激素相关方案治疗West综合征的研究进展  

Advances in the treatment of West syndrome with hormonal therapies

作　　者：阳柳(综述) 廖建湘(审校) Yang Liu;Liao Jianxiang(Zhuhai Campus of Zunyi Medical University,Zhuhai 519090,China;Department of Neurology,Shenzhen Children′s Hospital,Shenzhen 518038,China)

机构地区：[1]遵义医科大学珠海校区,519090 [2]深圳市儿童医院神经内科,518038

出　　处：《国际儿科学杂志》2021年第3期186-190,共5页International Journal of Pediatrics

基　　金：深圳市医疗卫生三名工程(SZSM201812005)。

摘　　要：West综合征(West syndrome,WS)是较为常见的难治性癫痫综合征,通常婴儿期起病,预后较差。国外文献报道发病率为2/10000~5/10000。激素(促肾上腺皮质激素以及糖皮质激素)作为治疗West综合征的一线用药,可有效控制痉挛发作、改善认知及发育损害。但激素治疗相关不良反应发生率较高,常见的有高血压、易激惹和感染。该文将对激素相关方案治疗West综合征的作用机制、用药方案、短期疗效、远期疗效、预后以及不良反应等方面的研究进展进行综述。West syndrome is one of the most common refractory epileptic syndromes with onsets mostly in infancy.The prognosis is generally poor.The morbidity rate reported in the literature is 0.2‰to 0.5‰.Hormonal therapies(Adrenocorticotropic hormone and glucocorticosteroid)are recommended as preferred treatment options for West syndrome.It can effectively control spasms,ameliorate recognition impairment,and improve developmental outcomes.The incidence of side effects after hormonal therapy are high,and irritation,hypertension and infection are frequently reported.In this paper,advances in therapeutic mechanism,usage and dosage,short-term effectiveness,long-term effectiveness,prognosis and adverse effects of hormonal therapies are reviewed.

关 键 词：WEST综合征 激素治疗 促肾上腺皮质激素 糖皮质激素 

分 类 号：R74[医药卫生—神经病学与精神病学]