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Factor IX Deficiency

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61. Factor IX (Diagnosis)

Factor IX (Diagnosis) Factor IX Deficiency (Hemophilia B): 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTk5MDg4LW92ZXJ2aWV3 processing > Factor IX Deficiency (...) (Hemophilia B) Updated: Apr 02, 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP Share Email Print Feedback Close Sections Sections Factor IX Deficiency (Hemophilia B) Overview Practice Essentials Factor IX (FIX) deficiency or dysfunction, or hemophilia B, is an X-linked inherited bleeding disorder, usually manifested in males and transmitted by females who carry the causative mutation on the X chromosome. Hemophilia B results from a variety of defects in the FIX

2014 eMedicine.com

62. Factor XI Deficiency (Overview)

. Zivelin A, Bauduer F, Ducout L, et al. Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene. Blood . 2002 Apr 1. 99(7):2448-54. . Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies (...) Factor XI Deficiency (Overview) Factor XI Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LW92ZXJ2aWV3 processing > Factor XI Deficiency Updated: May 13

2014 eMedicine.com

63. Factor XI Deficiency (Treatment)

XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies. of 1 Tables Contributor Information and Disclosures Author Jamie E Siegel, MD Director, Cardeza Foundation Hemophilia Treatment Center, Thomas Jefferson University Jamie E Siegel, MD is a member of the following medical societies (...) Factor XI Deficiency (Treatment) Factor XI Deficiency Treatment & Management: Approach Considerations 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LXRyZWF0bWVudA== processing > Factor XI Deficiency

2014 eMedicine.com

64. Factor XI Deficiency (Follow-up)

. . Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies. of 1 Tables Contributor Information and Disclosures Author Jamie E Siegel, MD Director, Cardeza Foundation Hemophilia Treatment Center, Thomas Jefferson University Jamie E Siegel, MD is a member (...) Factor XI Deficiency (Follow-up) Factor XI Deficiency Follow-up: Prognosis, Patient Education 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LWZvbGxvd3Vw processing > Factor XI Deficiency Follow-up Updated

2014 eMedicine.com

65. Factor XI Deficiency (Diagnosis)

. Zivelin A, Bauduer F, Ducout L, et al. Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene. Blood . 2002 Apr 1. 99(7):2448-54. . Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies (...) Factor XI Deficiency (Diagnosis) Factor XI Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LW92ZXJ2aWV3 processing > Factor XI Deficiency Updated: May

2014 eMedicine.com

66. Factor VII Deficiency (Follow-up)

. Korean J Hematol . 2012 Dec. 47(4):281-5. . . Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts (...) Factor VII Deficiency (Follow-up) Pediatric Factor VII Deficiency Follow-up: Further Outpatient Care, Deterrence/Prevention, Complications 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTkyLWZvbGxvd3Vw

2014 eMedicine Pediatrics

67. Factor VII Deficiency (Treatment)

in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin. of 1 Tables Contributor Information and Disclosures Author Helge Dirk Hartung, MD Attending Physician, Division of Hematology (...) Factor VII Deficiency (Treatment) Pediatric Factor VII Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTkyLXRyZWF0bWVudA

2014 eMedicine Pediatrics

68. Factor XIII Deficiency (Overview)

with mild to moderate bleeding disorders found that out of 418 individuals with these conditions, just one (0.2%) had factor XIII deficiency. Factor VIII, IX, and XI deficiencies occurred in 11 (2.6%), 3 (0.7%), and 3 (0.7%) patients, respectively. [ ] Mortality/Morbidity The mortality and morbidity are primarily related to bleeding; intracranial hemorrhage can be life threatening. The spontaneous abortion rate in women with severe factor XIII deficiency approaches 80%. Sex This is an autosomal (...) Factor XIII Deficiency (Overview) Pediatric Factor XIII Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTE1LW92ZXJ2aWV3 processing > Pediatric Factor XIII

2014 eMedicine Pediatrics

69. Factor VII Deficiency (Overview)

and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin. Previous Next: Epidemiology Frequency United States Inherited factor VII deficiency is rare. Incidence is 1 case per 500,000 population. International The frequency is higher in countries where consanguineous marriage is more common. For example, the reported (...) with congenital factor VII deficiency: A single center experience. Korean J Hematol . 2012 Dec. 47(4):281-5. . . Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation

2014 eMedicine Pediatrics

70. Factor XIII Deficiency (Diagnosis)

with mild to moderate bleeding disorders found that out of 418 individuals with these conditions, just one (0.2%) had factor XIII deficiency. Factor VIII, IX, and XI deficiencies occurred in 11 (2.6%), 3 (0.7%), and 3 (0.7%) patients, respectively. [ ] Mortality/Morbidity The mortality and morbidity are primarily related to bleeding; intracranial hemorrhage can be life threatening. The spontaneous abortion rate in women with severe factor XIII deficiency approaches 80%. Sex This is an autosomal (...) Factor XIII Deficiency (Diagnosis) Pediatric Factor XIII Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTE1LW92ZXJ2aWV3 processing > Pediatric Factor XIII

