Factor XIII Deficiency

A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.

Also Known As:

Deficiency, Factor 13; Deficiency, Factor Thirteen; Deficiency, Factor XIII; Factor 13 Deficiency; Factor Thirteen Deficiency; Deficiencies, Factor 13; Deficiencies, Factor Thirteen; Deficiencies, Factor XIII; Factor 13 Deficiencies; Factor Thirteen Deficiencies; Factor XIII Deficiencies

Networked: 212 relevant articles (2 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Bacteremia
2.	Hemorrhage
3.	Disseminated Intravascular Coagulation
4.	Venous Thrombosis (Deep-Vein Thrombosis)
5.	Disease Progression

Experts

1.	Ichinose, Akitada: 9 articles (05/2022 - 12/2010)
2.	Souri, Masayoshi: 7 articles (05/2022 - 12/2010)
3.	Dorgalaleh, Akbar: 5 articles (11/2020 - 09/2015)
4.	Carcao, Manuel: 4 articles (02/2022 - 05/2012)
5.	Nugent, Diane: 3 articles (02/2022 - 05/2012)
6.	Schroeder, Verena: 3 articles (01/2018 - 01/2006)
7.	Peyvandi, F: 3 articles (12/2017 - 11/2013)
8.	Inbal, Aida: 3 articles (02/2017 - 03/2006)
9.	Oldenburg, Johannes: 3 articles (02/2017 - 06/2010)
10.	Naderi, Majid: 3 articles (12/2016 - 09/2015)

Drugs and Biologics

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

1.	Factor XIII (Coagulation Factor XIII)IBA 05/31/2012 - "Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency." 05/01/2000 - "We analysed the early implantation tissues of normal women and of a patient with congenital factor XIII deficiency in order to study the role of maternal subunit A of factor XIII (XIIIA) in the development of extravillous cytotrophoblast. " 01/01/1996 - "Acquired factor XIII deficiency, however, is much more common, and preliminary studies suggest a lack of factor XIII to be an important feature of various diseases. " 10/24/2023 - "Successful perinatal management of a woman with congenital factor XIII deficiency using recombinant factor XIII: A case report and literature review." 10/12/2023 - "Heterozygosity in factor XIII genes and the manifestation of mild inherited factor XIII deficiency."
2.	Factor XIIIaIBA 01/01/2008 - "We report a novel mutation in factor XIIIA gene that caused severe congenital factor XIII deficiency in a 6 year and 8 month old male. " 01/01/2006 - "Today, more than 60 mutations in the factor XIIIA- and B-subunit genes are known leading to congenital factor XIII deficiency. " 12/01/2005 - "Factor XIII deficiency: new nonsense and deletion mutations in the human factor XIIIA gene." 12/25/2003 - "The 2 mutations of the factor XIIIA cause the instability, degradation, and rapid disappearance of FXIIIA in cytoplasm, thus resulting in hereditary factor XIII deficiency." 12/25/2003 - "To investigate the mechanisms of two novel missense mutations of factor XIIIA subunit gene (Arg77-->Cys,Ser413-->Trp) in the pathogenesis of hereditary factor XIII deficiency. "
3.	Fibronectins (Fibronectin)IBA 01/01/1984 - "Finally, the wound healing problems of patients with severe factor XIII deficiencies may occur because of their inability to incorporate fibronectin into blood clots." 11/01/1986 - "Infusion of activated Factor XIII (20 units/rat) during a period of hyperfibronectinemia (908.0 +/- 55.1 micrograms/ml) resulted in a significant (p less than 0.05) decrease in plasma fibronectin (548.5 +/- 49.9 micrograms/ml) within 30 min. Thus Factor XIII deficiency in rats with bacteremia may contribute to the elevation in plasma fibronectin by altering kinetics associated with the clearance of fibronectin from the blood."
4.	AntigensIBA 01/01/1996 - "Half-life studies of factor XIII from plasma in comparison with factor XIII from placenta were done in rabbits by determination of the antigen and in patients with congenital factor XIII deficiency by determination of the activity and antigen. " 01/01/1978 - "Studies on factor XIII antigen in congenital factor XIII deficiency. " 12/01/2018 - "Currently available assays for laboratory diagnosis of factor XIII deficiency include clot solubility assays, quantitative factor XIII activity assays, factor XIII antigen assays, and genetic testing. " 01/01/2014 - "Factor XIII deficiency was identified by an automated assay that measured factor XIII antigen. " 09/01/2011 - "Factor XIII antigen levels were 25% of predicted, and the diagnosis of factor XIII deficiency was made. "
5.	FibrinIBA 01/01/1988 - "Presumptive diagnosis of Factor XIII deficiency was made with a clot solubility screening test, and confirmation was accomplished by demonstrating the absence of cross-linked fibrin chains by electrophoresis. " 05/01/1980 - "Fibrin cross-linking in congenital factor XIII deficiency." 10/01/2013 - "Fibrin abnormalities (hypo/dysfibrinogenemia) and factor XIII deficiency are associated with infertility and pregnancy loss. " 12/29/1984 - "This latter finding suggests that, in addition to factor XIII deficiency, other causes are responsible for the high rate of fibrin crosslinking impairment in acute leukemia." 12/15/1975 - "In patients with congenital factor XIII deficiency or acquired immune inhibitors of fibrin stabilization a severe bleeding tendency is evident. "
6.	Proteins (Proteins, Gene)FDA Link 04/01/1976 - "[Studies on abnormal protein in seven patients with congenital factor XIII deficiency (author's transl)]." 11/01/1995 - "Molecular basis of inherited factor XIII deficiency: identification of multiple mutations provides insights into protein function." 02/22/1989 - "The concentration of the XIII-s protein (approximately 18 mg/l) was unaffected by the coagulation process and normal in plasma and serum from the case of congenital factor XIII deficiency. " 05/01/1980 - "Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. " 01/01/2000 - "To understand the structure-function relationships of the factor XIII molecule and its clinical implications in factor XIII deficiency, we characterized its genetic defects and closely examined its gene products, including mRNA and protein levels. "
7.	Fibrinolysin FDA Link 01/01/2016 - "Long Term Follow up Study on a Large Group of Patients with Congenital Factor XIII Deficiency Treated Prophylactically with Fibrogammin P®." 06/01/2011 - "Successful long-term replacement therapy with FXIII concentrate (Fibrogammin(®) P) for severe congenital factor XIII deficiency: a prospective multicentre study." 01/01/2013 - " diagnosis of disease all patients with severe factor XIII deficiency(<1 U/dl) shouldreceive prophylactic substitution therapywith fresh frozen plasma (FFP) and cryoprecipitate as traditional choices or purified concentrateof blood coagulation factor XIII (Fibrogammin P) inorder to control severe and life-threatening clinical complications of factor XIII deficiency. " 01/01/1988 - "In light of the long half-life of this factor and the relatively low risk associated with new Factor XIII concentrates, such as Fibrogammin, prophylactic life-long replacement therapy should be considered for patients with severe Factor XIII deficiency." 06/01/1984 - "These results suggest that an increased affinity of noncrosslinked fibrin for plasmin(ogen) is contributory to the accelerated fibrinolysis observed in factor XIII deficiency, in addition to an absence of crosslinking of alpha 2-plasmin inhibitor to fibrin."
8.	Messenger RNA (mRNA)IBA 02/01/2015 - "[Study on the molecular mechanisms of a novel large deletion of FXIIIA mRNA in a new hereditary factor XIII deficiency]." 03/01/1998 - "Thus, both mutations impaired normal processing of mRNA for the A subunit, resulting in his severe factor XIII deficiency." 03/01/1998 - "Novel deletion and insertion mutations cause splicing defects, leading to severe reduction in mRNA levels of the A subunit in severe factor XIII deficiency." 02/01/2015 - "To investigate the mechanisms of DelCD11-279 of factor XIII subunit A mRNA in the pathogenesis of hereditary factor XIII deficiency. " 01/01/2000 - "To understand the structure-function relationships of the factor XIII molecule and its clinical implications in factor XIII deficiency, we characterized its genetic defects and closely examined its gene products, including mRNA and protein levels. "
9.	Platelet Membrane Glycoprotein IIbIBA 10/01/2009 - "In this study, we evaluated the effect of factor XIII inhibition and additional glycoprotein (GP) IIb/IIIa blockade on (platelet-inhibited) whole blood thrombelastography and whether a modified routine assay (using factor XIII antibody) can be used to detect factor XIII deficiency. "
10.	Peptide Hydrolases (Proteases)FDA Link 12/31/1976 - "We conclude that the acid clot solubility test is not a specific indicator of factor XIII deficiency, but this simple assay is recommended for further studies of acid plasma protease activity. "

