منابع مشابه
Treatment with Recombinant Factor XIII (Tretten) in a Pregnant Woman with Factor XIII Deficiency
BACKGROUND Factor XIII deficiency is associated with recurrent miscarriages in women. CASE REPORT In this report, we present a patient with factor XIII deficiency and some comorbidities who had had previous miscarriages. She began treatment with factor XIII subunit A (XIII-A) replacement treatment Recombinant factor XIII (Tretten) at a dose of 2500 units monthly and was able, for the first time...
متن کاملRecombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency.
Congenital factor XIII (FXIII) deficiency is a rare, autosomal-recessive disorder, with most patients having an A-subunit (FXIII-A) deficiency. Patients experience life-threatening bleeds, impaired wound healing, and spontaneous abortions. In many countries, only plasma or cryoprecipitate treatments are available, but these carry a risk for allergic reactions and infection with blood-borne path...
متن کاملRecombinant factor XIII (rFXIII): a novel treatment of congenital factor XIII deficiency
2015 Factor XIII (FXIII) is composed of two catalytic A subunits and two carrier B subunits. Following activation by thrombin FXIII becomes plasma transglutaminase, which crosslinks the γ-glutamyl-ɛ-lysine residues of fibrin chains stabilizing fibrin clot. Congenital deficiency of factor XIII results in a severe life-long hemorrhagic disorder, abnormal wound healing in about 30% of patients and...
متن کاملFactor XIII deficiency: a review of literature
Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...
متن کاملFactor XIII deficiency.
Inherited factor XIII (FXIII) deficiency is a rare bleeding disorder that can present with umbilical bleeding during the neonatal period, delayed soft tissue bruising, mucosal bleeding and life-threatening intracranial haemorrhage. FXIII deficiency has also been associated with poor wound healing and recurrent miscarriages. FXIII plays an integral role in haemostasis by catalysing the cross-lin...
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ژورنال
عنوان ژورنال: Nature Biotechnology
سال: 2014
ISSN: 1087-0156,1546-1696
DOI: 10.1038/nbt0314-210a