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Factor XI Deficiency (Hemophilia C)

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A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia.

Also Known As:

Hemophilia C; Deficiency, Factor XI; Plasma Thromboplastin Antecedent Deficiency; Rosenthal's Syndrome; Deficiencies, Factor XI; Factor XI Deficiencies; Rosenthal Syndromes; Rosenthal's Syndromes; Rosenthals Syndrome; Syndrome, Rosenthal; Syndrome, Rosenthal's; Rosenthal Syndrome

Networked: 194 relevant articles (2 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hemophilia A (Haemophilia)
2.	Wounds and Injuries (Trauma)
3.	Factor XII Deficiency
4.	Hemorrhage
5.	von Willebrand Diseases (von Willebrand's Disease)

Experts

1.	Seligsohn, Uri: 7 articles (05/2010 - 04/2002)
2.	Zivelin, Ariella: 6 articles (08/2015 - 04/2002)
3.	Salomon, Ophira: 5 articles (08/2015 - 06/2003)
4.	Livnat, Tami: 3 articles (08/2015 - 06/2003)
5.	Franchini, Massimo: 3 articles (10/2011 - 10/2006)
6.	Gailani, D: 3 articles (09/2006 - 10/2004)
7.	Tamarin, Ilia: 2 articles (08/2015 - 01/2005)
8.	Dardik, Rima: 2 articles (08/2015 - 06/2003)
9.	Bridey, F: 2 articles (07/2015 - 09/2011)
10.	Bauduer, F: 2 articles (07/2015 - 01/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Factor XI Deficiency:

1.	Factor XI (Plasma Thromboplastin Antecedent)IBA 07/01/2015 - "Factor XI replacement for inherited factor XI deficiency in routine clinical practice: results of the HEMOLEVEN prospective 3-year postmarketing study." 06/15/2003 - "This study was undertaken to determine the prevalence of acquired inhibitors against factor XI in patients with severe factor XI deficiency, discern whether these inhibitors are related to specific mutations, and characterize their activity. " 10/01/1989 - "Cloning of abnormal factor XI genes and studies on the molecular genetics of factor XI deficiency show that the cause for factor XI deficiency is heterogeneous. " 08/01/1988 - "A study of 20 Jewish and four non-Jewish kindreds transmitting factor XI deficiency (164 individuals) confirmed inheritance to be autosomal with severe deficiency in homozygotes (mean factor XI level 3.8 u/dl, SD 2.91) and partial deficiency in heterozygotes (mean factor XI level 57 u/dl, SD 10.42; normal mean factor XI level 96 u/dl, SD 11.6). " 12/01/1985 - "Failure to detect variant (CRM+) plasma thromboplastin antecedent (factor XI) molecules in hereditary plasma thromboplastin antecedent deficiency: a study of 125 patients of several ethnic backgrounds."
2.	Immunoglobulins (Immunoglobulin)IBA 01/01/1990 - "Clinical and immunological examinations of adults and children with Melkersson-Rossolimo-Rosenthal syndrome have revealed immunity deficiency: a decrease of the number of T and B cells, a low immunoglobulin content and the presence of the ++neuro-allergic syndrome according to the increased level of cerebral antibodies. " 12/27/1982 - "The low factor XI levels were not due to a classical coagulation inhibitor, but to a plasma factor (probably an immunoglobulin) that selectively bound factor XI. The factor XI deficiency improved after treatment with corticosteroids. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 12/27/1982 - "The low factor XI levels were not due to a classical coagulation inhibitor, but to a plasma factor (probably an immunoglobulin) that selectively bound factor XI. The factor XI deficiency improved after treatment with corticosteroids. "
4.	Proteins (Proteins, Gene)IBA 06/01/1988 - "Results of the assay, performed in three groups of subjects, correlate well with results of the coagulant assay as performed in our laboratory and clearly differentiate between total factor XI deficiency and the deficiency of any of the other contact proteins. " 01/01/1979 - "Studies of plasmas from individuals with Hageman trait (factor XII deficiency), plasma thromboplastin antecedent (PTA, factor XI) deficiency, Fletcher trait (plasma prekallikrein deficiency) and Fitzgerald trait (high molecular weight-kininogen deficiency) have revealed the importance of these proteins in blood coagulation. "
5.	PrekallikreinIBA 01/01/1979 - "Studies of plasmas from individuals with Hageman trait (factor XII deficiency), plasma thromboplastin antecedent (PTA, factor XI) deficiency, Fletcher trait (plasma prekallikrein deficiency) and Fitzgerald trait (high molecular weight-kininogen deficiency) have revealed the importance of these proteins in blood coagulation. "
6.	Flaujeac factor deficiencyIBA 01/01/1979 - "Studies of plasmas from individuals with Hageman trait (factor XII deficiency), plasma thromboplastin antecedent (PTA, factor XI) deficiency, Fletcher trait (plasma prekallikrein deficiency) and Fitzgerald trait (high molecular weight-kininogen deficiency) have revealed the importance of these proteins in blood coagulation. "
7.	First StepIBA 05/01/2014 - "This study is the first step in building a national database for anesthetic cases and outcomes of parturients with Factor XI deficiency. "
8.	Factor VIII (Coagulation Factor VIII)IBA 07/01/1978 - "Three case reports of mild Factor VIII and Factor XI deficiency, undiagnosed prior to the author's medical treatment, are presented. " 01/01/2005 - "Low factor VIII levels are a risk factor for bleeding in patients with factor XI deficiency." 11/01/2003 - "Among them, 1069 (89.8%) patients had haemophilia, 68 had vWD, 20 had factor XI deficiency, 10 had acquired factor VIII inhibitor and 23 had other coagulation disorders. " 02/01/1994 - "Combined factor VIII/factor XI deficiency may cause intra-familial clinical variability in haemophilia A among Ashkenazi Jews." 12/01/2002 - "Particularly, the epidemiological, clinical and diagnostic characteristics of von Willebrand disease, factor VIII, factor IX and factor XI deficiencies are examined. "
9.	recombinant FVIIa (rFVIIa)IBA 11/01/2011 - "The role of recombinant factor VIIa for obstetric block in women with severe factor XI deficiency." 09/01/2011 - "Monitoring low dose recombinant factor VIIa therapy in patients with severe factor XI deficiency undergoing surgery." 09/01/2009 - "We describe four patients with severe factor XI deficiency and inhibitors to FXI, undergoing eight (four major) surgical procedures treated with continuous infusion of rFVIIa. " 09/01/2008 - "Use of recombinant factor VIIa for hip replacement surgery in a patient with severe factor XI deficiency and drug-induced platelet defect." 01/01/2008 - "Successful use of recombinant factor VIIa (NovoSeven) during cardiac surgery in a pediatric patient with congenital factor XI deficiency."
10.	Factor VII (Proconvertin)IBA 05/01/2008 - "Characterization of combined factor VII and factor XI deficiencies." 08/01/1992 - "In addition to being associated with heterozygous factor XI deficiency, the abnormal factor VII molecule in the plasma of affected individuals in this kindred appears to represent a newly described mutation. " 08/01/1992 - "A novel congenital haemostatic defect: combined factor VII and factor XI deficiency." 01/01/2013 - "Factor VII and factor XI deficiencies show a poor relationship between the factor level and bleeding risk. " 06/01/2009 - "A retrospective review was carried out on the methods of obstetric analgesia/anesthesia used in 80 pregnancies amongst 63 women with inherited bleeding disorders (19 factor XI deficiency, 16 carriers of haemophilia, 15 von Willebrand disease, seven platelet function disorders, four factor VII deficiency, one factor VII and XI deficiency and one factor X deficiency). "

