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Article(id=1198558168910492535, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1198558165093675863, articleNumber=null, orderNo=null, doi=10.11855/j.issn.0577-7402.0212.2024.0229, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=null, receivedDate=1678636800000, receivedDateStr=2023-03-13, revisedDate=null, revisedDateStr=null, acceptedDate=1689609600000, acceptedDateStr=2023-07-18, onlineDate=1763688135601, onlineDateStr=2025-11-21, pubDate=1724774400000, pubDateStr=2024-08-28, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1763688135601, onlineIssueDateStr=2025-11-21, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1763688135601, creator=13701087609, updateTime=1763688135601, updator=13701087609, issue=Issue{id=1198558165093675863, tenantId=1146029695717560320, journalId=1189873630562394117, year='2024', volume='49', issue='8', pageStart='855', pageEnd='976', issueExtLink='null', onlineDate='null', pubDate='null', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=0, createTime=1763688134691, creator=13701087609, updateTime=1763689174168, updator=13701087609, preIssue=null, nextIssue=null, ext={EN=IssueExt(id=1198562525043327039, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1198558165093675863, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1198562525043327040, tenantId=1146029695717560320, journalId=1189873630562394117, issueId=1198558165093675863, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null}, startPage=868, endPage=875, ext={EN=ArticleExt(id=1198558171154445253, articleId=1198558168910492535, tenantId=1146029695717560320, journalId=1189873630562394117, language=EN, title=Influence factors and predictors on infantile epileptic spasms syndrome treatment response after adrenocorticotropic hormone, columnId=1190310109000602400, journalTitle=Medical Journal of Chinese People’s Liberation Army, columnName=Clinical Research, runingTitle=null, highlight=null, articleAbstract=

Objective To explore the influence factors and predictors of treatment response after adrenocorticotropic hormone (ACTH) in infantile epileptic spasms syndrome(IESS). Methods A retrospective analysis was conducted on 80 cases of IESS infants(50 males and 30 females) who were diagnosed and treated with ACTH in Chengdu Women's and Children's Central Hospital from January 2016 to December 2020. Patients were divided into effective group (n=39) and ineffective group (n=41) based on their response of ACTH treatment after 28 days, and their clinical data including the patients' basic information, etiology, treatment programmer, per- and post-treatment Kramer scores of electroencephalogram (EEG) hypsarrhythmia severity and so on, were collected to compare and analyze between the two groups. A modified Poisson regression model was constructed to discover predictors of outcome, and the receiver operating characteristic (ROC) curves were used to assess the prognosis evaluation of the positive predictive value. Results The ages at seizure onset ranged from one month and seven days to one year and nine months. Seizure types included simple epileptic spasms in 66 cases and combined with other types(focal and secondarily generalized seizures) in 14 cases. Thirty-two cases had been given anti-seizure medications (ASMs) before ACTH treatment. The median Kramer scores per-treatment and at 14 days post-treatment were 10.0 (8.3, 12.0) and 6.0 (4.0, 7.0), respectively. After ACTH treatment, 39(48.8%) cases were effective. Compared with the effective group, the ineffective group had significantly higher proportion of abnormal perinatal conditions, unknown aetiology with normal development, ASMs given before ACTH treatment, the dosages of ACTH greater than 2 U/(kg·d), combinations of two or more ASMs, poor control, and still seizure attack after ACTH treatment of 14 days (P<0.05). Additionally, the Kramer scores after ACTH treatment of 14 days in the ineffective group were also significantly higher (P<0.05). The modified Poisson regression model showed that there were significant statistic differences between the two groups on ASMs given before ACTH treatment (RR=0.546, 95%CI 0.357-0.833, P=0.005) and Kramer scores of hypsarrhythmia severity (RR=0.701, 95%CI 0.620-0.792, P<0.001),while there were no significant differences between the two groups in term of ages, gender, perinatal conditions, etiologies, seizure types, Kramer scores before treatment, time lag between onset and treatment, duration of ACTH treatment, kinds of ASMs combination. ROC curve analysis showed that only Kramer scores at 14 days after ACTH treatment could predict the treatment response with sensitivity and specificity of 92.7% and 84.6%, respectively, with Youden index of 0.773. The area under the ROC curve was 0.930 (95%CI 0.873-0.987, P<0.001) and the cut-point of the score was 6, indicating that the higher the Kramer scores at 14 days after ACTH treatment, the worse the treatment response. The treatment response rate would reduce by about 30.0% if the Kramer score increased by one point. Conclusion ASMs given before ACTH treatment may influence the treatment response. Kramer scores greater than 6 at day 14 after ACTH treatment may be used as a predictor of treatment response after ACTH in IESS patients.

, correspAuthors=Ping Liu, authorNote=null, correspAuthorsNote=
E-mail:
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目的 探讨促肾上腺皮质激素(ACTH)治疗婴儿癫痫性痉挛综合征(IESS)疗效的影响因素及其预测指标。方法 回顾性分析成都市妇女儿童中心医院2016年1月-2020年12月收治并给予ACTH治疗的80例IESS患儿(男50例，女30例)的临床资料。根据ACTH治疗28 d疗效分为有效组(n=39)与无效组(n=41)，收集并比较两组的临床资料，包括患儿的基本信息、病因、治疗方案及治疗前后脑电图高度失律严重程度评分(即Kramer评分)等指标。构建修正Poisson回归模型以发现疗效预测因素，采用受试者工作特征(ROC)曲线评估阳性指标的预测价值。结果 患儿起病年龄为1个月7 d~1岁9个月。发作形式包括单纯痉挛发作66例，合并其他发作如局灶发作、继发全面性发作14例。32例ACTH治疗前已给予抗癫痫发作药物(ASMs)治疗。治疗前、治疗后14 d的Kramer评分分别为10.0(8.3，12.0)分和6.0(4.0，7.0)分。经ACTH治疗后39例(48.8%)有效。与有效组比较，无效组中围产期存在异常、病因不明发育迟滞、ACTH治疗前已使用ASMs、ACTH给药剂量>2 U/(kg.d)、联合2种以上ASMs、治疗14 d时仍有发作的构成比高，且ACTH治疗14 d Kramer评分高，差异均具有统计学意义(P<0.05)。修正Poisson回归模型结果显示，两组ACTH治疗前是否使用ASMs(RR=0.546，95%CI 0.357~0.833，P=0.005)、治疗14 d后的Kramer评分(RR=0.701，95%CI 0.620~0.792，P<0.001)差异有统计学意义，而两组性别、年龄、围产期情况、病因、发作形式、治疗前Kramer评分、发病至治疗间隔时间、ACTH疗程、联合ASMs种类方面差异均无统计学意义(P>0.05)。ROC曲线分析显示，治疗14 d的Kramer评分对疗效有预测价值[曲线下面积(AUC)=0.930，95%CI 0.873~0.987，P<0.001]，敏感度为92.7%，特异度为84.6%，约登指数为0.773，即治疗14 d Kramer评分越高，疗效越差，以6分为截点值，每升高1分，疗效降低接近30.0%。结论 治疗前已使用ASMs可能会影响ACTH的疗效，治疗14 d后的Kramer评分≥6分或可作为ACTH治疗IESS疗效不佳的独立预测指标。

, correspAuthors=刘平, authorNote=null, correspAuthorsNote=
刘平，E-mail：
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毛丹丹，医学硕士，主治医师，主要从事儿童神经病学方面的研究

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毛丹丹，医学硕士，主治医师，主要从事儿童神经病学方面的研究

