目的:考虑到南亚国家关于西方综合征(WS)的文献匮乏,本研究旨在通过在线调查和荟萃分析评估南亚的管理实践。
方法:向印度的223名儿科神经学家/儿科医生发送了一份在线调查问卷,巴基斯坦,缅甸,斯里兰卡,不丹,尼泊尔,孟加拉国。通过荟萃分析评估和补充他们的反应。
结果:收到125份答复(答复率:56%),约60%的应答者观察到男性占优,治疗前时间(LTTT)约为4-12周.观察到的最常见的病因是静态结构损伤(88.6%的应答者)。最常用的一线药物(国家)如下:印度-促肾上腺皮质激素激素(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)。ACTH和vigabatrin在缅甸和尼泊尔不可用。在印度,ACTH最常用的方案是起始时最大剂量方案,斯里兰卡,和孟加拉国,并逐渐升级巴基斯坦的政权。泼尼松龙的最大剂量是印度最常见的反应:3-4mg/kg/d;巴基斯坦,不丹,孟加拉国:2毫克/千克/天;斯里兰卡,尼泊尔,缅甸:5-8毫克/千克/天或60毫克/天。激素治疗(包括逐渐减少)的总持续时间为4至12周(67/91)。大多数反应者将痉挛停止四周视为完全反应(54/111),并建议脑电图(EEG;104/123)检查心律失常的解决情况。在不丹和尼泊尔,难以获得小儿脑电图。超过95%的响应者认为需要更多的意识。荟萃分析支持男性占主导地位(68%;置信区间[CI]:64%-73%),结构性病因(80%;CI73%-86%),较长的LTTT(2.4个月;CI2.1-2.6个月),南亚WS对激素治疗的反应率低(ACTH和口服类固醇分别为18%和28%)。
结论:本研究强调了南亚WS管理的实践和挑战。这些包括男性性别和结构病因的优势,较长的LTTT,难以获得小儿脑电图,在一些国家没有ACTH和vigabatrin,以及该地区激素治疗的低效率。
OBJECTIVE: Considering 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.
METHODS: An 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.
RESULTS: Of 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.
CONCLUSIONS: This
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.