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血友病甲是一种X连锁隐性遗传性出血性疾病,通常男性发病,女性携带,患病率大约为(5~10)/10万人。出血为其主要临床表现,出血部位可见于皮肤黏膜、关节、肌肉、软组织、消化道、泌尿生殖系统及中枢神经系统等。关节出血是本病最常见的特征性出血,颅内出血发生率虽低,但却是本病最重要死亡原因,需引起足够重视。现有1例甲型血友病反复颅内出血4次的病例,报告如下。
Hemophilia A is an X-linked occult hereditary hemorrhagic disease, usually male onset, female carriers, the prevalence of about (5 ~ 10) / 100000 people. Bleeding is its main clinical manifestations, bleeding sites can be found in the skin and mucous membranes, joints, muscles, soft tissues, digestive tract, genitourinary system and the central nervous system. Joint bleeding is the most common characteristic bleeding of the disease, although the incidence of intracranial hemorrhage is low, but it is the most important cause of death of the disease, need to pay enough attention. The existing 1 case of hemophilia A recurrent intracranial hemorrhage four cases, the report is as follows.