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Wirrell E, Yozawitz E, journalName=Epilepsia, refType=null, unstructuredReference=Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: position statement by the ILAE Task Force on Nosology and Definitions[J]. Epilepsia, 2022, 63(6): 1349-1397., articleTitle=ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: position statement by the ILAE Task Force on Nosology and Definitions, refAbstract=null), Reference(id=1198558178892935425, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2018, volume=38, issue=2, pageStart=113, pageEnd=124, url=null, language=null, rfNumber=[2], rfOrder=1, authorNames=D'Alonzo R, Rigante D, Mencaroni E, journalName=Clin Drug Investig, refType=null, unstructuredReference=D'Alonzo R, Rigante D, Mencaroni E, et al. West syndrome: a review and guide for paediatricians[J]. Clin Drug Investig, 2018, 38(2): 113-124., articleTitle=West syndrome: a review and guide for paediatricians, refAbstract=null), Reference(id=1198558178993598725, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2016, volume=64, issue=null, pageStart=239, pageEnd=241, url=null, language=null, rfNumber=[3], rfOrder=2, authorNames=Krag A, Holmes GL, journalName=Epilepsy Behav, refType=null, unstructuredReference=Krag A, Holmes GL. Diagnosing infantile spasms: accuracy of the internet[J]. Epilepsy Behav, 2016, 64(Pt A): 239-241., articleTitle=Diagnosing infantile spasms: accuracy of the internet, refAbstract=null), Reference(id=1198558179081679112, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=40, issue=2, pageStart=236, pageEnd=245, url=null, language=null, rfNumber=[4], rfOrder=3, authorNames=Messer R, Knupp KG, journalName=Semin Neurol, refType=null, unstructuredReference=Messer R, Knupp KG. Infantile spasms: opportunities to improve care[J]. Semin Neurol, 2020, 40(2): 236-245., articleTitle=Infantile spasms: opportunities to improve care, refAbstract=null), Reference(id=1198558179186536718, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=62, issue=2, pageStart=358, pageEnd=370, url=null, language=null, rfNumber=[5], rfOrder=4, authorNames=Howell KB, Freeman JL, MacKay MT, journalName=Epilepsia, refType=null, unstructuredReference=Howell KB, Freeman JL, MacKay MT, et al. The severe epilepsy syndromes of infancy: a population-based study[J]. Epilepsia, 2021, 62(2): 358-370., articleTitle=The severe epilepsy syndromes of infancy: a population-based study, refAbstract=null), Reference(id=1198558179278811412, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=27, issue=1, pageStart=29, pageEnd=37, url=null, language=null, rfNumber=[6], rfOrder=5, authorNames=Weingarten MM, Cokley JA, Moffett B, journalName=J Pediatr Pharmacol Ther, refType=null, unstructuredReference=Weingarten MM, Cokley JA, Moffett B, et al. Trends and costs associated with the diagnosis and treatment of infantile spasms: a 10-year multicenter retrospective review[J]. J Pediatr Pharmacol Ther, 2022, 27(1): 29-37., articleTitle=Trends and costs associated with the diagnosis and treatment of infantile spasms: a 10-year multicenter retrospective review, refAbstract=null), Reference(id=1198558179417223451, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=10, issue=1, pageStart=11509, pageEnd=null, url=null, language=null, rfNumber=[7], rfOrder=6, authorNames=Mao L, Kessi M, Peng P, journalName=Sci Rep, refType=null, unstructuredReference=Mao L, Kessi M, Peng P, et al. The patterns of response of 11 regimens for infantile spasms[J]. Sci Rep, 2020, 10(1): 11509., articleTitle=The patterns of response of 11 regimens for infantile spasms, refAbstract=null), Reference(id=1198558179538858270, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=173, issue=null, pageStart=106629, pageEnd=null, url=null, language=null, rfNumber=[8], rfOrder=7, authorNames=Bhanudeep S, Madaan P, Sankhyan N, journalName=Epilepsy Res, refType=null, unstructuredReference=Bhanudeep S, Madaan P, Sankhyan N, et al. Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome[J]. Epilepsy Res, 2021, 173: 106629., articleTitle=Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome, refAbstract=null), Reference(id=1198558179622744357, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2012, volume=78, issue=24, pageStart=1974, pageEnd=1980, url=null, language=null, rfNumber=[9], rfOrder=8, authorNames=Go CY, MacKay MT, Weiss SK, journalName=Neurology, refType=null, unstructuredReference=Go CY, MacKay MT, Weiss SK, et al. Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society[J]. Neurology, 2012, 78(24): 1974-1980., articleTitle=Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society, refAbstract=null), Reference(id=1198558179694047526, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2016, volume=47, issue=3, pageStart=139, pageEnd=150, url=null, language=null, rfNumber=[10], rfOrder=9, authorNames=Tibussek D, Klepper J, Korinthenberg R, journalName=Neuropediatrics, refType=null, unstructuredReference=Tibussek D, Klepper J, Korinthenberg R, et al. Treatment of infantile spasms: report of the interdisciplinary guideline committee coordinated by the german-speaking society for neuropediatrics[J]. Neuropediatrics, 2016, 47(3): 139-150., articleTitle=Treatment of infantile spasms: report of the interdisciplinary guideline committee coordinated by the german-speaking society for neuropediatrics, refAbstract=null), Reference(id=1198558179777933608, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2015, volume=56, issue=8, pageStart=1185, pageEnd=1197, url=null, language=null, rfNumber=[11], rfOrder=10, authorNames=Wilmshurst JM, Gaillard WD, Vinayan KP, journalName=Epilepsia, refType=null, unstructuredReference=Wilmshurst JM, Gaillard WD, Vinayan KP, et al. Summary of recommendations for the management of infantile seizures: task Force Report for the ILAE Commission of Pediatrics[J]. Epilepsia, 2015, 56(8): 1185-1197., articleTitle=Summary of recommendations for the management of infantile seizures: task Force Report for the ILAE Commission of Pediatrics, refAbstract=null), Reference(id=1198558179849236778, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2016, volume=79, issue=3, pageStart=475, pageEnd=484, url=null, language=null, rfNumber=[12], rfOrder=11, authorNames=Knupp KG, Coryell J, Nickels KC, journalName=Ann Neurol, refType=null, unstructuredReference=Knupp KG, Coryell J, Nickels KC, et al. Response to treatment in a prospective national infantile spasms cohort[J]. Ann Neurol, 2016, 79(3): 475-484., articleTitle=Response to treatment in a prospective national infantile spasms cohort, refAbstract=null), Reference(id=1198558179954094384, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2017, volume=19, issue=4, pageStart=357, pageEnd=365, url=null, language=null, rfNumber=[13], rfOrder=12, authorNames=Yin J, Lu Q, Yin F, journalName=Paediatr Drugs, refType=null, unstructuredReference=Yin J, Lu Q, Yin F, et al. Effectiveness and safety of different once-daily doses of adrenocorticotropic hormone for infantile spasms[J]. Paediatr Drugs, 2017, 19(4): 357-365., articleTitle=Effectiveness and safety of different once-daily doses of adrenocorticotropic hormone for infantile spasms, refAbstract=null), Reference(id=1198558180058951988, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2004, volume=45, issue=11, pageStart=1416, pageEnd=1428, url=null, language=null, rfNumber=[14], rfOrder=13, authorNames=Lux AL, Osborne JP, journalName=Epilepsia, refType=null, unstructuredReference=Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi Group[J]. Epilepsia, 2004, 45(11): 1416-1428., articleTitle=A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi Group, refAbstract=null), Reference(id=1198558180147032376, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=1997, volume=48, issue=1, pageStart=197, pageEnd=203, url=null, language=null, rfNumber=[15], rfOrder=14, authorNames=Kramer U, Sue WC, Mikati MA, journalName=Neurology, refType=null, unstructuredReference=Kramer U, Sue WC, Mikati MA. Hypsarrhythmia: frequency of variant patterns and correlation with etiology and outcome[J]. Neurology, 1997, 48(1): 197-203., articleTitle=Hypsarrhythmia: frequency of variant patterns and correlation with etiology and outcome, refAbstract=null), Reference(id=1198558180243501372, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=36, issue=10, pageStart=794, pageEnd=797, url=null, language=null, rfNumber=[16], rfOrder=15, authorNames=孙于林, 陈健, 杨光, journalName=中华实用儿科临床杂志, refType=null, unstructuredReference=孙于林, 陈健, 杨光. 脑电图评分在婴儿痉挛症的应用进展[J]. 中华实用儿科临床杂志, 2021, 36(10): 794-797., articleTitle=脑电图评分在婴儿痉挛症的应用进展, refAbstract=null), Reference(id=1198558180327387454, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=39, issue=7, pageStart=538, pageEnd=543, url=null, language=null, rfNumber=[17], rfOrder=16, authorNames=Lachhwani D, journalName=J Clin Neurophysiol, refType=null, unstructuredReference=Lachhwani D. Scoring systems for the evaluation of hypsarrhythmia[J]. J Clin Neurophysiol, 2022, 39(7): 538-543., articleTitle=Scoring systems for the evaluation of hypsarrhythmia, refAbstract=null), Reference(id=1198558180415467840, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=104, issue=null, pageStart=23, pageEnd=29, url=null, language=null, rfNumber=[18], rfOrder=17, authorNames=Mytinger JR, Albert DVF, Twanow JD, journalName=Pediatr Neurol, refType=null, unstructuredReference=Mytinger JR, Albert DVF, Twanow JD, et al. Compliance with standard therapies and remission rates after implementation of an infantile spasms management guideline[J]. Pediatr Neurol, 2020, 104: 23-29., articleTitle=Compliance with standard therapies and remission rates after implementation of an infantile spasms management guideline, refAbstract=null), Reference(id=1198558180507742530, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=39, issue=7, pageStart=521, pageEnd=528, url=null, language=null, rfNumber=[19], rfOrder=18, authorNames=Pestana Knight EM, Mani J, journalName=J Clin Neurophysiol, refType=null, unstructuredReference=Pestana Knight EM, Mani J. Historical overview of hypsarrhythmia and its association to epileptic spasms: a review of the medical literature from 1952 to 1982[J]. J Clin Neurophysiol, 2022, 39(7): 521-528., articleTitle=Historical overview of hypsarrhythmia and its association to epileptic spasms: a review of the medical literature from 1952 to 1982, refAbstract=null), Reference(id=1198558180604211525, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=13, issue=null, pageStart=772333, pageEnd=null, url=null, language=null, rfNumber=[20], rfOrder=19, authorNames=Guang S, Mao L, Zhong L, journalName=Front Neurol, refType=null, unstructuredReference=Guang S, Mao L, Zhong L, et al. Hormonal therapy for infantile spasms: a systematic review and meta-analysis[J]. Front Neurol, 2022, 13: 772333., articleTitle=Hormonal therapy for infantile spasms: a systematic review and meta-analysis, refAbstract=null), Reference(id=1198558180709069128, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=97, issue=12, pageStart=e1217, pageEnd=e1228, url=null, language=null, rfNumber=[21], rfOrder=20, authorNames=Grinspan ZM, Knupp KG, Patel AD, journalName=Neurology, refType=null, unstructuredReference=Grinspan ZM, Knupp KG, Patel AD, et al. Comparative effectiveness of initial treatment for infantile spasms in a contemporary US cohort[J]. Neurology, 