Factor XIII Deficiency

Definition - What does Factor XIII Deficiency mean?

Factor XIII deficiency is a rare bleeding disorder present since birth that occurs in 1 out of every 5 million births. It is inherited in an autosomal recessive fashion, which means that both parents must carry the abnormal gene in order to pass it on to their children. Factor XIII deficiency can affect both males and females. In females, it tends to cause various kinds of bleeding issues, recurrent miscarriages, and infertility. Though commonly a bleeding disorder, factor XIII deficiency can also otherwise contribute to the formation of blood clots in veins and arteries.

Factor XIII deficiency is also referred to as a fibrin stabilizing factor deficiency.

FertilitySmarts explains Factor XIII Deficiency

Factor XIII is a factor that helps in the formation of a strong clot and then prevent its breakdown. Its deficiency, thereby, interferes with the generation of a strong clot resulting in bleeding.

Factor XIII deficiency can give rise to the following problems depending on the degree of deficiency (i.e. if it's mild, moderate, or severe):

  • Easy bruising
  • Bleeding from the umbilical cord stump (occurring between 1-19 days after birth)
  • Bleeding after minor trauma or surgery
  • Nosebleeds
  • Bleeding from the gums
  • Prolonged, heavy menstrual bleeding in women
  • Bleeding into the joints or muscles
  • Life-threatening bleeding within the brain: This can be spontaneous (particularly in adults) or occur after a trauma
  • Vaginal spotting or bleeding during early pregnancy in females, heralding a spontaneous miscarriage
  • Inability to carry a pregnancy to term in the affected women
  • Infertility: Repeated miscarriages contribute to infertility
  • Bleeding after delivery in pregnant women who reach term or a premature separation of the placenta prior to birth

The doctor performs a clotting profile, including a factor XIII assay to confirm a suspected case of factor XIII deficiency. The condition can be diagnosed soon after birth due to the early appearance of symptoms, or otherwise prior to birth via DNA analysis of the fetus.

Replacement therapy with factor XIII deficiency is the mainstay of treatment in people with factor XIII deficiency. Women with factor XIII deficiency can achieve successful pregnancies with the replacement of factor XIII before and during their pregnancy.

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