecular medicine

31. A two-component system regulates gene expression of the type IX secretion component proteins via an ECF sigma factor Full Text available with Trip Pro

A two-component system regulates gene expression of the type IX secretion component proteins via an ECF sigma factor The periodontopathogen Porphyromonas gingivalis secretes potent pathogenic proteases, gingipains, via the type IX secretion system (T9SS). This system comprises at least 11 components; however, the regulatory mechanism of their expression has not yet been elucidated. Here, we found that the PorY (PGN_2001)-PorX (PGN_1019)-SigP (PGN_0274) cascade is involved in the regulation (...) deficient in a putative extracytoplasmic function (ECF) sigma factor, PGN_0274 (SigP), similar to the porX mutant. Electrophoretic gel shift assays showed that rSigP bound to the putative promoter regions of T9SS component-encoding genes. The SigP protein was lacking in the porX mutant. Co-immunoprecipitation and SPR analysis revealed the direct interaction between SigP and PorX. Together, these results indicate that the PorXY TCS regulates T9SS-mediated protein secretion via the SigP ECF sigma factor.

2016 Scientific reports

32. Ultrasound-targeted hepatic delivery of factor IX in hemophiliac mice Full Text available with Trip Pro

Ultrasound-targeted hepatic delivery of factor IX in hemophiliac mice Ultrasound-targeted microbubble destruction (UTMD) was used to direct the delivery of plasmid and transposase-based vectors encoding human factor IX (hFIX) to the livers of hemophilia B (FIX-/-) mice. The DNA vectors were incorporated into cationic lipid microbubbles, injected intravenously, and transfected into hepatocytes by acoustic cavitation of the bubbles as they transited the liver. Ultrasound parameters were (...) identified that produced transfection of hepatocytes in vivo without substantial damage or bleeding in the livers of the FIX-deficient mice. These mice were treated with a conventional expression plasmid, or one containing a piggyBac transposon construct, and hFIX levels in the plasma and liver were evaluated at multiple time points after UTMD. We detected hFIX in the plasma by western blotting from mice treated with either plasmid during the 12 days after UTMD, and in the hepatocytes of treated livers

2016 Gene therapy

33. Preliminary study on non-viral transfection of F9 (factor IX) gene by nucleofection in human adipose-derived mesenchymal stem cells Full Text available with Trip Pro

Preliminary study on non-viral transfection of F9 (factor IX) gene by nucleofection in human adipose-derived mesenchymal stem cells Background. Hemophilia is a rare recessive X-linked disease characterized by a deficiency of coagulation factor VIII or factor IX. Its current treatment is merely palliative. Advanced therapies are likely to become the treatment of choice for the disease as they could provide a curative treatment. Methods. The present study looks into the use of a safe non-viral (...) transfection method based on nucleofection to express and secrete human clotting factor IX (hFIX) where human adipose tissue derived mesenchymal stem cells were used as target cells in vitro studies and NOD. Cg-Prkdcscid Il2rgtm1Wjl/SzJ mice were used to analyze factor IX expression in vivo studies. Previously, acute liver injury was induced by an injected intraperitoneal dose of 500 mg/kg body weight of acetaminophen. Results. Nucleofection showed a percentage of positive cells ranging between 30.7

2016 PeerJ

34. Single synonymous mutation in factor IX alters protein properties and underlies haemophilia B. Full Text available with Trip Pro

Single synonymous mutation in factor IX alters protein properties and underlies haemophilia B. Haemophilia B is caused by genetic aberrations in the F9 gene. The majority of these are non-synonymous mutations that alter the primary structure of blood coagulation factor IX (FIX). However, a synonymous mutation c.459G>A (Val107Val) was clinically reported to result in mild haemophilia B (FIX coagulant activity 15%-20% of normal). The F9 mRNA of these patients showed no skipping or retention (...) of introns and/or change in mRNA levels, suggesting that mRNA integrity does not contribute to the origin of the disease in affected individuals. The aim of this study is to elucidate the molecular mechanisms that can explain disease manifestations in patients with this synonymous mutation.We analyse the molecular mechanisms underlying the FIX deficiency through in silico analysis and reproducing the c.459G>A (Val107Val) mutation in stable cell lines. Conformation and non-conformation sensitive

2016 Journal of Medical Genetics

35. Laboratory Diagnosis of Functional Iron Deficiency

ferritin values), a Ret‐He cut‐off of 25 pg may also help to distinguish iron deficiency (values <25 pg) from ACD (values >25 pg). A Ret‐He cut‐off value of 30·6 pg is a better predictor of response to intravenous iron than baseline serum ferritin or transferrin saturation values in CKD patients undergoing thrice‐weekly haemodialysis (Buttarello et al , ). Red blood cell size factor (Rsf) This variable is derived from the square root of the product of the MCVs of mature RBC and reticulocytes. It shows (...) being this technique remains a research investigation. Functional iron deficiency and the anaemia of chronic disease The failure of adequate iron incorporation into the developing erythron is just one component of ACD and cancer‐related anaemia. With these disorders the actions of γ‐interferon, transforming growth factor‐β and tumour necrosis factor produce a down‐regulation of the erythron, early erythroid precursor cell death and reduced Epo sensitivity. Increased levels of both hepcidin and IL6