2021, 97(12): e1217-e1228., articleTitle=Comparative effectiveness of initial treatment for infantile spasms in a contemporary US cohort, refAbstract=null), Reference(id=1198558180834898249, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=27, issue=1, pageStart=40, pageEnd=44, url=null, language=null, rfNumber=[22], rfOrder=21, authorNames=Alrifai MT, Al-Rumayyan AR, Al-Tuwaijri WA, journalName=Neurosciences (Riyadh), refType=null, unstructuredReference=Alrifai MT, Al-Rumayyan AR, Al-Tuwaijri WA, et al. The response patterns of infantile spasms to treatments in 156 patients: Hormonal therapy with intravenous synthetic ACTH appears promising[J]. Neurosciences (Riyadh), 2022, 27(1): 40-44., articleTitle=The response patterns of infantile spasms to treatments in 156 patients: Hormonal therapy with intravenous synthetic ACTH appears promising, refAbstract=null), Reference(id=1198558180914590027, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2012, volume=78, issue=6, pageStart=735, pageEnd=737, url=null, language=null, rfNumber=[23], rfOrder=22, authorNames=Shi XY, Zou LP, Yang G, journalName=Med Hypotheses, refType=null, unstructuredReference=Shi XY, Zou LP, Yang G, et al. Prenatal stress exposure hypothesis for infantile spasms[J]. Med Hypotheses, 2012, 78(6): 735-737., articleTitle=Prenatal stress exposure hypothesis for infantile spasms, refAbstract=null), Reference(id=1198558181019447632, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=746, issue=null, pageStart=135618, pageEnd=null, url=null, language=null, rfNumber=[24], rfOrder=23, authorNames=Basu T, Maguire J, Salpekar JA, journalName=Neurosci Lett, refType=null, unstructuredReference=Basu T, Maguire J, Salpekar JA. Hypothalamic-pituitary-adrenal axis targets for the treatment of epilepsy[J]. Neurosci Lett, 2021, 746: 135618., articleTitle=Hypothalamic-pituitary-adrenal axis targets for the treatment of epilepsy, refAbstract=null), Reference(id=1198558181107528021, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=21, issue=4, pageStart=420, pageEnd=424, url=null, language=null, rfNumber=[25], rfOrder=24, authorNames=张艳, 姬辛娜, 陈倩, journalName=中华神经医学杂志, refType=null, unstructuredReference=张艳, 姬辛娜, 陈倩. 促肾上腺皮质激素治疗婴儿痉挛症的临床和基础研究进展[J]. 中华神经医学杂志, 2022, 21(4): 420-424., articleTitle=促肾上腺皮质激素治疗婴儿痉挛症的临床和基础研究进展, refAbstract=null), Reference(id=1198558181208191322, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2019, volume=61, issue=11, pageStart=1295, pageEnd=1301, url=null, language=null, rfNumber=[26], rfOrder=25, authorNames=Sumanasena SP, Wanigasinghe J, Arambepola C, journalName=Dev Med Child Neurol, refType=null, unstructuredReference=Sumanasena SP, Wanigasinghe J, Arambepola C, et al. Developmental profile at initial presentation in children with infantile spasms[J]. Dev Med Child Neurol, 2019, 61(11): 1295-1301., articleTitle=Developmental profile at initial presentation in children with infantile spasms, refAbstract=null), Reference(id=1198558181308854623, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=108, issue=null, pageStart=54, pageEnd=64, url=null, language=null, rfNumber=[27], rfOrder=26, authorNames=Riikonen R, journalName=Pediatr Neurol, refType=null, unstructuredReference=Riikonen R. Infantile spasms: outcome in clinical studies[J]. Pediatr Neurol, 2020, 108: 54-64., articleTitle=Infantile spasms: outcome in clinical studies, refAbstract=null), Reference(id=1198558181413712225, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=89, issue=null, pageStart=73, pageEnd=80, url=null, language=null, rfNumber=[28], rfOrder=27, authorNames=Bitton JY, Desnous B, Sauerwein HC, journalName=Seizure, refType=null, unstructuredReference=Bitton JY, Desnous B, Sauerwein HC, et al. Cognitive outcome in children with infantile spasms using a standardized treatment protocol. A five-year longitudinal study[J]. Seizure, 2021, 89: 73-80., articleTitle=Cognitive outcome in children with infantile spasms using a standardized treatment protocol. A five-year longitudinal study, refAbstract=null), Reference(id=1198558181501792612, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=63, issue=5, pageStart=1164, pageEnd=1176, url=null, language=null, rfNumber=[29], rfOrder=28, authorNames=Chourasia N, Yuskaitis CJ, Libenson MH, journalName=Epilepsia, refType=null, unstructuredReference=Chourasia N, Yuskaitis CJ, Libenson MH, et al. Infantile spasms: assessing the diagnostic yield of an institutional guideline and the impact of etiology on long-term treatment response[J]. Epilepsia, 2022, 63(5): 1164-1176., articleTitle=Infantile spasms: assessing the diagnostic yield of an institutional guideline and the impact of etiology on long-term treatment response, refAbstract=null), Reference(id=1198558182185464168, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2021, volume=85, issue=null, pageStart=6, pageEnd=11, url=null, language=null, rfNumber=[30], rfOrder=29, authorNames=Daida A, Hamano SI, Hayashi K, journalName=Seizure, refType=null, unstructuredReference=Daida A, Hamano SI, Hayashi K, et al. Comparison of adrenocorticotropic hormone efficacy between aetiologies of infantile spasms[J]. Seizure, 2021, 85: 6-11., articleTitle=Comparison of adrenocorticotropic hormone efficacy between aetiologies of infantile spasms, refAbstract=null), Reference(id=1198558182365819242, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=47, issue=6, pageStart=1585, pageEnd=1595, url=null, language=null, rfNumber=[31], rfOrder=30, authorNames=Dang HD, Zou LP, Tian JH, journalName=Eur J Nucl Med Mol Imaging, refType=null, unstructuredReference=Dang HD, Zou LP, Tian JH, et al. Etiologic classification of infantile spasms using positron emission/magnetic resonance imaging and the efficacy of adrenocorticotropic hormone therapy[J]. Eur J Nucl Med Mol Imaging, 2020, 47(6): 1585-1595., articleTitle=Etiologic classification of infantile spasms using positron emission/magnetic resonance imaging and the efficacy of adrenocorticotropic hormone therapy, refAbstract=null), Reference(id=1198558182441316718, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2022, volume=64, issue=11, pageStart=1330, pageEnd=1343, url=null, language=null, rfNumber=[32], rfOrder=31, authorNames=Jain P, Sahu JK, Horn PS, journalName=Dev Med Child Neurol, refType=null, unstructuredReference=Jain P, Sahu JK, Horn PS, et al. Treatment of children with infantile spasms: a network meta-analysis[J]. Dev Med Child Neurol, 2022, 64(11): 1330-1343., articleTitle=Treatment of children with infantile spasms: a network meta-analysis, refAbstract=null), Reference(id=1198558182516814188, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2017, volume=58, issue=12, pageStart=2098, pageEnd=2103, url=null, language=null, rfNumber=[33], rfOrder=32, authorNames=Demarest ST, Shellhaas RA, Gaillard WD, journalName=Epilepsia, refType=null, unstructuredReference=Demarest ST, Shellhaas RA, Gaillard WD, et al. The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium[J]. Epilepsia, 2017, 58(12): 2098-2103., articleTitle=The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium, refAbstract=null), Reference(id=1198558182638449006, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2012, volume=34, issue=22, pageStart=2257, pageEnd=2259, url=null, language=null, rfNumber=[34], rfOrder=33, authorNames=钟金花, 廖建湘, 胡雁, journalName=第三军医大学学报, refType=null, unstructuredReference=钟金花, 廖建湘, 胡雁, 等. 促肾上腺皮质激素治疗婴儿痉挛症的疗效分析[J]. 第三军医大学学报, 2012, 34(22): 2257-2259., articleTitle=促肾上腺皮质激素治疗婴儿痉挛症的疗效分析, refAbstract=null), Reference(id=1198558182743306608, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2011, volume=52, issue=7, pageStart=1359, pageEnd=1364, url=null, language=null, rfNumber=[35], rfOrder=34, authorNames=O'Callaghan FJ, Lux AL, Darke K, journalName=Epilepsia, refType=null, unstructuredReference=O'Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study[J]. Epilepsia, 2011, 52(7): 1359-1364., articleTitle=The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study, refAbstract=null), Reference(id=1198558182852358514, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2020, volume=14, issue=null, pageStart=100397, pageEnd=null, url=null, language=null, rfNumber=[36], rfOrder=35, authorNames=Surana P, Symonds JD, Srivastava P, journalName=Epilepsy Behav Rep, refType=null, unstructuredReference=Surana P, Symonds JD, Srivastava P, et al. Infantile spasms: Etiology, lead time and treatment response in a resource limited setting[J]. Epilepsy Behav Rep, 2020, 14: 100397., articleTitle=Infantile spasms: Etiology, lead time and treatment response in a resource limited setting, refAbstract=null), Reference(id=1198558182936244597, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2017, volume=48, issue=4, pageStart=280, pageEnd=287, url=null, language=null, rfNumber=[37], rfOrder=36, authorNames=Sehgal R, Gulati S, Sapra S, journalName=Clin EEG Neurosci, refType=null, unstructuredReference=Sehgal R, Gulati S, Sapra S, et al. Prognostic utility of clinical epilepsy severity score versus pretreatment hypsarrhythmia scoring in children with west syndrome[J]. Clin EEG Neurosci, 2017, 48(4): 280-287., articleTitle=Prognostic utility of clinical epilepsy severity score versus pretreatment hypsarrhythmia scoring in children with west syndrome, refAbstract=null), Reference(id=1198558183028519287, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, doi=null, pmid=null, pmcid=null, year=2019, volume=184, issue=null, pageStart=105402, pageEnd=null, url=null, language=null, rfNumber=[38], rfOrder=37, authorNames=Zhang Y, Yu L, Zhou Y, journalName=Clin Neurol Neurosurg, refType=null, unstructuredReference=Zhang Y, Yu L, Zhou Y, et al. Prognostic utility of hypsarrhythmia scoring in children with West syndrome after ketogenic diet[J]. Clin Neurol Neurosurg, 2019, 184: 105402., articleTitle=Prognostic utility of hypsarrhythmia scoring in children with West syndrome after ketogenic diet, refAbstract=null)], funds=[Fund(id=1198558178410590451, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, awardId=2019018, language=EN, fundingSource=Medical Science Research Project of Chengdu(2019018), fundOrder=null, country=null), Fund(id=1198558178637082871, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, awardId=2019018, language=CN, fundingSource=成都市医学科研课题(2019018), fundOrder=null, country=null)], companyList=[AuthorCompany(id=1198558172257546243, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, xref=null, ext=[AuthorCompanyExt(id=1198558172265934852, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, companyId=1198558172257546243, language=EN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=Pediatric Neurology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan 611731, China), AuthorCompanyExt(id=1198558172274323462, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, companyId=1198558172257546243, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=电子科技大学医学院附属妇女儿童医院.成都市妇女儿童中心医院神经内科，四川成都 611731)])], figs=[ArticleFig(id=1198558176586068177, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=EN, label=Fig.1, caption=ROC curve of the Kramer scores after ACTH treatment for 14 days in IESS, figureFileSmall=KVlbRhYqC0agMYjf4ns+ng==, figureFileBig=6yUXAYcy0iYp0lAHjVvqVg==, tableContent=null), ArticleFig(id=1198558176674148567, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=CN, label=图1, caption=ACTH治疗IESS 14 d后 Kramer评分预测ACTH疗效的ROC曲线

