Hyperfibrinolysis and acquired factor XIII deficiency in newly diagnosed pediatric malignancies.
نویسندگان
چکیده
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منابع مشابه
Current understanding in diagnosis and management of factor XIII deficiency
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...
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 کاملIndirect Molecular Diagnosis of Congenital Factor ΧІІІ Deficiency by Candidate Microsatellites and Single Nucleotide Polymorphisms
Background: Congenital factor XIII (FXIII) deficiency is one of the rarest bleeding disorders with a prevalence of one per 2 million in the general population. The disorder is accompanied by a high rate of life-threatening bleeding. Due to normal results of routine coagulation tests, diagnosis of the disorder is challenging, but molecular methods can be used for precise diagnosis. Direct mutati...
متن کاملیک مورد سکته مغزی هموراژیک در کودکان
Stroke, though rare in children is among the top 10 causes of death in childhood. Incidence of ischemic and hemorrhagic stroke is the same in children .We report a case of hemorrhagic stroke in a two year old girl who presented with a limp, inability to stand on the left leg and left hemiparesia. Her complaint began 10 days ago after a bout of left clonic seizure. She had been admitted to the h...
متن کاملشیوع، تشخیص آزمایشگاهی و درمان کمبود فاکتور سیزده در ایران
Factor XIII deficiency is a bleeding disorder that its prevalence in the general population is about 1 in 2 million people around the world. In Iran, the high rate of consanguineous marriages lead to high rate of disorder with 473 factor XIII deficient patients that is about 12 times higher than the global Prevalence of disorder. The study, is a comprehensive review of all aspects of factor XII...
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ورودعنوان ژورنال:
- Haematologica
دوره 98 8 شماره
صفحات -
تاریخ انتشار 2013