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Factor XII Deficiency

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An absence or reduced level of blood coagulation factor XII. It normally occurs in the absence of patient or family history of hemorrhagic disorders and is marked by prolonged clotting time.

Also Known As:

Coagulation Factor 12 Deficiency; Deficiency, Factor XII; Factor 12 Deficiency; Hageman Factor Deficiency; Deficiencies, Factor 12; Deficiencies, Factor XII; Deficiency, Factor 12; Deficiency, Hageman Factor; Factor 12 Deficiencies; Factor Deficiency, Hageman; Factor XII Deficiencies; Trait, Hageman; Hageman Trait

Networked: 126 relevant articles (1 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Factor XII Deficiency
2.	Factor XI Deficiency (Hemophilia C)
3.	Arthus Reaction
4.	Habitual Abortion (Recurrent Miscarriage)
5.	Hemorrhage

Experts

1.	Girolami, B: 2 articles (07/2005 - 04/2000)
2.	Girolami, A: 2 articles (07/2005 - 04/2000)
3.	Jin, Peipei: 1 article (01/2016)
4.	Gu, Song: 1 article (01/2016)
5.	Jiang, Wenli: 1 article (01/2016)
6.	Liu, Lanbo: 1 article (01/2016)
7.	Shen, Lisong: 1 article (01/2016)
8.	Wang, Xuefeng: 1 article (01/2016)
9.	Mo, Xi: 1 article (01/2016)
10.	Yan, Hui: 1 article (01/2016)

