Pediatric Factor XIII Deficiency Medication

Updated: Mar 15, 2019
  • Author: Helge Dirk Hartung, MD; Chief Editor: Cameron K Tebbi, MD  more...
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Medication

Medication Summary

Scheduled factor XIII (FXIII) replacement every 4-6 weeks maintains factor XIII levels above the critical threshold for spontaneous bleeding and allows patients to participate in regular activities.

For patients with congenital factor XIII A-subunit deficiency, which includes the majority of patients, treatment with recombinant factor XIII, as well as treatment with factor XIII concentrate, can be considered.

For patients with a B-subunit deficiency, the recombinant product cannot be used.

For all patients, treatment with cryoprecipitate and fresh frozen plasma (FFP) should be considered in the event of acute bleeding if concentrate and recombinant factor are not available.

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Clotting factors

Class Summary

Hemostasis is the physiological response to bleeding. Injury to the blood vessel wall and factors released by platelets initiate the coagulation cascade. Formation of an insoluble fibrin clot, which reinforces the initial platelet plug, is mediated by blood clotting factors. Clotting factors function as cofactors in the blood-coagulation cascade.

Factor XIII A-subunit, recombinant (Tretten)

Recombinant human factor XIII-A2 homodimer composed of 2 FXIII A-subunits. FXIII is the terminal enzyme in the blood coagulation cascade; when activated by thrombin at the site of vessel wall injury, FXIII plays an important role in the maintenance of hemostasis through cross-linking of fibrin and other proteins in the fibrin clot.

Factor XIII concentrate (Corifact)

FXIII is a proenzyme that is activated, in the presence of calcium ion, by thrombin cleavage of the A-subunit to become activated FXIII (FXIIIa). Promotes cross-linking of fibrin during coagulation and is essential to the physiological protection of the clot against fibrinolysis.

Indicated for routine prophylactic treatment of congenital factor XIII (FXIII) deficiency.

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