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  广州4月17日电(记者蔡敏婕)4月17日是第37个“世界血友病日”。南方医科大学南方医院血液科教授孙竞表示,近20年来血友病的诊疗已经取得很大的进步,“随着诊疗技术的进步,如今20岁以下的患者,未来活到70岁、80岁是没有问题的。

  血友病是一种由于血液中某些凝血因子的缺乏而导致患者产生严重凝血障碍的遗传性出血性疾病。作为我国首批公布的罕见病之一,血友病虽然发病率仅为2.73/10万,但其对患者生活质量的影响不容忽视。

  血友病患者非常脆弱,一旦受到外伤,由于缺乏凝血因子会过度出血,其中关节出血最为常见,约70%至80%的出血发生在关节部位。重型血友病患者占了将近一半,他们一年出血至少20次到50次,急性出血这种疼法是一辈子都伴随。反复慢性出血造成关节残疾,没有办法正常上学,没有办法工作,被称为“玻璃人”。

  孙竞表示血友病的诊断并不困难。反复出血倾向、莫名瘀斑等都是重要信号,通过凝血功能筛查和因子活性检测即可确诊。目前我国二级以上医院基本都具备诊断能力。

  孙竞表示,血友病的治疗就是补充凝血因子,是替代治疗。替代治疗有两个策略,一是按需治疗,也就是患者在出血之后,立即补充凝血因子以止血,但这个方法就像“亡羊补牢”,不能阻止下一次出血的发生。第二种是定期规律地补充凝血因子,提前维持体内凝血因子的最低有效水平,避免出血的发生,这种方式我们称为“预防治疗”。

  然而,预防治疗的推广仍面临诸多挑战。孙竞指出:“首先是经济负担问题,另外还面临着一旦出现抗体,也就是VIII因子或者IX因子的抑制物,凝血因子治疗就失效的难题”。

  值得期待的是,近年来血友病治疗领域取得重大突破。孙竞表示,最近5年有创新,是非因子的药,可以模拟凝血因子或者凝血再平衡,还可以避免抑制物的难题。近期国外获批的新一代非因子疗法可实现皮下注射,可以1个月甚至2个月给药一次,提高治疗便利性。

  在居家安全方面,孙竞主张游泳,提醒避免剧烈运动,谨慎用药。他强调:“患者尽量不要吃鱼,刺扎到喉咙会出血,尽量不要吃酸辣的,喝酒肯定不行。”(完) 新闻结尾 傅沛洲沈葭小说,助你快速实现自出版梦想!的相关文章
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