Factor XIII deficiency

Common Name(s)

Factor XIII deficiency

Factor XIII deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding. Signs and symptoms occur as the result of a deficiency in the blood clotting factor 13, which is responsible for stabilizing the formation of a blood clot. In affected individuals, the blood fails to clot appropriately, resulting in poor wound healing. Blood may seep into surrounding soft tissues, resulting in local pain and swelling. Internal bleeding may occur; about 25 percent of affected individuals experience bleeding in the brain. FXIII deficiency is usually caused by mutations in the F13A1 gene, but mutations have also been found in the F13B gene. It is usually inherited in an autosomal recessive fashion. Acquired forms have also been reported in association with liver failure, inflammatory bowel disease, and myeloid leukemia.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Factor XIII deficiency" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

Last Updated: 29 Apr 2014

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The Haemophilia Society

To ensure that people affected by bleeding disorders have the freedom to make choices and seize opportunities To enable people affected by bleeding disorders to better understand and manage their condition or situation. To enable people affected by bleeding disorders to participate in decision making and service delivery. To influence policy and improve services to people with bleeding disorders.

Last Updated: 3 Apr 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Factor XIII deficiency" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

http://www.caringvoice.org

Last Updated: 29 Apr 2014

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The Haemophilia Society

To ensure that people affected by bleeding disorders have the freedom to make choices and seize opportunities To enable people affected by bleeding disorders to better understand and manage their condition or situation. To enable people affected by bleeding disorders to participate in decision making and service delivery. To influence policy and improve services to people with bleeding disorders.

http://www.haemophilia.org.uk/

Last Updated: 3 Apr 2013

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Factor XIII deficiency" returned 48 free, full-text research articles on human participants. First 3 results:

Diagnosis and management of severe congenital factor XIII deficiency in the Emergency Department: lessons from a "model" family.
 

Author(s): Marta Bertamino, Laura Banov, Angelo C Molinari

Journal: Blood Transfus. 2015 Apr;13(2):324-7.

 

Last Updated: 7 Apr 2015

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Last Updated: 2 Feb 2015

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Pharmacokinetics of recombinant factor XIII at steady state in patients with congenital factor XIII A-subunit deficiency.
 

Author(s): B Kerlin, B Brand, A Inbal, S Halimeh, D Nugent, M Lundblad, R Tehranchi

Journal: J. Thromb. Haemost.. 2014 Dec;12(12):2038-43.

 

The use of monthly recombinant factor XIII (rFXIII) recently demonstrated favorable safety and efficacy for congenital FXIII A-subunit deficiency patients aged ≥ 6 years (mentor(™) 1 trial), although the pharmacokinetics (PK) were not fully evaluated.

Last Updated: 16 Dec 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Factor XIII deficiency" returned 3 free, full-text review articles on human participants. First 3 results:

Factor XIII deficiency in Henoch-Schönlein purpura - report on two cases and literature review.
 

Author(s): Anna Hogendorf, Wojciech Młynarski

Journal: Dev Period Med. ;18(3):318-22.

 

Factor XIII (FXIII) deficiency is a rare, inherited or acquired coagulation disorder that potentially precipitates fatal haemorrhage. We report two consecutive pediatric patients with Henoch-Schönlein purpura (HSP) and symptomatic decrease in FXIII. The possible FXIII deficiency ...

Last Updated: 3 Sep 2014

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Congenital factor XIII deficiency in Switzerland: from the worldwide first case in 1960 to its molecular characterisation in 2005.
 

Author(s): V Schroeder, D Durrer, E Meili, G Schubiger, H P Kohler

Journal: Swiss Med Wkly. 2007 May;137(19-20):272-8.

 

Coagulation factor XIII (FXIII) has a major role in the final stage of blood coagulation, is important for wound healing and maintaining pregnancy. Severe congenital FXIII deficiency is a rare disorder with 1 patient in 1-3 million. Untreated, it causes bleeding events, with intracranial ...

Last Updated: 27 Jun 2007

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Acquired factor XIII deficiency due to an inhibitor: a case report and review of the literature.
 

Author(s): A V M Nijenhuis, L van Bergeijk, P C Huijgens, S Zweegman

Journal:

 

Last Updated: 11 May 2004

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Wound Healing Abnormalities in Major Abdominal Surgery
 

Status: Recruiting

Condition Summary: Impaired Wound Healing; Factor XIII Deficiency

 

Last Updated: 27 Dec 2015

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