IESS. 婴儿癫痫性痉挛综合征；ASMs. 抗癫痫发作药物；ACTH. 促肾上腺皮质激素；ROC. 受试者工作特征

, figureFileSmall=KVlbRhYqC0agMYjf4ns+ng==, figureFileBig=6yUXAYcy0iYp0lAHjVvqVg==, tableContent=null), ArticleFig(id=1198558176900640992, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=EN, label=Tab.1, caption=

Comparison of clinical data between effective group and ineffective group of pediatric IESS patients

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特征 有效组(n=39) 无效组(n=41) χ2/Z P
性别[例(%)] 1.204 0.273
男 22(56.4) 28(68.3)
女 17(43.6) 13(31.7)
年龄[例(%)] 0.904 0.342
3~12月龄 32(82.1) 30(73.2)
其他年龄 7(17.9) 11(26.8)
围产期异常[例(%)] 4.984 0.026
无 31(79.5) 23(56.1)
有 8(20.5) 18(43.9)
痉挛前发育异常[例(%)] 0.803 0.370
否 12(30.8) 9(22.0)
有 27(69.2) 32(78.0)
病因[例(%)] 7.093 0.029
病因不明发育正常 5(12.8) 5(12.2)
结构性异常 23(59.0) 13(31.7)
病因不明发育迟滞 11(28.2) 23(56.1)a
头颅MRI异常[例(%)] 4.698 0.095
无 12(30.8) 22(53.6)
非特异性异常 8(20.5) 4(9.8)
特异性异常 19(48.7) 15(36.6)
发作形式[例(%)] 1.154 0.283
痉挛发作 34(87.2) 32(78.0)
合并其他形式 5(12.8) 9(22.0)
治疗前已用ASMs[例(%)] 4.411 0.036
否 28(78.1) 20(48.8)
是 11(28.2) 21(51.2)
治疗前Kramer评分[分, M(Q1, Q3)] 10.0(8.0, 11.0) 10.0(8.5, 12.0) 0.628 0.530
发病至治疗间隔时间[例(%)] 1.204 0.273
≤30 d 19(48.7) 15(36.6)
>30 d 20(51.3) 26(63.4)
ACTH给药剂量[例(%)] 10.196 0.001
1~2 U/(kg.d) 21(53.8) 8(19.5)
>2 U/(kg.d) 18(46.2) 33(80.5)
ACTH疗程[例(%)] 2.209 0.137
2周 6(15.4) 12(29.3)
4周 33(84.6) 29(70.7)
联合ASMs种类[例(%)] 4.003 0.045
1种 22(56.4) 14(34.1)
≥2种 17(43.6) 27(65.9)
治疗14 d发作情况[例(%)] 20.799 <0.001
临床无发作 22(56.4) 5(12.2)bc
发作减少≥50% 11(28.2) 13(31.7)
发作减少<50% 6(15.4) 23(56.1)
治疗14 d的Kramer评分[分, M(Q1, Q3)] 4.0(3.0, 5.0) 7.0(6.5, 9.0) 6.669 <0.001
), ArticleFig(id=1198558177009692901, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=CN, label=表1, caption=

