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韦斯特综合征的促肾上腺皮质激素治疗:芬兰的观点。

ACTH therapy of West syndrome: Finnish views.

作者信息

Riikonen R

机构信息

Department of Child Neurology, Kuopio University, Hospital, P.O.B. 1777, Kuopio 70211, Finland.

出版信息

Brain Dev. 2001 Nov;23(7):642-6. doi: 10.1016/s0387-7604(01)00306-0.

Abstract

To provide up-to-date information on adrenocorticotropic hormone (ACTH) therapy in the treatment of West syndrome, a review of the Finnish studies was made in answer to the questions: what are (1) its efficacy: doses and comparison with vigabatrin (VGB), (2) its tolerability, (3) its mechanism of action? Why do some patients respond, but others do not? No other drugs have been shown to be more effective than ACTH. High doses were not more effective than low doses. Synthetic derivatives were associated with more frequent side effects. Individualized therapy was developed on the basis of etiology and response. With therapy consisting of ACTH 3-6IU/kg/day, all the cryptogenic and half of the symptomatic spasms could be controlled within over 2-3 weeks therapy and with minimal risk of side effects. In a Finnish study, 26% of the patients responded to VGB as the first-line drug. Some of the non-responders responded to ACTH. In tuberous sclerosis, the initial response rate to ACTH was high (73%) and did not differ from the response rate to VGB in other series. Both drugs have severe side effects. The visual field defects caused by VGB occur even in children (in 18/91 Finnish children). The patients with cryptogenic spasms, who responded well to ACTH, differed in their biochemical parameters from the patients with symptomatic spasms. The therapeutic action of ACTH may be mediated by potentiation of nerve growth promoting activity. Neurodegeneration may be due to imbalance between nerve growth factors and nitrate/nitrite in the brain. ACTH should be used as the first choice for treatment of West syndrome (at the minimal effective dose and for shortest effective time). The side effects of steroids, unlike VGB, are well known, treatable, and reversible.

摘要

为提供有关促肾上腺皮质激素(ACTH)治疗韦斯特综合征的最新信息,针对以下问题对芬兰的研究进行了综述:(1)其疗效如何:剂量以及与氨己烯酸(VGB)的比较;(2)其耐受性如何;(3)其作用机制是什么?为什么有些患者有反应,而有些患者没有?没有其他药物被证明比ACTH更有效。高剂量并不比低剂量更有效。合成衍生物的副作用更频繁。基于病因和反应制定了个体化治疗方案。采用ACTH 3 - 6IU/kg/天的治疗方案,在2 - 3周以上的治疗时间内,所有隐源性痉挛和一半的症状性痉挛都可以得到控制,且副作用风险最小。在一项芬兰研究中,26%的患者对作为一线药物的VGB有反应。一些无反应者对ACTH有反应。在结节性硬化症中,ACTH的初始反应率很高(73%),与其他系列中VGB的反应率没有差异。两种药物都有严重的副作用。VGB引起的视野缺损甚至在儿童中也会出现(在91名芬兰儿童中有18名出现)。对ACTH反应良好的隐源性痉挛患者,其生化参数与症状性痉挛患者不同。ACTH的治疗作用可能是通过增强神经生长促进活性来介导的。神经退行性变可能是由于大脑中神经生长因子与硝酸盐/亚硝酸盐之间的失衡所致。ACTH应作为韦斯特综合征治疗的首选药物(采用最小有效剂量并在最短有效时间内使用)。与VGB不同,类固醇的副作用是众所周知的,可治疗且可逆。

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