2014 eMedicine Pediatrics

71. Factor VII Deficiency (Diagnosis)

and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin. Previous Next: Epidemiology Frequency United States Inherited factor VII deficiency is rare. Incidence is 1 case per 500,000 population. International The frequency is higher in countries where consanguineous marriage is more common. For example, the reported (...) with congenital factor VII deficiency: A single center experience. Korean J Hematol . 2012 Dec. 47(4):281-5. . . Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation

2014 eMedicine Pediatrics

72. Involvement of the Type IX Secretion System in Capnocytophaga ochracea Gliding Motility and Biofilm Formation Full Text available with Trip Pro

Involvement of the Type IX Secretion System in Capnocytophaga ochracea Gliding Motility and Biofilm Formation Capnocytophaga ochracea is a Gram-negative, rod-shaped bacterium that demonstrates gliding motility when cultured on solid agar surfaces. C. ochracea possesses the ability to form biofilms; however, factors involved in biofilm formation by this bacterium are unclear. A type IX secretion system (T9SS) in Flavobacterium johnsoniae was shown to be involved in the transport of proteins (e.g (...) ., several adhesins) to the cell surface. Genes orthologous to those encoding T9SS proteins in F. johnsoniae have been identified in the genome of C. ochracea; therefore, the T9SS may be involved in biofilm formation by C. ochracea. Here we constructed three ortholog-deficient C. ochracea mutants lacking sprB (which encodes a gliding motility adhesin) or gldK or sprT (which encode T9SS proteins in F. johnsoniae). Gliding motility was lost in each mutant, suggesting that, in C. ochracea, the proteins

2016 Applied and environmental microbiology

73. Voncento - human coagulation factor VIII / human von willebrand factor

. VWF and FVIII are two distinct glycoproteins that circulate in plasma in the form of a non-covalently bound complex. Both factors are essential for normal haemostasis in humans. Deficiencies in FVIII or VWF lead to two distinct hereditary coagulation disorders: haemophilia A and von Willebrand disease (VWD). Voncento belongs to the pharmacological class of hemostatic agents. The product is administered by intravenous infusion to raise FVIII and VWF levels in patients with corresponding (...) Voncento - human coagulation factor VIII / human von willebrand factor 11 June 2013 EMA/404213/2013 Committee for Medicinal Products for Human Use (CHMP) Assessment report Voncento HUMAN COAGULATION FACTOR VIII / VON WILLEBRAND FACTOR Procedure No. EMEA/H/C/002493/0000 Note Assessment report as adopted by the CHMP with all information of a commercially confidential nature deleted. 7 Westferry Circus ? Canary Wharf ? London E14 4HB ? United Kingdom An agency of the European Union Telephone +44

2013 European Medicines Agency - EPARs

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

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

2012 Clinical Trials

75. Open-Label Single Ascending Dose of Adeno-associated Virus Serotype 8 Factor IX Gene Therapy in Adults With Hemophilia B

Detailed Description: Hemophilia B is a genetic X-linked bleeding disorder caused by a deficiency in blood-clotting Factor IX (FIX) activity. FIX is synthesized in the liver and circulates in the blood as a proenzyme. Current treatment for hemophilia B is based on replacement of the deficient FIX with IV injections of recombinant FIX protein prophylactically or as needed to treat bleeding episodes. This clinical program will test a gene transfer approach involving the use of a gene delivery vector (...) Studies a U.S. FDA-regulated Device Product: No Keywords provided by Shire ( Baxalta now part of Shire ): Hemophilia B factor IX deficiency gene therapy Additional relevant MeSH terms: Layout table for MeSH terms Hemophilia A Hemophilia B Blood Coagulation Disorders, Inherited Blood Coagulation Disorders Hematologic Diseases Coagulation Protein Disorders Hemorrhagic Disorders Genetic Diseases, Inborn Genetic Diseases, X-Linked

2012 Clinical Trials

76. Coagulation Factor IX for Hemophilia B Therapy
 Full Text available with Trip Pro

Coagulation Factor IX for Hemophilia B Therapy
 Factor IX is a zymogen enzyme of the blood coagulation cascade. Inherited absence or deficit of the IX functional factor causes bleeding disorder hemophilia B, which requires constant protein replacement therapy. Reviewed herein are the current state in the manufacturing of FIX, improved variants of the recombinant protein for therapy, transgenic organisms for obtaining FIX, and the advances in the gene therapy of hemophilia B.

2012 Acta naturae

77. 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

78. Surgical Operation in Hemophilia B—Use of Factor IX Concentrate Full Text available with Trip Pro

Surgical Operation in Hemophilia B—Use of Factor IX Concentrate A concentrate containing plasma clotting factors II, VII, IX and X was used to secure hemostasis for a herniorrhaphy, an osteotomy of a femur, a cup arthroplasty of a hip, and a tonsillectomy in patients with factor IX deficiency. After single infusions of concentrate, the net increase in plasma factor IX activity was 0.7 to 1.0 percent for each in-vitro unit of factor IX infused per kilogram of body weight. After large infusions (...) of concentrate in two patients, the disappearance pattern of factor IX had two phases: a first component with half-disappearance times of 4.4 and 6 hours, and a second component with half-disappearance times of 26 and 32.6 hours.

1970 California Medicine

79. 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

80. Combined Antihaemophilic Globulin and Christmas Factor Deficiency in Haemophilia Full Text available with Trip Pro

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

1955 British medical journal

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