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.

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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.

Logo
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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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:

Recurrent Post Tonsillectomy Secondary Hemorrhage in Patients with Factor XIII Deficiency: A Case Series and Review of Literature.
 

Author(s): Zaid Abu Rajab ALtamimi, Rashid Sheikh, Hassen Omar, Hayam Al Taweel, Shanmugam Ganesan

Journal:

 

BACKGROUND Post-tonsillectomy hemorrhage (PTH) has been reported in the literature as a serious complication after tonsillectomy that has high morbidity and can be life threatening. In cases of recurrent secondary PTH, one should consider coagulopathies as the hidden pathology. Factor ...

Last Updated: 15 Nov 2016

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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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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:

Laboratory Diagnosis of Factor XIII Deficiency, Routine Coagulation Tests with Quantitative and Qualitative Methods.
 

Author(s): Akbar Dorgalaleh, Ahmad Kazemi, Farhad Zaker, Morteza Shamsizadeh, Jamal Rashidpanah, Mojtaba Mollaei

Journal: Clin. Lab.. 2016 ;62(4):491-8.

 

Factor XIII (FXIII) deficiency is a severe bleeding disorder with normal routine coagulation tests that makes diagnosis of the disorder complicated. After normal results in routine coagulation tests, clot solubility test, and FXIII activity, antigen assays along with molecular methods ...

Last Updated: 24 May 2016

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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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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

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Last Updated: 11 May 2004

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Factor XIII in Major Burns Coagulation
 

Status: Not yet recruiting

Condition Summary: Burns; Coagulation Disorder; Factor XIII Deficiency; Cicatrization; Endothelial Dysfunction

 

Last Updated: 15 Jun 2017

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