Drugs and Biologics

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

1.	Lupus Coagulation Inhibitor (Lupus Anticoagulant)IBA 12/01/1997 - "One patient had lupus anticoagulant, one had Hageman Factor deficiency, and one was cleared for surgery with no diagnosis. " 04/01/1996 - "The discrimination of factor XII deficiency and lupus anticoagulant." 03/01/1994 - "Congenital factor XII deficiency and lupus anticoagulant." 08/01/1993 - "Transient lupus anticoagulant associated with hypoprothrombinemia and factor XII deficiency following adenovirus infection." 01/01/1987 - "Major abdominal surgery on a patient with factor XII deficiency and lupus anticoagulant: no postoperative DVT as judged by the fibrinogen uptake test."
2.	Factor XII (Hageman Factor)IBA 10/19/1968 - "[Familial study in congenital Hageman factor deficiency (blood coagulation factor XII)]." 01/01/2016 - "Several mutations in factor XII have been reported in patients with factor XII deficiency. " 03/01/2015 - "Molecular characterization of cat factor XII gene and identification of a mutation causing factor XII deficiency in a domestic shorthair cat colony." 09/01/2013 - "Molecular analysis of factor XII gene in Thai patients with factor XII deficiency." 01/01/2004 - "Characterization of factor XII Tenri, a rare CRM-negative factor XII deficiency."
3.	AntigensIBA 07/01/2005 - "Comparable levels of activity and antigen in factor XII deficiency: a study of 21 homozygotes and 58 heterozygotes." 09/01/1976 - "Confirming earlier studies, HF antigen was absent in Hageman trait plasma, but other congenital deficient plasmas, including those of individuals with Fletcher trait and Fitzgerald trait, contained normal amounts of HF antigen. " 01/01/1987 - "In 7 patients factor XII antigen and activity were both decreased, suggesting an apparent factor XII deficiency. " 08/01/1979 - "The plasmas of three heterozygotes in these families contained approximately twice as much Hageman factor antigen as Hageman factor activity, whereas those of 16 heterozygotes in ordinary (CRM-) Hageman trait families contained approximately equal amounts of activity and antigen. " 10/29/1993 - "The cause of recurrent pelvic and leg venous thromboses in a 24-year-old man was found to be a combination of two rare anomalies, hypoplasia of the hepatic, prerenal segment of the inferior vena cava and factor XII deficiency (factor XII activity 38%, its antigen 39% of normal), the latter considered a risk factor for thromboembolism. "
4.	PrekallikreinIBA 01/01/1991 - "Factor XII deficiency and impaired prekallikrein activity were diagnosed in a 1-year-old Chinese Shar Pei. " 04/01/1971 - "Conversion of peak 2 to peaks 3, 4, and 5 was associated with a progressive decrease in size, increase in net negative charge, increased prekallikrein-activating activity, and decreased ability to correct Hageman factor deficiency. " 12/15/1990 - "Among the other etiologies not associated with a bleeding tendency, there were 31 proved or suspected cases with inhibitors of the PTT, most of whom were children, 2 factor XII deficiencies, 2 prekallikrein deficiencies and 4 contact phase activations. " 03/01/2009 - "Here we describe heparin monitoring during CPB using the chromogenic anti Xa assay in two patients with severe factor XII deficiency (FXII < 0.01 U/mL) and one patient with severe prekallikrein (PK) deficiency (PK < 0.01 U/mL). " 01/01/1979 - "Homozygous states of factor XII deficiency, Fletcher factor deficiency, and high-molecular-weight kininogen deficiency (Fitzgerald trait) also showed abnormally long APTTs by all systems. "
5.	Factor XI (Plasma Thromboplastin Antecedent)IBA 08/23/1991 - "Factor XII deficiency is not associated with bleeding, which suggests that another mechanism for factor XI activation exists in vivo. " 10/01/2002 - "The main possible diagnoses for a women with unforeseen prolonged APTT are the presence of an unspecified circulating anticoagulant, factor XI or factor XII deficiency, or factor VIII deficiency associated with von Willebrand disease. " 11/01/2010 - "The way by which contact of blood with foreign surface accelerates clotting has been elucidated from the discovery of four rare disorders of blood coagulation; Hageman trait, plasma thromboplastin antecedent (PTA) deficiency, Fletcher trait, and Fitzgerald trait. " 10/01/1971 - "II. Contact activation of plasma prekallikrein and factor XI in factor XII-deficiency plasma." 12/22/2011 - "Factor XI deficiency is associated with a bleeding diathesis, but factor XII deficiency is not, indicating that, in normal hemostasis, factor XI must be activated in vivo by a protease other than factor XIIa. "
6.	AntibodiesIBA 06/01/1997 - "In the prospective part of the study, we found 9.4% of the patients (95% C.I.: 6.8-12%) with an isolated factor XII deficiency, 7.4% of the patients (5.0-9.8%) with primary antiphopholipid antibodies, 47% of the patients (42.6-51.4%) with an insufficient response to the venous occlusion test and an isolated hypofibrinolysis was found in 42.6% (38.2-47%) of the patients (reference groups: respectively 0/150, 3/150, 2/150, p < 10(-3)). " 04/01/1996 - "Pseudo factor XII deficiency and phospholipid antibodies." 05/01/2001 - "These antibodies may be associated with slightly reduced plasma concentrations of factor XII and consequently a differential diagnosis with a mild factor XII deficiency should be considered." 05/01/2006 - "The molecular mechanisms responsible for the pathogenetic potential of these antibodies include: resistance to activated protein C, acquired Factor XII deficiency resulting in suppression of intrinsic fibrinolytic activity, activation of endothelial cells through the nuclear factor kappaB pathway leading to tissue factor upregulation, adhesion molecule and cytokine expression and activation of platelets. " 10/01/2002 - "Anti-phosphatidylethanolamine antibodies, anti-annexin antibodies, and factor XII deficiency were more common factors in recurrent spontaneous abortion patients than were anti-cardiolipin antibodies in terms of induction of recurrent pregnancy losses. "
7.	Flaujeac factor deficiencyIBA 01/01/1979 - "Homozygous states of factor XII deficiency, Fletcher factor deficiency, and high-molecular-weight kininogen deficiency (Fitzgerald trait) also showed abnormally long APTTs by all systems. " 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. " 03/01/1979 - "Collagen activation of platelet-associated Factor XI has been proposed as a mechanism for initiating intrinsic clotting independent of Factor XII. Since this could explain the lack of bleeding in patients with hereditary Factor XII deficiency, prekallikrein deficiency and high molecular weight kininogen deficiency, we subjected the hypothesis to rigorous testing. "
8.	Proteins (Proteins, Gene)IBA 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. "
9.	Factor VIII (Coagulation Factor VIII)IBA 03/01/1982 - "Frequently, the first indication of an additional factor XII deficiency is the disproportionate prolongation of the activated partial thromboplastin time (PTT) as regards the factor VIII level. " 11/01/2004 - "Orbital subperiosteal hemorrhage in a patient with factor VIII and factor XII Deficiency." 11/01/2004 - "This article describes the case of a patient with factor VIII and factor XII deficiency who had a subgaleal hemorrhage with orbital extension and optic nerve compression following head trauma." 09/01/1990 - "Two children with bleeding disorders (factor VIII [antihemophilic factor] and factor XII [Hageman factor] deficiency) did not require packed red blood cells transfusion. " 01/01/1986 - "A retrospective analysis was made of 265 patients who had such an isolated prolongation of the activated partial thromboplastin time on two successive blood samples: the causal abnormality remained unexplained in 135 patients; a well defined coagulation disorder without abnormal bleeding tendency was present in 110 patients (1 severe factor XII deficiency, 58 partial factor XI or XII deficiencies and 51 lupus anticoagulants); a bleeding disorder was diagnosed in 20 patients (8 haemophilias, 8 Von Willebrand's diseases, 4 factor VIII inhibitors). "
10.	Kallikreins (Kallikrein)IBA 01/01/1982 - "In a fourth member of the family factor XII deficiency was found with normal kallikrein activity. " 12/21/1979 - "The conclusion was reached that the additional determination of the amount of Kallikrein released may be of value in the detection of carriers of the Hageman trait." 01/01/1985 - "We studied the behavior of renin, prorenin, and kallikrein in a patient affected by congenital factor XII deficiency. " 12/21/1979 - "In four healthy subjects with Factor XII levels equal to or below 50% (selected at random from 100 persons) the release of Kallikrein was studied in order to establish the relationship between the above data and those found in four Hageman trait carriers showing levels of Factor XII above the lowest normal limits. " 12/21/1979 - "Relationship between kallikrein release and factor XII in normal persons and carriers of the Hageman trait."

