Hemophilia B (Haemophilia B)

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1022 relevant articles (35 outcomes, 109 trials/studies) found for this Disease

Description: A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)

Also Known As:

Haemophilia B; Christmas Disease; Factor IX Deficiency; Deficiency, Factor IX; B, Haemophilia; B, Hemophilia; Bs, Haemophilia; Bs, Hemophilia; Deficiencies, Factor IX; Disease, Christmas; Factor IX Deficiencies; Haemophilia Bs; Hemophilia Bs

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases

Hematologic Diseases: 1480

Hemorrhagic Disorders: 456

Hemophilia B: 1022

Blood Coagulation Disorders: 88

Inherited Blood Coagulation Disorders: 34

Hemophilia B: 1022

Coagulation Protein Disorders

Hemophilia B: 1022

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 449

Inborn Genetic Diseases: 578

Inherited Blood Coagulation Disorders: 34

Hemophilia B: 1022

X-Linked Genetic Diseases: 12

Hemophilia B: 1022

Related Diseases

1.	Hemophilia A (Haemophilia)
2.	Hemorrhage
3.	Body Weight (Weight, Body)
4.	Hepatitis
5.	Acquired Immunodeficiency Syndrome (AIDS)

Experts

1.	Arruda, Valder R: 6 articles (09/2006 - 04/2002)
2.	High, Katherine A: 5 articles (09/2006 - 04/2002)
3.	Herzog, Roland W: 5 articles (03/2005 - 04/2002)
4.	Nichols, Timothy C: 4 articles (01/2004 - 04/2002)
5.	Nichols, T C: 4 articles (01/2004 - 02/2000)
6.	Schuettrumpf, Joerg: 3 articles (03/2005 - 01/2004)
7.	Bellinger, D A: 3 articles (01/2004 - 02/2000)
8.	Read, M S: 3 articles (03/2001 - 02/2000)
9.	High, K A: 3 articles (01/2001 - 03/2000)
10.	Okano, Teruo: 2 articles (07/2008 - 05/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hemophilia B:

