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The body makes 13 blood-clotting factors and if any are deficient, blood doesn’t clot properly and a bleeding disorder results. The Factor XIII protein stabilizes blood clot formation—without the protein, clots form, but then break down.
Factor XIII (fibrin stabilizing factor) deficiency is the rarest bleeding disorder, impacting one in every 5 million births.
Factor XIII is typically caused by mutations on the F13A1 gene, but can also result from F13B gene mutations.
Both parents must pass on the defective gene for children to develop Factor XIII.
People with Factor XIII experience internal bleeding, bruising, nose and mouth bleeds, muscle bleeds and delayed bleeding after surgery. Almost 80 percent of babies with Factor XIII bleed from the umbilical cord at birth (higher than any other bleeding disorder).
After analyzing family history and symptoms, and eliminating other bleeding disorders, Factor XIII is diagnosed by quantifying Factor XIII activity with assays (blood content tests) and a clot solubility test.
Small infusions of blood plasma and/or Factor XIII concentrate every three or four weeks as a preventative treatment helps promote proper blood clotting.
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