婴幼儿癫痫治疗是否应使用标签外抗癫痫药物?基于草稿综合征病例报告的讨论

IF 0.2 Q4 PEDIATRICS Journal of Pediatric Epilepsy Pub Date : 2020-05-28 DOI:10.1055/s-0040-1712498
M. Vințan
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引用次数: 0

摘要

癫痫是一种负担沉重的神经系统疾病,18岁前发病率较高。治疗是医学上的,包括长期服用抗癫痫药物(AED)。有一些已知的高耐药综合征,发病于婴儿期和儿童期,德拉韦综合征就是其中之一。众所周知,早期癫痫治疗可以预防相关的合并症、认知和运动障碍,并改善长期预后。有几种可用的aed,但并不是所有的都被批准用于婴儿。这是由于需要在这个年龄进行额外的毒理学研究和开发合适的配方。一个14个月大的女孩患有德拉韦综合征。根据临床特征及时诊断,并经基因检测证实。她对丙戊酸盐和氯巴唑有部分反应,但持续出现长时间的发热性惊厥。我们在查阅了2岁以下婴儿的研究报告并征得家属同意后加入了斯曲妥醇。她的反应良好,癫痫持续状态发作减少,精神运动发展改善,斯立哌丁醇耐受性良好。当与年龄较大的儿童相比,没有主要的药代动力学差异时,非标签使用某些aed可以使婴儿受益。
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Should Off Label Antiepileptic Drugs Be Used for Treatment of Infancy and Childhood Epilepsy? Discussions Based on a Dravet Syndrome Case Report
Abstract Epilepsy represents a burdensome neurological disorder with higher incidence before the age of 18 years. The treatment is medical and involves long-term administration of antiepileptic drugs (AED). There are known high-resistant syndromes with onset in infancy and childhood, Dravet syndrome, being one of them. It is a well-known fact that early seizures treatment prevents associated comorbidities, cognitive and motor disabilities, and improve long-term prognosis. There are several AEDs available but not all of them are approved for use in infants. This is due to the need for additional toxicology studies at this age and for development of suitable formulations. A 14-month-old girl with Dravet syndrome was presented here. Prompt diagnosis was made based on clinical features and confirmed by the genetic tests. She partially responded to valproate and clobazam but continued to have prolonged febrile seizures. We added stiripentol after consulting reports of studies in infants younger than 2 years and after obtaining family consent. She responded well with decrease in episodes of status epilepticus and improvement in psychomotor development and stiripentol was tolerated well. Off label use of certain AEDs can benefit infants when there are no major pharmacokinetic differences in comparison to older children.
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期刊介绍: The Journal of Pediatric Epilepsy is an English multidisciplinary peer-reviewed international journal publishing articles on all topics related to epilepsy and seizure disorders, epilepsy surgery, neurology, neurosurgery, and neuropsychology in childhood. These topics include the basic sciences related to the condition itself, the differential diagnosis, natural history, and epidemiology of seizures, and the investigation and practical management of epilepsy (including drug treatment, neurosurgery and non-medical and behavioral treatments). Use of model organisms and in vitro techniques relevant to epilepsy are also acceptable. Journal of Pediatric Epilepsy provides an in-depth update on new subjects and current comprehensive coverage of the latest techniques used in the diagnosis and treatment of childhood epilepsy.
期刊最新文献
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