两组IESS患儿临床资料比较

, figureFileSmall=null, figureFileBig=null, tableContent=
特征 有效组(n=39) 无效组(n=41) χ2/Z P
性别[例(%)] 1.204 0.273
男 22(56.4) 28(68.3)
女 17(43.6) 13(31.7)
年龄[例(%)] 0.904 0.342
3~12月龄 32(82.1) 30(73.2)
其他年龄 7(17.9) 11(26.8)
围产期异常[例(%)] 4.984 0.026
无 31(79.5) 23(56.1)
有 8(20.5) 18(43.9)
痉挛前发育异常[例(%)] 0.803 0.370
否 12(30.8) 9(22.0)
有 27(69.2) 32(78.0)
病因[例(%)] 7.093 0.029
病因不明发育正常 5(12.8) 5(12.2)
结构性异常 23(59.0) 13(31.7)
病因不明发育迟滞 11(28.2) 23(56.1)a
头颅MRI异常[例(%)] 4.698 0.095
无 12(30.8) 22(53.6)
非特异性异常 8(20.5) 4(9.8)
特异性异常 19(48.7) 15(36.6)
发作形式[例(%)] 1.154 0.283
痉挛发作 34(87.2) 32(78.0)
合并其他形式 5(12.8) 9(22.0)
治疗前已用ASMs[例(%)] 4.411 0.036
否 28(78.1) 20(48.8)
是 11(28.2) 21(51.2)
治疗前Kramer评分[分, M(Q1, Q3)] 10.0(8.0, 11.0) 10.0(8.5, 12.0) 0.628 0.530
发病至治疗间隔时间[例(%)] 1.204 0.273
≤30 d 19(48.7) 15(36.6)
>30 d 20(51.3) 26(63.4)
ACTH给药剂量[例(%)] 10.196 0.001
1~2 U/(kg.d) 21(53.8) 8(19.5)
>2 U/(kg.d) 18(46.2) 33(80.5)
ACTH疗程[例(%)] 2.209 0.137
2周 6(15.4) 12(29.3)
4周 33(84.6) 29(70.7)
联合ASMs种类[例(%)] 4.003 0.045
1种 22(56.4) 14(34.1)
≥2种 17(43.6) 27(65.9)
治疗14 d发作情况[例(%)] 20.799 <0.001
临床无发作 22(56.4) 5(12.2)bc
发作减少≥50% 11(28.2) 13(31.7)
发作减少<50% 6(15.4) 23(56.1)
治疗14 d的Kramer评分[分, M(Q1, Q3)] 4.0(3.0, 5.0) 7.0(6.5, 9.0) 6.669 <0.001
), ArticleFig(id=1198558178133766374, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=EN, label=Tab.2, caption=

Influence factors and predictors on treatment response after ACTH treatment in IESS

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特征 β 标准误 RR(95%Cl) Wald χ2 P
围产期异常 -0.359 0.196 0.698(0.475~1.026) 3.340 0.068
有
无*
病因
病因不明发育迟滞 0.104 0.280 1.110(0.641~1.921) 0.138 0.710
获得结构性 0.254 0.226 1.290(0.828~2.009) 1.264 0.261
病因不明发育正常*
ACTH治疗前已用ASMs -0.605 0.216 0.546(0.357~0.833) 7.861 0.005
是
否*
ACTH给药剂量 0.331 0.224 1.393(0.897~2.162) 2.182 0.140
联合ASMs种类 0.297 0.212 1.345(0.888~2.039) 1.953 0.162
治疗14 d疗效 0.225 0.178 1.253(0.885~1.774) 1.611 0.204
治疗14 d的Kramer评分 -0.355 0.063 0.701(0.620~0.792) 32.511 <0.001
), ArticleFig(id=1198558178242818284, tenantId=1146029695717560320, journalId=1189873630562394117, articleId=1198558168910492535, language=CN, label=表2, caption=

IESS患儿ACTH疗效的影响因素及预测指标

, figureFileSmall=null, figureFileBig=null, tableContent=
特征 β 标准误 RR(95%Cl) Wald χ2 P
围产期异常 -0.359 0.196 0.698(0.475~1.026) 3.340 0.068
有
无*
病因
病因不明发育迟滞 0.104 0.280 1.110(0.641~1.921) 0.138 0.710
获得结构性 0.254 0.226 1.290(0.828~2.009) 1.264 0.261
病因不明发育正常*
ACTH治疗前已用ASMs -0.605 0.216 0.546(0.357~0.833) 7.861 0.005
是
否*
ACTH给药剂量 0.331 0.224 1.393(0.897~2.162) 2.182 0.140
联合ASMs种类 0.297 0.212 1.345(0.888~2.039) 1.953 0.162
治疗14 d疗效 0.225 0.178 1.253(0.885~1.774) 1.611 0.204
治疗14 d的Kramer评分 -0.355 0.063 0.701(0.620~0.792) 32.511 <0.001
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促肾上腺皮质激素治疗婴儿癫痫性痉挛综合征疗效的影响因素及其预测指标
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毛丹丹 , 刘平 * , 胡文广 , 李思秀 , 蓝明平 , 杨帆
解放军医学杂志 | 临床研究 2024,49(8): 868-875
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解放军医学杂志 | 临床研究 2024, 49(8): 868-875
促肾上腺皮质激素治疗婴儿癫痫性痉挛综合征疗效的影响因素及其预测指标
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毛丹丹, 刘平* , 胡文广, 李思秀, 蓝明平, 杨帆
作者信息
  • 电子科技大学医学院附属妇女儿童医院.成都市妇女儿童中心医院神经内科，四川成都 611731

    • 毛丹丹，医学硕士，主治医师，主要从事儿童神经病学方面的研究

通讯作者:

刘平，E-mail：
Influence factors and predictors on infantile epileptic spasms syndrome treatment response after adrenocorticotropic hormone
Dan-Dan Mao, Ping Liu* , Wen-Guang Hu, Si-Xiu Li, Ming-Ping Lan, Fan Yang
Affiliations
  • Pediatric Neurology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan 611731, China
出版时间: 2024-08-28 doi: 10.11855/j.issn.0577-7402.0212.2024.0229
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摘要
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目的 探讨促肾上腺皮质激素(ACTH)治疗婴儿癫痫性痉挛综合征(IESS)疗效的影响因素及其预测指标。方法 回顾性分析成都市妇女儿童中心医院2016年1月-2020年12月收治并给予ACTH治疗的80例IESS患儿(男50例，女30例)的临床资料。根据ACTH治疗28 d疗效分为有效组(n=39)与无效组(n=41)，收集并比较两组的临床资料，包括患儿的基本信息、病因、治疗方案及治疗前后脑电图高度失律严重程度评分(即Kramer评分)等指标。构建修正Poisson回归模型以发现疗效预测因素，采用受试者工作特征(ROC)曲线评估阳性指标的预测价值。结果 患儿起病年龄为1个月7 d~1岁9个月。发作形式包括单纯痉挛发作66例，合并其他发作如局灶发作、继发全面性发作14例。32例ACTH治疗前已给予抗癫痫发作药物(ASMs)治疗。治疗前、治疗后14 d的Kramer评分分别为10.0(8.3，12.0)分和6.0(4.0，7.0)分。经ACTH治疗后39例(48.8%)有效。与有效组比较，无效组中围产期存在异常、病因不明发育迟滞、ACTH治疗前已使用ASMs、ACTH给药剂量>2 U/(kg.d)、联合2种以上ASMs、治疗14 d时仍有发作的构成比高，且ACTH治疗14 d Kramer评分高，差异均具有统计学意义(P<0.05)。修正Poisson回归模型结果显示，两组ACTH治疗前是否使用ASMs(RR=0.546，95%CI 0.357~0.833，P=0.005)、治疗14 d后的Kramer评分(RR=0.701，95%CI 0.620~0.792，P<0.001)差异有统计学意义，而两组性别、年龄、围产期情况、病因、发作形式、治疗前Kramer评分、发病至治疗间隔时间、ACTH疗程、联合ASMs种类方面差异均无统计学意义(P>0.05)。ROC曲线分析显示，治疗14 d的Kramer评分对疗效有预测价值[曲线下面积(AUC)=0.930，95%CI 0.873~0.987，P<0.001]，敏感度为92.7%，特异度为84.6%，约登指数为0.773，即治疗14 d Kramer评分越高，疗效越差，以6分为截点值，每升高1分，疗效降低接近30.0%。结论 治疗前已使用ASMs可能会影响ACTH的疗效，治疗14 d后的Kramer评分≥6分或可作为ACTH治疗IESS疗效不佳的独立预测指标。

