156 例婴儿痉挛症治疗反应模式：静脉用合成 ACTH 激素治疗有一定前景。
The response patterns of infantile spasms to treatments in 156 patients: Hormonal therapy with intravenous synthetic ACTH appears promising.
机构信息
From the Department of Pediatrics (Alrifai, Al-Rumayyan, Al-Tuwaijri, Baarmah, Bali), Neurology Division, King Abdullah Specialist Children's Hospital, King Abdulaziz Medical City, from the College of Medicine (Alrifai, Al-Rumayyan, Al-Tuwaijri, Baarmah, Asiri, Bali, Alsadhan, Alsugheir), King Saud bin Abdulaziz University for Health Sciences, and from King Abdullah International Medical Research Center (Alrifai, Al-Rumayyan, Al-Tuwaijri, Baarmah, Asiri, Bali), Riyadh, Kingdom of Saudi Arabia.
出版信息
Neurosciences (Riyadh). 2022 Jan;27(1):40-44. doi: 10.17712/nsj.2022.1.20210116.
OBJECTIVES
To report the efficacy of intravenous (IV) synthetic ACTH (Tetracosactide) in the treatment of infantile spasms.
METHODS
This is a retrospective chart review of patients with a diagnosis of infantile spasms conducted at the Pediatric Department of King Abdulaziz Medical City (KAMC) in Riyadh, Saudi Arabia, from 01-01-2005 to 31-12-2019.
RESULTS
Of the 156 cases, 141 were treated initially with vigabatrin (VGB) with a complete response seen in 42(30%). Synthetic ACTH (Tetracosactide) IV injections were used in a total of 52 cases with response in 25(48%). Of the 35 cases which initially failed with VGB, 20(57%) responded to synthetic ACTH. The injections were used as a first line in 8 cases with response in 6(75%). The response to oral steroids was seen in 4/14(29%) cases. A relapse was seen in 2/42(5%) of patients treated with VGB and in 5/25(20%) of those who were treated with synthetic ACTH. The response was highest in the idiopathic group with 7/7(100%). Epilepsy at 2 years was seen in 26/50(52%) and 50/57(88%) of the responders and non-responders, respectively (=0.000). Only 14/156(9%) of cases had a fair neurological outcome. All of them were from the responder group CONCLUSION: The response to VGB is suboptimal, while the response to synthetic ACTH is encouraging making it a good alternative for natural ACTH as a potential first line therapy in infantile spasms.
目的
报告静脉内（IV）合成 ACTH（四氢皮质素）治疗婴儿痉挛的疗效。
方法
这是对沙特阿拉伯利雅得阿卜杜勒阿齐兹医疗城（KAMC）儿科部门 2005 年 1 月 1 日至 2019 年 12 月 31 日期间诊断为婴儿痉挛的患者进行的回顾性图表审查。
结果
在 156 例病例中，141 例最初用氨己烯酸（VGB）治疗，完全缓解 42 例（30%）。总共对 52 例患者使用了合成 ACTH（四氢皮质素）IV 注射，其中 25 例（48%）有反应。在最初对 VGB 无反应的 35 例病例中，有 20 例（57%）对合成 ACTH 有反应。该注射作为一线药物使用了 8 例，其中 6 例（75%）有反应。4/14（29%）例患者口服类固醇有反应。VGB 治疗的 2/42（5%）例患者和合成 ACTH 治疗的 5/25（20%）例患者出现复发。特发性组的反应率最高，7/7（100%）。2 年时癫痫发作分别见于 VGB 治疗的 26/50（52%）和合成 ACTH 治疗的 50/57（88%）的患者（=0.000）。只有 14/156（9%）例患者有较好的神经功能结局。他们都来自反应者组。
结论
VGB 的反应不理想，而合成 ACTH 的反应令人鼓舞，使其成为婴儿痉挛的天然 ACTH 的良好替代药物，作为潜在的一线治疗方法。
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