1.	Factor IX (PTC)FDA LinkGeneric 01/01/1993 - "This direct approach, which is based on computer-aided analysis of the whole coding, promoter and exon-flanking factor IX gene sequences, proved to be helpful for carrier detection and prenatal diagnosis in most hemophilia B families, including sporadic cases. " 10/01/2008 - "Investigation of factor IX (FIX) has benefited from excellent gene-deleted mouse models that recapitulate many of the features of human haemophilia B. " 03/01/1992 - "The American Red Cross has developed an immunoaffinity chromatography method to purify human coagulation Factor IX to high levels of purity for therapeutic treatment of hemophilia B. " 07/01/1997 - "These studies demonstrate that recombinant factor IX is effective in the treatment of hemophilia B and has the safety profile expected from a product prepared by recombinant technology." 03/15/2005 - "Factor IX variants improve gene therapy efficacy for hemophilia B." Order ALL the reference details at left...
2.	prothrombin complex concentrates (PPSB)IBA 03/01/1999 - "Thus, continuous infusion with this highly purified factor IX concentrate with improved viral safety is effective for surgery in haemophilia B." 05/01/2008 - "OCTANINE F is a high-purity blood clotting factor IX concentrate that has been shown to be effective and safe in adults with haemophilia B. " 01/01/1996 - "The safety and efficacy of a monoclonal antibody purified factor IX concentrate were evaluated in two continuing trials of 32 previously untreated patients with mild, moderate, or severe hemophilia B. " 01/01/1996 - "Safety and efficacy of monoclonal antibody purified factor IX concentrate in previously untreated patients with hemophilia B." 06/01/1990 - "The present study reports two cases of thrombotic episodes following surgery in PPSB-treated hemophilia B patients. " Order ALL the reference details at left...
3.	AntigensIBA 12/15/2003 - "The 7 of 12 tolerized hemophilia B dogs exhibited shortened whole blood clotting times (WBCTs), sustained detectable FIX antigen, undetectable Bethesda inhibitors, transient or no detectable antihuman FIX antibody titers by enzyme-linked immunosorbent assay (ELISA), and normal clearance of infused rhFIX. " 05/15/2003 - "Three newborn hemophilia B dogs that were injected intravenously with RV achieved 12% to 36% of normal cFIX antigen levels, which improved coagulation tests. " 01/01/2006 - "This mutation causes mild hemophilia B with approximately 25% FIX coagulant activity and FIX antigen levels of around 90% of normal. " 09/01/2004 - "CONCLUSION: The rAAV2/hFIX transfected BHK-21 and C2C12 cells could efficiently express hFIX antigen and was of therapeutic effects for the hemophilia B model mice by intramuscularly injection.The" 10/01/1999 - "Endogenous canine FIX antigen exists in Chapel Hill strain hemophilia B canine." Order ALL the reference details at left...
4.	Factor VIII (Coagulation Factor VIII)IBA 10/01/1998 - "The use of dilution studies, chromogenic assays, a novel in-house enzyme-linked-immunosorbent-assay-based technique and phospholipid neutralization, demonstrated that Case 1 had a genuine factor VIII inactivator resulting in factor VIII levels of less than 1 IU/dl but no factor IX deficiency. " 12/29/1995 - "Transfected stromal cells may serve as suitable vehicles for gene delivery to correct single gene disorders in which the product of the target gene does not require stringent regulation as, for example, in the correction of Factor VIII and Factor IX deficiency. " 05/01/1995 - "Natural canine models of factor VIII and factor IX deficiency have been available for many years, and gene therapy attempts on these dogs have met with partial success. " 09/01/1993 - "Of 36 patients studied, 26 were Factor VIII deficient (13 mild, 3 moderate, and 10 severe) and 10 had Factor IX deficiency (7 mild and 3 severe). " 09/01/1989 - "Seventeen had a moderate to severe depletion of factor VIII and 2 had a severe factor IX deficiency. " Order ALL the reference details at left...
5.	Complementary DNA (cDNA)IBA 08/01/1997 - "The regulation of hFIX cDNA expression in myoblasts was discussed and it was strongly suggested that a myoblast-mediated gene delivery system had the potential to be optimized as a safe and effective therapeutic modality for hemophilia B." 03/01/2003 - "It was found that while mutations responsible to account for all 2348 haemophilia B patients covered 20% of the FIX cDNA, only 1% of the cDNA involving mostly CpG dinucleotides accounted for mutation in 42.41% of the patient pool. " 12/01/1993 - "Injection of the vector Av1H9B, which encodes human factor IX cDNA, into the tail veins of mice resulted in efficient liver transduction and plasma levels of human factor IX that would be therapeutic for haemophilia B patients. " 06/01/2008 - "The two initial phase I/II AAV clinical trials for hemophilia B, delivering a factor IX cDNA to skeletal muscle or liver, showed no serious adverse events. " 07/01/1995 - "To study the expression of human clotting factor IX cDNA in transgenic mice, which is an essential work on gene therapy for hemophilia B, 3 recombinant constructions containing different lengths of human clotting factor IX cDNA have been introduced into the cultured cells. " Order ALL the reference details at left...