2013 British Committee for Standards in Haematology

36. Riemerella anatipestifer Type IX Secretion System Is Required for Virulence and Gelatinase Secretion Full Text available with Trip Pro

Riemerella anatipestifer Type IX Secretion System Is Required for Virulence and Gelatinase Secretion Riemerella anatipestifer (RA), a major causative agent of septicemia anserum exsudativa in domesticated ducklings, has a protein secretion system known as the type IX secretion system (T9SS). It is unknown whether the T9SS contributes to the virulence of RA through secretion of factors associated with pathogenesis. To answer this question, we constructed an RA mutant deficient in sprT, which (...) -like serine proteases which are important virulence factors that interact with complement proteins may enable RA to evade immune surveillance in the avian innate immune system.

2017 Frontiers in microbiology

37. The Type IX Secretion System Is Required for Virulence of the Fish Pathogen Flavobacterium columnare Full Text available with Trip Pro

The Type IX Secretion System Is Required for Virulence of the Fish Pathogen Flavobacterium columnare Flavobacterium columnare, a member of the phylum Bacteroidetes, causes columnaris disease in wild and aquaculture-reared freshwater fish. The mechanisms responsible for columnaris disease are not known. Many members of the phylum Bacteroidetes use type IX secretion systems (T9SSs) to secrete enzymes, adhesins, and proteins involved in gliding motility. The F. columnare genome has all (...) of the genes needed to encode a T9SS. gldN, which encodes a core component of the T9SS, was deleted in wild-type strains of F. columnare The F. columnare ΔgldN mutants were deficient in the secretion of several extracellular proteins and lacked gliding motility. The ΔgldN mutants exhibited reduced virulence in zebrafish, channel catfish, and rainbow trout, and complementation restored virulence. PorV is required for the secretion of a subset of proteins targeted to the T9SS. An F. columnare ΔporV mutant

2017 Applied and environmental microbiology

38. Natural History Study of Factor IX Treatment and Complications

, Inc. Biogen/Bioverativ Swedish Orphan Biovitrum Information provided by (Responsible Party): Sharyne M. Donfield, Ph.D., Skane University Hospital Study Details Study Description Go to Brief Summary: This study will examine two groups of subjects with factor IX (FIX) deficiency: 1) those with a current or history of inhibitors to FIX, and; 2) groups of two or more affected brothers, with or without inhibitors. The overall goal is to characterize the study groups in terms of their medical history (...) , their patterns of bleeding, their care, quality of life, and complications including the development of joint disease, inhibitory antibodies to FIX, use of immune tolerance induction (ITI) and outcome. Condition or disease Intervention/treatment Factor IX Deficiency Other: Standard care with blood and urine sample collection Detailed Description: Hemophilia B, FIX deficiency, is the second most common type of hemophilia, occurring in about one in 25,000 male births. This disease is in some ways more complex

2015 Clinical Trials

39. Rate-limiting roles of tenase complex of factors VIII and IX in platelet procoagulant activity and formation of platelet-fibrin thrombi under flow. Full Text available with Trip Pro

Rate-limiting roles of tenase complex of factors VIII and IX in platelet procoagulant activity and formation of platelet-fibrin thrombi under flow. The importance of factor Xa generation in thrombus formation has not been studied extensively so far. Here, we used mice deficient in either factor VIII or factor IX to determine the role of platelet-stimulated tenase activity in the formation of platelet-fibrin thrombi on collagen. With tissue factor present, deficiency in factor VIII or IX (...) markedly suppressed thrombus growth, fibrin formation and platelet procoagulant activity (phosphatidylserine exposure). In either case, residual fibrin formation was eliminated in the absence of tissue factor. Effects of factor deficiencies were antagonized by supplementation of the missing coagulation factor. In wild-type thrombi generated under flow, phosphatidylserine-exposing platelets bound (activated) factor IX and factor X, whereas factor VIII preferentially co-localized at sites of von

2015 Haematologica

40. Epac1-deficient mice have bleeding phenotype and thrombocytes with decreased GPIbβ expression Full Text available with Trip Pro

revealed that Epac1-/- mouse platelets (thrombocytes) had unbalanced expression of key components of the glycoprotein Ib-IX-V (GPIb-IX-V) complex, with decrease of GP1bβ and no change of GP1bα. This complex is critical for platelet adhesion under arterial shear conditions. Furthermore, Epac1-/- mice have reduced levels of plasma coagulation factors and fibrinogen, increased size of circulating platelets, increased megakaryocytes (the GP1bβ level was decreased also in Epac1-/- bone marrow) and higher (...) abundance of reticulated platelets. Viscoelastic measurement of clotting function revealed Epac1-/- mice with a dysfunction in the clotting process, which corresponds to reduced plasma levels of coagulation factors like factor XIII and fibrinogen. We propose that the observed platelet phenotype is due to deficient Epac1 activity during megakaryopoiesis and thrombopoiesis, and that the defects in blood clotting for Epac1-/- is connected to secondary hemostasis.

2017 Scientific reports

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