婴儿癫痫性痉挛综合征  /  痉挛发作  /  促肾上腺皮质激素  /  脑电图  /  治疗

Objective To explore the influence factors and predictors of treatment response after adrenocorticotropic hormone (ACTH) in infantile epileptic spasms syndrome(IESS). Methods A retrospective analysis was conducted on 80 cases of IESS infants(50 males and 30 females) who were diagnosed and treated with ACTH in Chengdu Women's and Children's Central Hospital from January 2016 to December 2020. Patients were divided into effective group (n=39) and ineffective group (n=41) based on their response of ACTH treatment after 28 days, and their clinical data including the patients' basic information, etiology, treatment programmer, per- and post-treatment Kramer scores of electroencephalogram (EEG) hypsarrhythmia severity and so on, were collected to compare and analyze between the two groups. A modified Poisson regression model was constructed to discover predictors of outcome, and the receiver operating characteristic (ROC) curves were used to assess the prognosis evaluation of the positive predictive value. Results The ages at seizure onset ranged from one month and seven days to one year and nine months. Seizure types included simple epileptic spasms in 66 cases and combined with other types(focal and secondarily generalized seizures) in 14 cases. Thirty-two cases had been given anti-seizure medications (ASMs) before ACTH treatment. The median Kramer scores per-treatment and at 14 days post-treatment were 10.0 (8.3, 12.0) and 6.0 (4.0, 7.0), respectively. After ACTH treatment, 39(48.8%) cases were effective. Compared with the effective group, the ineffective group had significantly higher proportion of abnormal perinatal conditions, unknown aetiology with normal development, ASMs given before ACTH treatment, the dosages of ACTH greater than 2 U/(kg·d), combinations of two or more ASMs, poor control, and still seizure attack after ACTH treatment of 14 days (P<0.05). Additionally, the Kramer scores after ACTH treatment of 14 days in the ineffective group were also significantly higher (P<0.05). The modified Poisson regression model showed that there were significant statistic differences between the two groups on ASMs given before ACTH treatment (RR=0.546, 95%CI 0.357-0.833, P=0.005) and Kramer scores of hypsarrhythmia severity (RR=0.701, 95%CI 0.620-0.792, P<0.001),while there were no significant differences between the two groups in term of ages, gender, perinatal conditions, etiologies, seizure types, Kramer scores before treatment, time lag between onset and treatment, duration of ACTH treatment, kinds of ASMs combination. ROC curve analysis showed that only Kramer scores at 14 days after ACTH treatment could predict the treatment response with sensitivity and specificity of 92.7% and 84.6%, respectively, with Youden index of 0.773. The area under the ROC curve was 0.930 (95%CI 0.873-0.987, P<0.001) and the cut-point of the score was 6, indicating that the higher the Kramer scores at 14 days after ACTH treatment, the worse the treatment response. The treatment response rate would reduce by about 30.0% if the Kramer score increased by one point. Conclusion ASMs given before ACTH treatment may influence the treatment response. Kramer scores greater than 6 at day 14 after ACTH treatment may be used as a predictor of treatment response after ACTH in IESS patients.