Therapies and Procedures

1.	Cardiopulmonary Bypass (Heart-Lung Bypass) 09/01/2014 - "Factor XII Deficiency and Cardiopulmonary Bypass." 09/01/2014 - "Cardiopulmonary Bypass in a Patient with Factor XII Deficiency." 10/01/2012 - "This paper presents two patients with factor XII deficiency, a rare coagulation disorder, who successfully underwent surgery with cardiopulmonary bypass (CPB) in Cardiac Surgery Clinic of the 4th Military Clinical Hospital in Wroclaw. " 02/01/2010 - "Factor XII deficiency is associated with a prolonged activated partial thromboplastin time and activated clotting time used for monitoring during cardiopulmonary bypass. " 10/01/1997 - "Factor XII deficiency and cardiopulmonary bypass: use of a novel modification of the activated clotting time to monitor anticoagulation."
2.	Tonsillectomy 09/01/1987 - "[Tonsillectomy in factor XII deficiency]." 01/01/1968 - "[Hemorrhage after tonsillectomy in a patient with PTA and Hageman factor deficiency: clinico-therapeutic considerations]."
3.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 02/01/2010 - "We present the strategy used for a case of coronary artery bypass grafting in a patient with factor XII deficiency, followed by a brief discussion of the important clinical considerations when patients with factor XII deficiency undergo cardiac surgery. " 09/01/2014 - "We present a case of a patient with factor XII deficiency that had coronary artery bypass grafting and cardiopulmonary bypass using heparin for anticoagulation. "
4.	Liver Transplantation 07/01/2006 - "Factor XII deficiency acquired by orthotopic liver transplantation: case report and review of the literature." 07/01/2006 - "We report a case of transmission of factor XII deficiency with liver transplantation in a patient with Budd-Chiari syndrome. "
5.	Heart Transplantation (Grafting, Heart) 06/01/2005 - "Mitral valve repair and cardiac transplantation in a patient with factor XII deficiency."