Recombinant factor XIII and congenital factor XIII deficiency: an update from human and animal studies
نویسندگان
چکیده
منابع مشابه
Recombinant 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 کاملCongenital factor XIII deficiency in a neonate.
Pelvic examination showed that it was normal and adequate for vaginal delivery. Her haemoglobin at the time of booking was 12 g/dl, group 0, rhesus positive. Haemoglobin electrophoretic pattern was A+C. Serological test was negative. Urine analysis showed no abnormality. The pregnancy progressed normally and she went into labour spontaneously at term. On 18 February 1976, after being in labour ...
متن کاملCongenital factor XIII deficiency in a neonate
Pelvic examination showed that it was normal and adequate for vaginal delivery. Her haemoglobin at the time of booking was 12 g/dl, group 0, rhesus positive. Haemoglobin electrophoretic pattern was A+C. Serological test was negative. Urine analysis showed no abnormality. The pregnancy progressed normally and she went into labour spontaneously at term. On 18 February 1976, after being in labour ...
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ژورنال
عنوان ژورنال: Research Reports in Clinical Cardiology
سال: 2013
ISSN: 1179-8475
DOI: 10.2147/rrcc.s53225