Factor XIII deficiency

The factor XIII deficiency is a very rare disorder of hemostasis due to an autosomal recessive deficiency on fibrin - stabilizing factor (factor XIII). When the person concerned is less than one percent before the normal amount of the factor.

Symptoms

In homozygous newborns often occur ( in up to 80 percent of births ) on massive umbilical cord bleeding. In homozygous adults it comes to impaired wound healing, increased to cerebral hemorrhage, or bleed. Also miscarriages are heaped. In male sufferers oligospermia and a reduction of the testes has been described.

Heterozygous individuals are asymptomatic.

Therapy

For treatment, a factor XIII concentrate is administered prophylactically. Such concentrates are obtained by plasma fractionation of blood plasma. For several years there has been produced in vitro preparations.