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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Factor XIII deficiency


Other Names for this Disease

  • Congenital Factor XIII deficiency
  • Fibrin stabilizing factor deficiency
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Treatment

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How might factor XIII be treated?

The amount of Factor XIII necessary for a normal response to trauma is only about 10 percent of that in the normal plasma. People with Factor XIII deficiency are generally given small infusions of fresh or frozen blood plasma (cryoprecipitates), or Factor XIII concentrates every three or four weeks. This has proven to be a highly successful preventive treatment for the disorder. Patients typically have a normal response to trauma while on these transfusions. When patients with Factor XIII deficiency have a high incidence of bleeding inside the head (intracranial), preventive treatment is necessary.[1]

In February 2011, the US Food and Drug Administration approved Corifact, a product manufactured by CSL Behring of Marburg, Germany, to prevent bleeding in people with congenital Factor XIII deficiency. Corifact is made from the pooled plasma of healthy donors. It can be used for individuals with absent or decreased levels of FXIII.[2] 

People receiving Corifact may develop antibodies against Factor XIII that may make the product ineffective. It potentially can cause adverse events from abnormal clotting if doses higher than the labeled dose are given to patients. Cryoprecipitate should not be used to treat patients with factor XIII deficiency except in life- and limb-threatening emergencies when Factor XIII concentrate is not immediately available.[2]
Last updated: 8/16/2011

References
  1. Factor XIII Deficiency. National Organization for Rare Disorders (NORD). 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/66/viewAbstract. Accessed 8/16/2011.
  2. Factor XIII Deficiency. National Hemophilia Foundation. http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=71&contentid=58. Accessed 8/16/2011.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Factor XIII deficiency. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name Factor Xiii Concentrate (Human)
Trade Name
(Manufacturer Name)
Corifact
(CSL Behring L.L.C.)
Indication
The FDA has approved this product to be used in this manner.
For the routine prophylactic treatment of congenital factor XIII deficiency

Generic Name coagulation factor XIII A-subunit (recombinant)
Trade Name
(Manufacturer Name)
Tretten
(Novo Nordisk, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Routine prophylaxis of bleeding in patients with congenital Factor XIII A-subunit deficiency.
More Information about this product Drug Information Portal

Other Names for this Disease
  • Congenital Factor XIII deficiency
  • Fibrin stabilizing factor deficiency
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.