infantile epileptic spasms syndrome  /  epileptic spasms  /  adrenocorticotropic hormone  /  electroencephalogram  /  treatment
毛丹丹, 刘平, 胡文广, 李思秀, 蓝明平, 杨帆. 促肾上腺皮质激素治疗婴儿癫痫性痉挛综合征疗效的影响因素及其预测指标. 解放军医学杂志, 2024 , 49 (8) : 868 -875 . DOI: 10.11855/j.issn.0577-7402.0212.2024.0229
Dan-Dan Mao, Ping Liu, Wen-Guang Hu, Si-Xiu Li, Ming-Ping Lan, Fan Yang. Influence factors and predictors on infantile epileptic spasms syndrome treatment response after adrenocorticotropic hormone[J]. Medical Journal of Chinese People’s Liberation Army, 2024 , 49 (8) : 868 -875 . DOI: 10.11855/j.issn.0577-7402.0212.2024.0229
正文
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婴儿癫痫性痉挛综合征(infantile epileptic spasms syndrome，IESS)又称为婴儿痉挛症(infantile spams，IS)，是一种罕见的具有年龄特异性的癫痫性脑病，发病率为(2~5)/10 000例活产儿，3~7月龄高发，男略多于女，以成串癫痫性痉挛发作、高度失律或多灶/局灶异常放电脑电图模式、智力运动发育落后或倒退为主要特征，治疗难度大，预后较差[1-4]。50%~70%的患儿后续会经历多种类型的癫痫发作，其中70%生活质量下降，90%左右遗留不同程度的智力障碍，给家庭和社会带来沉重负担[5-8]。美国神经病学会、德国儿科神经学会以及国际抗癫痫联盟(International League Against Epilepsy，ILAE)均推荐促肾上腺皮质激素(adrenocorticotropic hormone，ACTH)作为IESS的一线治疗药物，但其优选剂量及疗程均未统一[9-11]。Messer等[4]指出，一线治疗2~3周评估疗效，以便及时调整方案。一项来自美国婴儿痉挛协会的前瞻性队列研究显示，潜在病因、痉挛前存在中重度发育迟滞、初始标准方案的选择可以作为惊厥预后相关因素[12]，但目前仍缺乏可靠的生物标志物预测疗效。本研究回顾性分析给予ACTH治疗的IESS患儿的临床资料，探寻疗效的影响因素及相关预测指标，旨在为临床早期合理调整治疗方案提供依据。
1 资料与方法
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1.1 研究对象
选择成都市妇女儿童中心医院2016年1月-2020年12月神经内科接诊并给予ACTH治疗的80例IESS患儿进行回顾性分析。纳入标准：(1)符合2022年ILAE关于IESS诊断标准[1]，表现为癫痫性痉挛发作，发作间期脑电图为高度失律模式，可伴发育迟滞；(2)接受ACTH治疗且耐受，疗程≥14 d；(3)随访时间≥3个月。排除标准：(1)入院后因感染或应用维生素B6注射液后痉挛缓解；(2)合并严重肝肾功能不全；(3)失访或临床资料不完善。所有患儿根据ACTH治疗28 d的疗效分为有效组(n=39)与无效组(n=41)。本研究已获成都市妇女儿童中心医院医学伦理委员会审批[科研伦审2022(98)号]。
1.2 ACTH方案
用药前充分告知药物可能的获益及不良反应，监护人均签署知情同意书。用药前均完善血常规、肝肾功能、电解质、乙肝病毒+艾滋病毒+梅毒病原学、EB病毒+TORCH(包括单纯疱疹病毒Ⅰ型及Ⅱ型、风疹病毒、弓形虫、巨细胞病毒)抗体、心电图、结核菌素试验、胸片、腹部B超、头颅核磁共振成像(MRI)、视频脑电图(VEEG)检查，除外感染后开始用药。所有患儿入院后均给予维生素B6注射液10~20 mg/(kg.d)静脉滴注3 d，无效者参照中南大学湘雅医院彭镜教授团队方案[13]，给予ACTH 1~2 U/(kg.d)，加入5%葡萄糖中静脉泵入6 h，1次/d，连用14 d，复查VEEG，若临床发作未缓解或脑电图无改善，ACTH维持原剂量或增倍(总量≤40 U/d)再使用2周。结局判定[14]：ACTH治疗开始14 d内，并持续28 d无肉眼可见的痉挛发作(距末次痉挛开始计算)，VEEG监测4~6 h亦未见发作定义为有效。ACTH停用后给予泼尼松1.5~2.0 mg/(kg.d)晨起顿服，3个月内减停。
1.3 脑电图严重程度评分(即Kramer评分)[15-17]
采用国际10-20电极放置系统记录脑电图，分别于ACTH治疗前和治疗14 d进行VEEG监测4~6 h，包括清醒期及睡眠期。由两名具有脑电图培训中级资格的神经内科医师进行独立评分，若意见不统一，与另一名具有10年脑电图工作经验的神经内科医师进行讨论。选取最具代表性的10 s片段来评价高度失律的严重程度，项目包括背景紊乱情况、弥漫性慢波、波幅、棘尖波频率，每一项得分0~3分，另需纳入电压衰减、睡眠期暴发抑制、缺乏正常睡眠、相对正常化(存在记为1分，无记为0分)。
1.4 资料收集及指标分析
收集所有患儿的临床资料，包括起病年龄、性别、围产期是否异常、发作类型、病因、ACTH治疗前是否已用抗癫痫发作药物(ASMs)、起病至开始治疗间隔时间、ACTH治疗方案、治疗前及治疗14 d的Kramer评分等指标，并比较ACTH治疗有效组与无效组间各指标的差异；探讨疗效预测因素并评估阳性指标的预测价值。
1.5 统计学处理
采用SPSS 25.0软件进行统计分析，计量资料进行正态性检验，正态分布者以$\bar{x}±s$表示，两组间比较采用独立样本t检验；非正态分布者以M(Q1，Q3)表示，两组间比较采用秩和检验。计数资料以例(%)表示，两组间比较采用χ2检验，双侧P<0.05为差异有统计学意义；三组两两比较时采用人工α分割法，此时双侧P<0.017为差异有统计学意义。为控制混杂因素，对单变量分析中有意义者纳入构建修正Poisson回归模型以发现预测指标；采用ROC曲线和曲线下面积(AUC)评估阳性指标预测疗效的准确性，采用约登指数确定截点值。
2 结果
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2.1 一般特征
105例患儿满足IESS诊断，15例拒绝接受ACTH治疗，3例因感染或应用维生素B6注射液后痉挛缓解，2例ACTH疗程不足，5例失访，最终纳入80例IESS患儿进行研究，其中男50例，女30例，男女比例1.7∶1。起病年龄1个月7 d~1岁9个月，其中3~12月龄62例(占77.5%)。发病至治疗间隔时间为1 d~1年2个月。59例病前发育迟缓(占73.8%)，围产期异常26例(占32.5%)，包括围产期缺氧窒息、颅内出血、感染、低血糖、早产。发作形式包括单纯痉挛发作66例，合并其他发作如局灶发作、继发全面性发作14例。
2.2 病因
所有患儿均完善头颅MRI检查，其中34例正常、12例非特异性异常(如脑外间隙增宽、脑室扩张、脑白质髓鞘化延迟、胼胝体薄、蛛网膜囊肿等)、34例特异性异常[包括13例发育畸形(灰质异位、巨脑回畸形、神经元移行障碍、脑裂畸形、皮质发育不良等)、13例脑软化萎缩、7例结节性硬化、1例脑积水]。34例完善基因检查，其中12例阳性，包括TSC1/2突变6例，SCN8A突变3例，SCN2A、ITPA、TUBAIA突变各1例；30例完善血尿代谢筛查，其中1例阳性，为短链脂肪酸代谢障碍(但由于经济原因，该患儿监护人未同意完善基因检查)。根据头颅MRI、发育情况，将病因分为3类[18]：(1)病因不明但发育正常10例；(2)结构性异常36例，包括任何获得性脑损伤、脑发育畸形和其他结构性异常；(3)病因不明发育迟滞34例。
2.3 治疗及Kramer评分
80例IESS患儿经ACTH治疗后39例有效，有效率48.8%。ACTH治疗前有32例已经给予ASMs治疗，占40.0%。80例IESS患儿在给予ACTH治疗过程中，29例给药剂量始终为1~2 U/(kg.d)，其中11例用药14 d后发作控制，但脑电图未改善，延长使用至28 d；51例加量或起始剂量为2~4 U/(kg.d)。18例治疗疗程为2周，62例疗程为4周。36例联合一种ASMs，44例联合两种及以上ASMs治疗。治疗前Kramer评分为10.0(8.3，12.0)分，治疗14 d后的Kramer评分为6.0(4.0，7.0)分。
2.4 两组临床资料比较
与有效组比较，无效组中围产期存在异常、病因不明发育迟滞、ACTH治疗前已使用ASMs、ACTH给药剂量>2 U/(kg.d)、联合2种以上ASMs、治疗14 d时仍有发作的构成比高，且ACTH治疗14 d的Kramer评分高，差异均有统计学意义(P<0.05)；而两组间性别、年龄、头颅MRI、发作形式、痉挛前发育情况、治疗前Kramer评分、发病至治疗间隔时间、ACTH疗程差异无统计学意义(P>0.05)(表1)。
2.5 ACTH疗效的影响因素及预测指标
修正Poisson回归模型显示，IESS患儿近期疗效与ACTH治疗前已用ASMs、治疗14 d的Kramer评分相关(P<0.05)，而与是否存在围产期异常、病因、ACTH给药剂量、联合ASMs种类、治疗14 d发作情况无关(P>0.05)(表2)。
2.6 阳性指标的预测价值
将上述阳性指标(ACTH治疗前已用ASMs、治疗14 d的Kramer评分)纳入ROC曲线分析，结果显示，仅治疗14 d的Kramer评分≥6分对疗效有预测价值(AUC=0.930，95%CI 0.873~0.987，P<0.001)，敏感度92.7%，特异度84.6%，约登指数为0.773(图1)，提示已使用ASMs或可影响ACTH近期疗效，但并不能作为疗效的预测指标，而治疗14 d后Kramer评分越高，疗效越差，以6分为截断值，每升高1分，疗效降低接近30.0%，差异有统计学意义(RR=0.701，95%CI 0.620~0.792，P<0.001)。
3 讨论
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IESS是婴幼儿时期严重的癫痫性脑病，对常规ASMs治疗反应差。自1958年ACTH首次治疗该病被报道以来，至今已有60多年的历史[19]。37%~87%的IESS患儿经ACTH治疗后可达到临床无发作、高度失律缓解状态[8,12,19-22]。本研究中IESS患儿经ACTH治疗后的短期应答率为48.8%，与Grinspan等[21]及Alrifai等[22]研究结果相近。
既往研究认为，IESS的风险随着产前应激程度加重而增高，即母体压力影响下丘脑-垂体-肾上腺轴(HPA)的功能，导致母体分泌糖皮质激素和儿茶酚胺增多，胎盘血流减少，胎儿营养缺乏，胎儿HPA轴受到影响，致使促肾上腺素糖皮质激素释放激素(CRH)过量产生，诱发痉挛发作[23]。此外，产前应激、脑发育不良、遗传代谢性疾病等因素可能导致婴儿大脑皮层和基底节N-甲基-D-天门冬氨酸受体(NMDAR)过度表达，触发痉挛发作[24]。动物模型研究发现，急性应激如游泳、寒冷等因素可激活HPA轴，提高神经类固醇四氢脱氧皮质酮(THDOC)的水平，增加海马齿状回中γ-氨基丁酸(GABA)-A受体δ亚单位的表达，降低神经元兴奋性，最终使惊厥阈值提高；而慢性应激被认为是一种促惊厥剂，可导致HPA功能障碍，增加惊厥易感性，而癫痫发作又可反过来加重HPA功能障碍[24]。ACTH主要通过调节HPA功能来作用于黑皮质素受体，通过促进神经类固醇生成、减少炎性因子产生、抑制神经系统炎症反应、减轻兴奋性神经毒性损伤等途径发挥神经保护和抗痉挛作用[25]。Sumanasena等[26]发现，IESS患儿发育障碍与围产期高危因素(如新生儿缺氧缺血性脑病史、早产并发症和新生儿感染)明显相关。本组病例中，近1/3的患儿存在围产期异常，而这部分患儿对ACTH的治疗反应较差，推测围产期不良事件或可影响ACTH治疗IESS的效果，但并不能预测疗效。
