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儿童多发性硬化的一线疾病修正治疗:全面概述。

First-line disease-modifying therapies in paediatric multiple sclerosis: a comprehensive overview.

机构信息

University of North Carolina at Chapel Hill, Eshelman School of Pharmacy, Chapel Hill, NC, USA.

出版信息

Drugs. 2012 Jun 18;72(9):1195-211. doi: 10.2165/11634010-000000000-00000.

Abstract

Paediatric multiple sclerosis (MS) is defined as the onset of MS before the age of 18 years. Immunomodulatory disease-modifying therapies (i.e. the interferons [IFNs] and glatiramer acetate) are considered first-line treatments in adult patients with MS, but they are largely understudied in the paediatric population. IFNβ is a type 1 IFN produced by fibroblasts. The therapeutic effect achieved by IFNβ in MS is believed to be the result of a variety of mechanisms, including the inhibition of T-cell proliferation and a shift in cytokine production. There are currently two forms of recombinant IFNβ used therapeutically for MS: IFNβ-1a and IFNβ-1b. Two formulations of IFNβ-1a exist, one administered as an intramuscular injection once weekly and the other by subcutaneous injection three times per week. Only one type of IFNβ-1b product is on the market, a subcutaneous injection administered every other day. Pharmacokinetic studies of these agents in children do not exist and available data are primarily from studies in healthy adults. It does not appear that the various formulations differ significantly in terms of bioavailability or efficacy in adults. The toxicity profiles of the interferon formulations are similar, with the most common adverse effects in children including flu-like symptoms, injection site reactions and transient elevations in liver enzymes. Glatiramer acetate is a mixture of synthetic polypeptide chains consisting of four different amino acids. Glatiramer acetate appears to mimic the antigenic properties of myelin basic protein (MBP), and by doing so, alters T-cell activation in the periphery. Glatiramer acetate is administered as a once-daily subcutaneous injection. Similar to the IFNβ formulations, there are no pharmacokinetic studies of this agent in children. The most common adverse effects include injection site reactions and transient chest tightness. Fingolimod, a sphingosine 1-phosphate receptor modulator, is a new disease-modifying therapy that was approved by the US FDA in 2010 for the first-line treatment of relapsing forms of MS in adults. However, due to a lack of information and clinical data on this agent in the paediatric population, it is not included in this discussion. Dose-finding studies of the IFNs and glatiramer acetate in the paediatric population are limited. Dosing recommendations are largely based on tolerability studies, with most children and adolescents tolerating the full adult doses. Clinical studies of IFNs in children have not been objectively designed to establish the efficacy of these therapies, and evidence is limited to that of observational trials and retrospective case reports. However, the largest cohort (130 cases) of paediatric MS patients studied to date reported a reduction in annual relapse rate with all three of the different IFNβ formulations and glatiramer acetate after a follow-up period of more than 4 years. Treatment with one of the first-line agents should be offered to any patient after the occurrence of a second demyelinating episode. The efficacy of the four first-line disease-modifying agents is considered to be relatively equivalent, and the choice of agent should be determined on an individual patient basis, taking into account potential adverse effects and patient preferences. Current data suggest that the IFNs and glatiramer acetate are safe and effective therapies in paediatric patients with MS. However, further studies evaluating the pharmacokinetics, appropriate dosing and comparisons of efficacy among these agents are needed to determine the most appropriate and evidence-based treatment decisions in this population.

摘要

儿科多发性硬化症(MS)定义为 18 岁以下发病的 MS。免疫调节疾病修饰疗法(即干扰素 [IFNs] 和醋酸格拉替雷)被认为是成人 MS 患者的一线治疗方法,但在儿科人群中研究甚少。IFNβ 是由成纤维细胞产生的 I 型 IFN。IFNβ 在 MS 中的治疗效果被认为是多种机制的结果,包括抑制 T 细胞增殖和细胞因子产生的转变。目前有两种用于治疗 MS 的重组 IFNβ 形式:IFNβ-1a 和 IFNβ-1b。IFNβ-1a 有两种制剂,一种每周肌内注射一次,另一种每周皮下注射三次。只有一种 IFNβ-1b 产品上市,每两天皮下注射一次。儿童的这些药物的药代动力学研究尚不存在,可用数据主要来自健康成年人的研究。似乎不同制剂在成人中的生物利用度或疗效方面没有显著差异。干扰素制剂的毒性谱相似,儿童中最常见的不良反应包括流感样症状、注射部位反应和肝酶短暂升高。醋酸格拉替雷是由四种不同氨基酸组成的合成多肽链混合物。醋酸格拉替雷似乎模仿髓鞘碱性蛋白(MBP)的抗原特性,通过这种方式改变外周 T 细胞的激活。醋酸格拉替雷每天皮下注射一次。与 IFNβ 制剂一样,儿童中也没有这种药物的药代动力学研究。最常见的不良反应包括注射部位反应和短暂的胸闷。芬戈莫德是一种鞘氨醇 1-磷酸受体调节剂,是一种新的疾病修饰疗法,于 2010 年被美国 FDA 批准用于成人复发性 MS 的一线治疗。然而,由于在儿科人群中缺乏关于该药物的信息和临床数据,因此不在本次讨论范围内。IFNs 和醋酸格拉替雷在儿科人群中的剂量确定研究有限。剂量建议主要基于耐受性研究,大多数儿童和青少年能耐受成人全剂量。IFN 在儿童中的临床研究并未专门设计来确定这些疗法的疗效,证据仅限于观察性试验和回顾性病例报告。然而,迄今为止研究最多的儿科 MS 患者队列(130 例)报告称,在超过 4 年的随访期后,所有三种不同的 IFNβ 制剂和醋酸格拉替雷均能降低年复发率。在发生第二次脱髓鞘事件后,应向任何患者提供一线治疗药物。这四种一线疾病修饰药物的疗效被认为是相对等效的,应根据个体患者的情况选择药物,同时考虑潜在的不良反应和患者的偏好。目前的数据表明,IFNs 和醋酸格拉替雷在儿科 MS 患者中是安全有效的治疗方法。然而,需要进一步研究来评估这些药物的药代动力学、适当剂量和疗效比较,以确定在该人群中最适当和最具循证依据的治疗决策。

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