状细胞病的管理:专家小组成员2014年基于证据的报告摘要
Barbara P Yawn1, George R Buchanan2, Araba N Afenyi-Annan3
1Olmsted Medical Center, Rochester, Minnesota.
JAMA
|September 10, 2014
概括
建议在状细胞疾病 (SCD) 管理中进行尿素和输血. 这些指导方针涵盖预防服务,急性和慢性并发症,以及对SCD患者铁过载的监测.
科学领域:
- 血液学 血液学 血液学
- 遗传学 是一个遗传学.
- 内部医学 内部医学
背景情况:
- 状细胞疾病 (SCD) 是一种危及生命的遗传疾病,在美国影响了近10万个人.
- SCD存在许多急性和慢性并发症,需要立即医疗照顾.
- 目前的疾病修饰疗法,如基尿素和输血,未得到充分利用.
研究的目的:
- 支持和扩大能够并愿意为SCD患者提供护理的医疗保健专业人员的数量.
- 为SCD的查,管理和监测制定基于证据的建议.
主要方法:
- 在多个数据库 (MEDLINE,EMBASE,Cochrane,CINAHL,TOXLINE,Scopus) 中进行了全面的文献搜索.
- 搜索包括随机临床试验,非随机干预研究和从1980年到2014年4月以英语出版的观察性研究.
- 专家小组和方法学家制定了关键问题,以指导文献综述.
主要成果:
- 强烈的预防建议包括预防性青素,每年进行跨的多普勒检查,以及预防中风的长期输血治疗.
- 急性并发症的管理包括在住院期间快速启动阿片类药物治疗疼痛和激励螺旋计.
- 慢性并发症的治疗包括止痛药和非血管性瘤的物理治疗,小albuminuria的ACE抑制剂,以及视网膜病变和肺高血压的专家转诊.
- 强烈推给患有频繁危机或干扰症状的成年人,适度推给婴儿和儿童.
- 对于特定的指示,建议进行手术前输血和铁化疗法,以及对铁过载的监测.
结论:
- 对于许多患有SCD的人来说,强烈建议使用基尿素和输血治疗.
- 许多建议的证据质量受到高质量的临床试验不足的限制.
- 需要进一步的研究来加强SCD的查,管理和监测策略.
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