凝血因子Ⅷ缺乏症研究现状
The Research Status of Factor Ⅷ Deficiency
摘要
凝血因子ⅩⅢ（FⅩⅢ）缺乏症是一种十分罕见的出血性疾病,发病率仅为1/2 000 000,占出血性疾病的6%,可分为遗传性和获得性两种类型。遗传性FⅩⅢ缺乏症为一种常染色体隐性遗传病,主要是由于FⅧ-A和（或）FⅩⅢ-B基因的缺陷所致,发病率约为1/3 000 000,目前全世界大约有500例相关报道,而获得性FⅩⅢ缺乏症国际上有正式文献报道的病例有38例。遗传性FⅩⅢ缺乏症临床主要表现为反复、大范围、自发性严重出血，死亡病例接近50％，儿科医师应高度关注该病。
出处
《儿科药学杂志》
CAS
2015年第12期49-52,共4页
Journal of Pediatric Pharmacy
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