Management practices for West syndrome in South Asia: A survey study and meta-analysis
Management practices for West syndrome in South Asia: A survey study and meta-analysis
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DOI：
10.1002/epi4.12419
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发表时间：
2020-09-01
期刊：
影响因子：
3
通讯作者：
Sahu, Jitendra Kumar
中科院分区：
文献类型：
--
作者：
Madaan, Priyanka;Chand, Prem;Sahu, Jitendra Kumar
针对南亚国家有关西方综合征(WS)的文献资料缺乏的情况，本研究旨在通过在线调查和元分析来评估南亚的管理实践。方法对印度、巴基斯坦、缅甸、斯里兰卡、不丹、尼泊尔和孟加拉国的223名儿科神经科医生/儿科医生进行在线问卷调查。结果在收到的125份回复中(应答率：56%)，约60%的应答者注意到男性占优势，治疗间隔时间(LTTT)约为4-12周。观察到的最常见的病因是静态结构性侮辱(88.6%的应答者)。最常用的一线药物(国别)如下：印度-促肾上腺皮质激素(ACTH，50%)；巴基斯坦-口服类固醇(45.5%)；缅甸、斯里兰卡和尼泊尔-口服类固醇(94.4%)；孟加拉国-ACTH(2/2)；不丹-Vigabatrin(3/5)。在缅甸和尼泊尔没有ACTH和Vigabatrin。ACTH最常用的治疗方案是印度、斯里兰卡和孟加拉国的启动时最大剂量方案，以及巴基斯坦逐渐升级的方案。强的松龙的最大剂量是可变的--最常见的反应来自印度：3-4 mg/kg/d；巴基斯坦、不丹和孟加拉国：2 mg/kg/d；斯里兰卡、尼泊尔和缅甸：5-8 mg/kg/d或60 mg/d。激素治疗(包括逐渐减少)的总持续时间为4至12周(67/91)。大多数应答者认为痉挛停止四周为完全反应(54/111)，并建议脑电图(EEG；104/123)检查低律律性心律失常的缓解。不丹和尼泊尔难以获得儿科脑电图仪令人担忧。超过95%的应答者认为需要更多的意识。Meta分析支持南亚WS中男性(68%；可信区间：%-73%)、结构病因(80%；CI 73%-86%)、较长的LTTT(2.4个月；CI 2.1-2.6个月)以及对激素治疗的低应答率(ACTH和口服类固醇分别为18%和28%)。这些因素包括男性占多数和结构性病因，较长的LTTT，难以获得儿科EEG，在一些国家无法获得ACTH和Vigabatrin，以及该地区激素治疗的低有效性。
ObjectivesConsidering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta-analysis.MethodsAn online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta-analysis.ResultsOf 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead-time-to-treatment (LTTT) of 4-12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first-line drug (country-wise) was as follows: India-adrenocorticotropin hormone (ACTH, 50%); Pakistan-oral steroids (45.5%); Myanmar, Sri Lanka, and Nepal-oral steroids (94.4%); Bangladesh-ACTH (2/2); Bhutan-vigabatrin (3/5). ACTH and vigabatrin are not available in Myanmar and Nepal. The most commonly used regime for ACTH was maximal-dose-at-initiation-regime in India, Sri Lanka, and Bangladesh and gradually escalating-regime in Pakistan. Maximum dose of prednisolone was variable-most common response from India: 3-4 mg/kg/d; Pakistan, Bhutan, and Bangladesh: 2 mg/kg/d; Sri Lanka, Nepal, and Myanmar: 5-8 mg/kg/d or 60 mg/d. The total duration of hormonal therapy (including tapering) ranged from 4 to 12 weeks (67/91). Most responders considered cessation of spasms for four weeks as complete response (54/111) and advised electroencephalography (EEG; 104/123) to check for hypsarrhythmia resolution. Difficult access to pediatric EEG in Bhutan and Nepal is concerning. More than 95% of responders felt a need for more awareness. The meta-analysis supported the preponderance of male gender (68%; confidence interval [CI]: 64%-73%), structural etiology(80%; CI 73%-86%), longer LTTT (2.4 months; CI 2.1-2.6 months), and low response rate to hormonal therapy(18% and 28% for ACTH and oral steroids respectively) in WS in South Asia.SignificanceThis study highlights the practices and challenges in the management of WS in South Asia. These include a preponderance of male gender and structural etiology, a longer LTTT, difficult access to pediatric EEG, nonavailability of ACTH and vigabatrin in some countries, and low effectiveness of hormonal therapy in this region.