6.	MononineIBA 01/01/1995 - "These studies indicate that Mononine is safe and effective in the treatment of hemorrhagic episodes in patients with hemophilia B." 02/01/1992 - "Mononine was evaluated for in vivo recovery, half-life, and for its safety and efficacy in 10 patients with hemophilia B. " 01/01/1995 - "Pharmacokinetic variables were studied in severe hemophilia B patients: Nanotiv was compared with Preconativ; Immunine was compared with Prothromplex TIM4 in crossover studies; and Mononine was tested in a single-drug study. " 11/15/1993 - "In vivo, Nanotiv was compared with Preconativ and Immunine with Prothromplex TIM4 in crossover studies in patients with severe hemophilia B, and Mononine was tested in a single drug study. " 05/01/1995 - "Monoclonal antibody purified factor IX concentrate, Mononine (Armour Pharmaceutical Company, Kankakee, Illinois, USA), is a recently developed replacement factor concentrate for the treatment of patients with hemophilia B. " Order ALL the reference details at left...
7.	recombinant FVIIa (rFVIIa)IBA 09/01/2001 - "Rationale for the use of high dose rFVIIa in a high-titre inhibitor patient with haemophilia B during major orthopaedic procedures." 04/01/2000 - "A third group comprised haemophilia B patients receiving rFVIIa prophylaxis and on-demand aPCC or rFVIIa. " 04/01/1993 - "Such treatment can be administered regardless of the inhibitor titer in these patients, and rFVIIa is active hemostatically in hemophilia B patients also. " 01/01/2005 - "Also, recombinant factor VIIa is a treatment of choice in patients with haemophilia B with high-responding inhibitors and those with factor VII deficiency. " 01/01/2004 - "Investigation of the WB clotting profile was performed before and after ex vivo addition of rFVIIa 20 nm to WB from 26 patients with hemophilia A, two patients with severe hemophilia B, and individuals with deficiencies of FV, FX, FXI, and FXIII. " Order ALL the reference details at left...
8.	6-Aminocaproic Acid (6 Aminocaproic Acid)FDA LinkGeneric 05/01/1971 - "Epsilon-Aminocaproic acid therapy for dental extractions in haemophilia and Christmas disease: a double blind controlled trial." 01/02/1968 - "Epsilon-aminocaproic acid in the treatment of haemophilia and Christmas disease with special reference to the extraction of teeth." 09/01/1976 - "The same protocol using a single infusion of the appropriate factor replacement with supplementary epsilon-aminocaproic acid can be used for the management of patients with other coagulation disorders such as factor IX deficiency (Christman disease), factor XI deficiency, and von Willebrand's disease. " 02/01/1996 - "This study evaluated the safety and efficacy of combined treatment with epsilon-aminocaproic acid or tranexamic acid and monoclonal antibody purified factor IX (MAb factor IX) for prophylaxis against bleeding in eight hemophilia B patients undergoing nine dental extraction procedures. " 01/01/1995 - "Concomitant use of the monoclonal antibody-purified factor IX concentrate (Mononine, Armour Pharmaceutical Company, Collegeville, Pa.) and two antifibrinolytic agents, epsilon-aminocaproic acid (EACA; Amicar, Immunex, Seattle, Wash.) or tranexamic acid (AMCA; Cyklokapron, Kabi Pharmacia, Piscataway, N.J.) was examined for safety and efficacy in patients with hemophilia B. " Order ALL the reference details at left...
9.	Tranexamic Acid (AMCA)FDA Link 05/06/1972 - "Use of tranexamic acid in control of haemorrhage after extraction of teeth in haemophilia and Christmas disease." 05/06/1972 - "Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease." 04/30/1976 - "Re: tranexamic acid and bleeding: a double-blind cross-over study on three brothers with Christmas disease (factor IX deficiency)." 02/01/1996 - "This study evaluated the safety and efficacy of combined treatment with epsilon-aminocaproic acid or tranexamic acid and monoclonal antibody purified factor IX (MAb factor IX) for prophylaxis against bleeding in eight hemophilia B patients undergoing nine dental extraction procedures. " 01/01/1995 - "Concomitant use of the monoclonal antibody-purified factor IX concentrate (Mononine, Armour Pharmaceutical Company, Collegeville, Pa.) and two antifibrinolytic agents, epsilon-aminocaproic acid (EACA; Amicar, Immunex, Seattle, Wash.) or tranexamic acid (AMCA; Cyklokapron, Kabi Pharmacia, Piscataway, N.J.) was examined for safety and efficacy in patients with hemophilia B. " Order ALL the reference details at left...
10.	Factor VIIa (Activated Factor VII)IBA 12/01/2005 - "High dose factor VIIa improves clot structure and stability in a model of haemophilia B." 02/01/1994 - "Factor VIIa levels are decreased in patients with hemophilia B." 01/01/1996 - "To evaluate the mechanism responsible for the generation of factor VIIa in vivo, we measured the levels of this enzyme after administering purified factor IX concentrates to patients with hemophilia B. " 03/01/2000 - "The efficacy of recombinant activated factor VII (rFVIIa, NovoSeven) in five Haemophiliacs (four Haemophilia A, one Haemophilia B), with high inhibitors ranging from 70 to 1900 Bethesda units, was evaluated. " 01/01/2008 - "Using an engineered factor VII that can be secreted as factor VIIa, viral-mediated delivery of this transgene in the mouse liver resulted in phenotypic correction of murine hemophilia B, at expression levels of approximately 1 microg/ml. This model of factor VIIa continuous infusion can be further used to address the potential risk of thrombosis, in a transgenic model approach. " Order ALL the reference details at left...

