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Factor XIII deficiency
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Factor XIII deficiency : ウィキペディア英語版
Factor XIII deficiency

Factor XIII deficiency occurs exceedingly rarely, causing a severe bleeding tendency. The incidence is one in a million to one in five million people, with higher incidence in areas of consanguineous marriage. Most are due to mutations in the A subunit gene (located on chromosome 6p25-p24). This mutation is inherited in an autosomal recessive fashion.
Deficiency of Factor XIII leads to defective cross-linking of fibrin and vulnerability to late re-bleeds when the primary hemostatic plug is overwhelmed. Bleeding tendencies similar to hemophiliacs develop, such as hemarthroses and deep tissue bleeding.
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.〔(【引用サイトリンク】 year = 2010 )
== Therapy options ==


抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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