Therapies and Procedures

1.	Liver Transplantation 12/01/2015 - "Correction of factor XI deficiency by liver transplantation." 01/01/2015 - "Acquired factor XI deficiency: a rare complication after liver transplantation." 05/01/2008 - "Liver transplantation for factor XI deficiency: cure before diagnosis." 06/02/2005 - "Correction of factor XI deficiency by liver transplantation." 12/01/1991 - "Factor XI deficiency acquired by liver transplantation."
2.	Transplants (Transplant) 02/01/2014 - "Factor XI deficiency: incidental diagnosis post coronary artery bypass graft." 04/01/1989 - "Management of factor XI deficiency in coronary artery bypass graft surgery by plasmapheresis and exchange transfusion." 12/01/2015 - "Orthotopic liver transplantation for other diseases typically results in a coincidental cure for hemophilia A and B; however, long-term outcomes of liver transplant in hemophilia C are not very well described. "
3.	Aftercare (After-Treatment) 12/27/1982 - "The low factor XI levels were not due to a classical coagulation inhibitor, but to a plasma factor (probably an immunoglobulin) that selectively bound factor XI. The factor XI deficiency improved after treatment with corticosteroids. "
4.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 02/01/2014 - "We present a case of an uncomplicated 12-month delayed diagnosis of Factor XI deficiency following coronary artery bypass grafting and sternal rewiring. " 01/01/1990 - "A 61-year-old man with severe factor XI deficiency presented for coronary artery bypass surgery. " 10/01/1984 - "We report the successful management of a 61-year-old man with hereditary factor XI deficiency who had coronary artery bypass. " 10/01/1984 - "Coronary artery bypass in hereditary factor XI deficiency." 02/01/2014 - "Factor XI deficiency: incidental diagnosis post coronary artery bypass graft."
5.	Cesarean Section (Caesarean Section) 04/18/2014 - "Yet the management of cesarean section with factor XI deficiency at home and abroad is still lack of standard guideline by now. " 03/01/2010 - "Anesthetic strategy for cesarean section in a patient with factor XI deficiency. " 04/18/2014 - "[Anesthesia and perioperative management for cesarean section in patient with factor XI deficiency: a case report]." 06/01/1993 - "Anesthetic management of a patient with factor XI deficiency and factor XI inhibitor undergoing a cesarean section."