Factor XIII deficiency
Factor XIII deficiency | |
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Specialty | Hematology |
Deficiency of Factor XIII leads to defective cross-linking of
As Factor XIII is composed of two subunit protein, A and B, for which the genes are located on different chromosomes, administration of recombinant A subunit improves clot stability and is becoming a therapeutic option for patients with this condition.[2][3]
Signs and symptoms
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Diagnosis
Bleeding manifestation with normal PT, aPTT, TT, BT, and CT is suspected as factor XIII Deficiency. Confirmatory test is urea lysis test. If clot is easily lysed in 5(M) urea solution then unstable clot and factor-XIII deficiency is confirmed.[citation needed]
Treatment
Fresh frozen plasma and cryoprecipitate
Fresh frozen plasma and cryoprecipitate are the mainstay of therapy for Factor XIII deficiency, but carry risk related to transfusion.[citation needed]
Factor XIII concentrates
Two commercially produced factor XIII concentrates are currently available in Europe, one manufactured by Bio Products Laboratory (BPL) and only available in the United Kingdom. The other, Fibrogammin-P, is produced by Beringwerke of Germany. In the U.S. FXIII concentrate is only available under the Federal Drug Administration's Investigational New Drug (IND) Program, or through clinical trial.[4]
Recombinant factor XIII
Recombinant factor XIII (rFXIII) is the only drug alternative to receiving blood transfusions, the traditional treatment for factor XIII deficiency.
rFXIII is synthetically bio-engineered through a yeast expression system and administered intravenously. In clinical trials, the drug was administered once every four weeks or administered on-demand in order to treat bleeding episodes.
See also
References
- ^ Dorgalaleh A, Naderi M, Hosseini MS, Alizadeh S, Hosseini S, Tabibian S, et al. (2015). "Factor XIII Deficiency in Iran: A Comprehensive Review of the Literature. Seminars in thrombosis and hemostasis". 41 (3 (41)): 323–329.
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(help) - ^ PMID 16556896.
- ^ "Recombinant Factor XIII". 2010.
- ^ "Factor XIII". 2014-03-05.
- PMID 10379818.
- ^ Clinical Trials at Novo Nordisk (2010). "Evaluation of Recombinant Factor XIII for Prevention of Bleeding in Patients with FXIII Inherited Deficiency". Archived from the original on 2022-11-12. Retrieved 2010-11-29.