Therapies and Procedures

1.	Intramuscular Injections 03/15/2005 - "Importantly, intramuscular injection of AAV-F.IX variants did not trigger antibody formation to F.IX in mice tolerant to F.IX-WT. These studies demonstrate that F.IX variants provide a promising strategy to improve the efficacy for a variety of gene-based therapies for hemophilia B." 04/15/2003 - "Previously, we established an experimental basis for gene transfer as a method of treating the disease in mice and hemophilic dogs through intramuscular injection of a recombinant adeno-associated viral (rAAV) vector expressing F.IX. In this study we investigated the safety of this approach in patients with hemophilia B. " 04/15/2000 - "Recent studies have shown that intramuscular injection of an adeno-associated viral (AAV) vector into mice and hemophilia B dogs results in vector dose-dependent, long-term expression of biologically active F.IX at therapeutic levels. " 03/01/2000 - "On the basis of these data and additional pre-clinical studies demonstrating an absence of vector-related toxicity, we initiated a clinical study of intramuscular injection of an AAV vector expressing human F.IX in adults with severe haemophilia B. " 05/01/2009 - "Direct intramuscular injection (IM) of adeno-associated virus (AAV) has been proven a safe and potentially efficient procedure for gene therapy of many genetic diseases including hemophilia B. " Order ALL the reference details at left...
2.	Transplantation (Transplant Recipients) 07/15/2008 - "The aim of this study was to investigate the efficacy of hepatocyte transplantation in the mouse model of hemophilia B. " 12/01/2006 - "5. The results of the present study suggest that transplantation of HSC results in the persistant expression of hFIX in mice, which may be useful in haemophilia B gene therapy." 07/15/2008 - "These results demonstrate that hepatocyte transplantation can provide therapeutic benefits in the treatment of hemophilia B." 07/15/2008 - "Therapeutic effects of hepatocyte transplantation on hemophilia B." 04/01/2007 - "Here, we report the observation of six T-lymphoblastic leukemia cases arising during the course of a gene therapy study for hemophilia B after transplantation of ex vivo transduced hematopoietic stem cells (HSCs) by a lentivirus vector. " Order ALL the reference details at left...
3.	Blood Transfusion (Blood Transfusions) 01/01/1989 - "Two patients required blood transfusions; one of these patients had a history of factor IX deficiency. " 03/01/1992 - "Immunological studies were performed on a group of 44 haemophilia A and 15 haemophilia B patients who were treated exclusively with blood products manufactured by the Scottish National Blood Transfusion Service (SNBTS). " Order ALL the reference details at left...
4.	Aftercare (After-Treatment) 08/01/1995 - "Previous studies have shown that the delivery of a recombinant adenoviral vector expressing canine FIX (cFIX) resulted in a complete correction of hemophilia B in FIX-deficient dogs, but that cFIX expression decreased to only about 1-2% of normal levels 3 weeks after treatment. " 05/01/1985 - "In a patient with severe haemophilia B and antibodies against factor IX in high titre, and known for many years to be a really high responder, it was possible to suppress the secondary antibody response after treatment with high doses of intravenous IgG (Gammonativ, KabiVitrum AB) combined with factor IX and cyclophosphamide. " Order ALL the reference details at left...
5.	Cardiac Surgical Procedures 03/01/2000 - "The present study summarizes the results of 12 cardiac surgical procedures performed in a carrier of Haemophilia B and in six patients with Haemophilia A at a single centre from 1979 to 1998. " Order ALL the reference details at left...

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