Therapies and Procedures

1.	Therapeutics 01/01/2021 - "Training Program for Home Therapy of People With Factor XIII Deficiency." 09/01/2019 - "Benefits of thromboelastometry for monitoring replacement therapy in patients with severe inherited factor XIII deficiency: 3 illustrative cases." 05/01/2014 - "A new era of therapy for congenital factor XIII deficiency." 01/01/2014 - "Acquired factor XIII deficiency: still a clinical challenge in the era of novel therapy." 01/01/2005 - "Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency: reply to a rebuttal."
2.	Thrombolytic Therapy 05/01/1984 - "These methods can be used to study fibrin degradation not only in patients with congenital factor XIII deficiency, but also in patients with disseminated intravascular coagulation or deep vein thrombosis during the course of thrombolytic therapy. "
3.	Tonsillectomy 06/01/2019 - "Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. " 11/15/2016 - "Recurrent Post Tonsillectomy Secondary Hemorrhage in Patients with Factor XIII Deficiency: A Case Series and Review of Literature."
4.	Plasma Exchange 02/01/2021 - "Acquired factor XIII deficiency in patients under therapeutic plasma exchange: A poorly explored etiology." 08/18/2023 - "Here, we report the case of a young woman with anti-NPX-2 antibody-positive dermatomyositis who developed massive haemothorax with acquired factor XIII deficiency during treatment, including plasma exchange therapy. "
5.	Cesarean Section (Caesarean Section) 06/01/2010 - "We report the peri-operative management of a 32-year-old patient suffering from symptomatic hypofibrinogenaemia and factor XIII deficiency scheduled for caesarean section. " 06/01/2010 - "Spinal anaesthesia and caesarean section in a patient with hypofibrinogenaemia and factor XIII deficiency." 12/22/2020 - "Her first baby, who was born by emergency Lower Segment Caesarean Section (LSCS) for fetal distress, had intracranial haemorrhage in the early neonatal period and was investigated elsewhere and diagnosed to have factor XIII deficiency. "