Congenital factor XIII deficiency

What is Congenital factor XIII deficiency?

Congenital factor XIII deficiency is an inherited bleeding disorder due to reduced levels and activity of factor XIII (FXIII) and characterized by hemorrhagic diathesis frequently associated with spontaneous abortions and defective wound healing. Factor XIII deficiency is one of the most rare coagulation factor deficiencies.

Other condition names

  • Fibrin-stabilizing factor deficiency

Inheritance type

Autosomal recessive, Not applicable


  • Europe: <1 in 1,000 000

Age of Onset

  • All ages
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