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孟加拉国达卡一家三级护理医院的婴儿痉挛综合征患儿的临床特征。

Clinical characteristics of children with infantile epileptic spasms syndrome from a tertiary-care hospital in Dhaka, Bangladesh.

作者信息

Abath Christina Briscoe, Chandra Saha Narayan, Hoque Seikh Azimul, Islam Ariful, Chowdhury Yamin Shahriar, Ara Begum Mosammat Shameem, Davalji Kanjiker Tahera Sultana, Yuskaitis Christopher J, Harini Chellamani, Alam Md Badrul, Mohammed Quazi Deen, Mazumdar Maitreyi

机构信息

Department of Neurology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, USA.

Department of Pediatric Neurology, National Institute of Neurosciences & Hospital, Sher-E-Bangla Nagar, Agargaon, Dhaka, 1207, Bangladesh.

出版信息

Heliyon. 2023 Mar 5;9(3):e14323. doi: 10.1016/j.heliyon.2023.e14323. eCollection 2023 Mar.

Abstract

BACKGROUND

We describe patient characteristics and response to initial treatment in a large case series of children presenting with infantile epileptic spasms syndrome to a tertiary-care hospital with a pediatric neurology service in Bangladesh. The purpose of the study was to add to the growing body of literature on infantile epileptic spasms syndrome in low- and middle-income countries.

METHODS

We enrolled 212 infants with new-onset infantile epileptic spasms syndrome (IESS) at the time of initial presentation to the National Institute of Neurosciences and Hospital (NINS) in Dhaka, Bangladesh, between January 2019 and August 2021. We collected data about seizure type and frequency, etiology, medication dosage, and available neuroimaging.

RESULTS

Median age at initial presentation to NINS was 9 months. Developmental delay and regression prior to presentation were found in 83% and 36%, respectively. Prior to their presentation at NINS, 197 (93%) patients had received anti-seizure medication to treat spasms, of whom only 8 (4%) had received standard therapy with ACTH, prednisolone, or vigabatrin. At NINS, 207 (98%) of patients received standard therapy, most frequently ACTH in 154 (73%). Median time between seizure onset to receipt of first-line therapy was 5 months. Of the 169 patients who were seen in follow-up at average of 5 weeks, 92 (54%) reported absence of clinical epileptic spasms. No serious adverse events requiring hospitalization were reported.

CONCLUSIONS

This study highlights the long lead times to treatment for IESS in a low- and middle-income country, and the need for early referral of children with suspected epileptic spasms to epilepsy care centers.

摘要

背景

我们描述了在孟加拉国一家设有儿科神经科服务的三级医院就诊的大量婴儿痉挛症综合征患儿的患者特征及对初始治疗的反应。本研究的目的是补充中低收入国家关于婴儿痉挛症综合征的不断增长的文献。

方法

2019年1月至2021年8月期间,我们在孟加拉国达卡的国立神经科学与医院(NINS)对212例初发婴儿痉挛症综合征(IESS)的婴儿进行了登记。我们收集了癫痫发作类型和频率、病因、药物剂量以及可用的神经影像学数据。

结果

初次到NINS就诊的中位年龄为9个月。分别有83%和36%的患儿在就诊前出现发育迟缓及发育倒退。在他们到NINS就诊之前,197例(93%)患者曾接受抗癫痫药物治疗痉挛,其中只有8例(4%)接受了促肾上腺皮质激素(ACTH)、泼尼松龙或氨己烯酸的标准治疗。在NINS,207例(98%)患者接受了标准治疗,最常用的是ACTH,有154例(73%)。癫痫发作开始至接受一线治疗的中位时间为5个月。在平均5周后接受随访的169例患者中,92例(54%)报告临床癫痫痉挛消失。未报告需要住院治疗的严重不良事件。

结论

本研究强调了在中低收入国家IESS治疗的长时间延迟,以及需要将疑似癫痫痉挛的儿童尽早转诊至癫痫护理中心。

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West Syndrome: A Review and Guide for Paediatricians.婴儿痉挛症:儿科医生的综述与指导。
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