Congenital deficiency of factor XIII with platelet dysfunction
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چکیده
منابع مشابه
Congenital 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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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 کامل[Congenital deficiency of fibrin stabilizing factor [factor XIII)].
The authors report a new case of congenital deficiency of fibrin stabilizing factor. The defect is transmitted as an autosomal recessive trait. The earliest symptom is umbilical bleeding when the cord separates. The diagnosis is confirmed by the rapid dispersion of the clot in urea 5 M or monochloroacetic acid.
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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...
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ژورنال
عنوان ژورنال: Blood & Vessel
سال: 1976
ISSN: 0386-9717,1884-2372
DOI: 10.2491/jjsth1970.7.36