What is the role of clotting factors in the treatment of pediatric factor XIII (FXIII) deficiency?

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

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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