随着基因技术、影像学及其他工具的广泛应用，IESS患儿病因构成比随时间而发生变化，关于病因与IESS预后关系的研究颇多[12,19,27-31]。Riikonen[27]对既往前瞻性研究进行综合分析发现，影响IESS预后的主要因素包括潜在病因、痉挛发生时是否存在发育异常，对于既往有新生儿低血糖、卒中或脑梗死、脑室周围白质软化、唐氏综合征、神经纤维瘤的IESS患儿，惊厥更易控制，而具有严重脑发育畸形、感染性病因、结节性硬化症的患儿惊厥控制更差，而由基因突变如SCN2A突变所致的IESS更容易出现发育障碍。Dang等[31]发现，对于遗传性、结构性和隐源性病因的IESS患儿，ACTH有效率分别为27.8%、65.2%和72.2%，提示ACTH对隐源性和结构性病因的IESS患儿效果较好。日本学者Daida等[30]回顾性分析107例IESS患儿的临床资料发现，在病因不明发育正常、获得结构性和先天性病因中，ACTH有效率分别为64.0%、65.0%和30.6%，多因素分析发现，病因不明发育正常(OR=4.63，95%CI 1.60~3.30，P<0.01)和获得结构性病因(OR=3.41，95%CI 1.01~11.50，P=0.047)的疗效较先天性病因好。Knupp等[12]对230例IESS患儿进行前瞻性队列研究，结果显示病因与疗效并无明确关系。Chourasia等[29]发现，具有获得性和非获得性病因的IESS患儿在治疗应答方面并无统计学差异。一项网络荟萃分析发现，在调整治疗间隔后，症状性病因的IESS患儿电临床缓解(即痉挛停止和高度失律消失)率更低[32]。Bitton等[28]对IESS患儿进行长达5年的随访发现，经过标准治疗后，病因不明者认知功能多在2~5年逐渐改善，而症状性病因则预示着较差的认知结局。本组病例中，有效组中结构性病因23例(58.9%)，无效组中病因不明发育迟滞者23例(56.1%)，构成比差异有统计学意义，与Riikonen[27]、Dang等[31]及Daida等[30]的研究存在相似之处，但并不能作为疗效的预测指标，造成这种差异的原因可能与各研究病因分组标准不一致有关。
既往多项研究显示，积极采用标准一线方案是IESS治疗反应性的决定性因素[7,12,21,33-34]。2016年一项来自美国IESS协会的前瞻性队列研究显示，以ACTH作为初始治疗的IESS患儿对治疗的反应率为54.6%，而以其他ASMs作为初始治疗的IESS患儿仅9.4%对治疗有反应，差异有统计学意义[12]。Demarest等[33]对447例IESS患儿进行观察性研究显示，与非标准疗法相比，ACTH作为首选初始治疗的反应率高，且差异有统计学意义(OR=10.247，95%CI 4.078~25.751，P<0.01)。一项来自美国的前瞻性、多中心观察性研究对比给予不同初始治疗方案后IESS的缓解率，结果显示ACTH的首选初始治疗反应率(46.3%)明显高于非标准治疗组(7.8%)，从非标准治疗调整为ACTH治疗后，反应率提高到39.0%[21]。钟金花等[34]回顾性分析IESS中ACTH作为首选和非首选治疗的疗效，结果显示首选组有效率明显高于非首选组(73.7% vs. 45.2%，χ2=3.889，P<0.05)。
Mao等[7]对312例IESS患儿的治疗方案进行回顾性分析发现，53.4%的患儿接受ACTH作为一线治疗后可达到痉挛无发作状态，而非标准治疗组仅32.6%，差异有统计学意义(HR=0.22，95%CI 0.07~0.70，P=0.011)。本研究显示，以ACTH作为初始治疗方案的患儿有效率(58.3%)明显高于治疗前已用ASMs的患儿有效率(34.4%)，与上述研究结论一致。
文献报道，发病至ACTH间隔时间或可影响IESS的预后[1,4,35-36]。而本研究显示，40.0%的患儿ACTH治疗前已接受其他ASMs治疗，但并未发现疗效与发病至治疗间隔时间相关，推测可能与样本量少、患儿监护人对激素治疗接受程度不高有关，临床实践过程中临床医师应加强对该病相关治疗方案及药物耐受性等方面的宣教。
及时终止痉挛发作和脑电图高度失律模式、改善预后是IESS治疗的目标[4]。本研究显示治疗14 d仍有发作的患儿有效率更低(28.2% vs. 31.7%)，因此，对于这部分患儿尤其需积极评估。然而，婴儿脑电图表现形式复杂多样，评价者主要通过主观判断来解读，一定程度上限制了VEEG在IESS患儿中的精准应用[16]，将脑电图数据量化或可提高诊断准确性，并为及时调整治疗、评估预后提供依据[16]。Kramer等[15]回顾性分析53例IESS患儿ACTH治疗前的脑电图表现，发现高度失律评分<10分的患儿预后良好。Sehgal等[37]将变异型高度失律脑电图纳入分析，结果显示治疗前Kramer评分≤8分的患儿预后更好。2019年，复旦大学Zhang等[38]回顾性分析39例接受生酮饮食(KD)治疗的IESS患儿的临床资料，发现KD治疗3个月时的Kramer评分≥8分提示癫痫预后不良，敏感度100.0%，特异度87.5%，而与治疗前的评分无关。本研究VEEG包含变异型和不典型高度失律，结果显示有效组与无效组治疗前Kramer评分差异并无统计学意义，而治疗14 d Kramer评分与近期疗效相关，每升高1分，疗效降低近30.0%，敏感度92.7%，特异度84.6%。
综上所述，对于接受ACTH治疗的IESS患儿，可在治疗2周左右进行评估，若Kramer评分≥6分，警惕疗效不佳，综合评估后及时调整治疗方案或可改善预后。治疗前已使用ASMs的IESS患儿ACTH疗效可能不佳。因此，对于新诊断的IESS患儿，临床医师应加强宣教，提高患儿监护人对该病的认识，在综合评估及条件允许情况下，ACTH或可作为优选初始治疗选择。治疗2周左右需评估临床疗效及脑电图情况，Kramer评分≥6分或可作为ACTH治疗IESS疗效不佳的独立预测指标。然而，本研究为回顾性队列研究，尚存在不足之处，如样本量小、癫痫基因完成率低、病因分组未细化、围产期不良因素未细分等，但本研究所发现的特点，特别是VEEG高度失律严重程度评分与预后的关系值得重视，未来可进一步开展前瞻性研究验证其预测价值，为合理有效的调整治疗方案提供科学依据。
基金
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  • 成都市医学科研课题(2019018)
文献
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参考文献 引证文献
排序方式：
[1]
Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: position statement by the ILAE Task Force on Nosology and Definitions[J]. Epilepsia, 2022, 63(6): 1349-1397.
[2]
D'Alonzo R, Rigante D, Mencaroni E, et al. West syndrome: a review and guide for paediatricians[J]. Clin Drug Investig, 2018, 38(2): 113-124.
[3]
Krag A, Holmes GL. Diagnosing infantile spasms: accuracy of the internet[J]. Epilepsy Behav, 2016, 64(Pt A): 239-241.
[4]
Messer R, Knupp KG. Infantile spasms: opportunities to improve care[J]. Semin Neurol, 2020, 40(2): 236-245.
[5]
Howell KB, Freeman JL, MacKay MT, et al. The severe epilepsy syndromes of infancy: a population-based study[J]. Epilepsia, 2021, 62(2): 358-370.
[6]
Weingarten MM, Cokley JA, Moffett B, et al. Trends and costs associated with the diagnosis and treatment of infantile spasms: a 10-year multicenter retrospective review[J]. J Pediatr Pharmacol Ther, 2022, 27(1): 29-37.
[7]
Mao L, Kessi M, Peng P, et al. The patterns of response of 11 regimens for infantile spasms[J]. Sci Rep, 2020, 10(1): 11509.
[8]
Bhanudeep S, Madaan P, Sankhyan N, et al. Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome[J]. Epilepsy Res, 2021, 173: 106629.
[9]
Go CY, MacKay MT, Weiss SK, et al. Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society[J]. Neurology, 2012, 78(24): 1974-1980.
[10]
Tibussek D, Klepper J, Korinthenberg R, et al. Treatment of infantile spasms: report of the interdisciplinary guideline committee coordinated by the german-speaking society for neuropediatrics[J]. Neuropediatrics, 2016, 47(3): 139-150.
[11]
Wilmshurst JM, Gaillard WD, Vinayan KP, et al. Summary of recommendations for the management of infantile seizures: task Force Report for the ILAE Commission of Pediatrics[J]. Epilepsia, 2015, 56(8): 1185-1197.
[12]
Knupp KG, Coryell J, Nickels KC, et al. Response to treatment in a prospective national infantile spasms cohort[J]. Ann Neurol, 2016, 79(3): 475-484.
[13]
Yin J, Lu Q, Yin F, et al. Effectiveness and safety of different once-daily doses of adrenocorticotropic hormone for infantile spasms[J]. Paediatr Drugs, 2017, 19(4): 357-365.
[14]
Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi Group[J]. Epilepsia, 2004, 45(11): 1416-1428.
[15]
Kramer U, Sue WC, Mikati MA. Hypsarrhythmia: frequency of variant patterns and correlation with etiology and outcome[J]. Neurology, 1997, 48(1): 197-203.
[16]
孙于林, 陈健, 杨光. 脑电图评分在婴儿痉挛症的应用进展[J]. 中华实用儿科临床杂志, 2021, 36(10): 794-797.
[17]
Lachhwani D. Scoring systems for the evaluation of hypsarrhythmia[J]. J Clin Neurophysiol, 2022, 39(7): 538-543.
[18]
Mytinger JR, Albert DVF, Twanow JD, et al. Compliance with standard therapies and remission rates after implementation of an infantile spasms management guideline[J]. Pediatr Neurol, 2020, 104: 23-29.
[19]
Pestana Knight EM, Mani J. Historical overview of hypsarrhythmia and its association to epileptic spasms: a review of the medical literature from 1952 to 1982[J]. J Clin Neurophysiol, 2022, 39(7): 521-528.
[20]
Guang S, Mao L, Zhong L, et al. Hormonal therapy for infantile spasms: a systematic review and meta-analysis[J]. Front Neurol, 2022, 13: 772333.
[21]
Grinspan ZM, Knupp KG, Patel AD, et al. Comparative effectiveness of initial treatment for infantile spasms in a contemporary US cohort[J]. Neurology, 2021, 97(12): e1217-e1228.
[22]
Alrifai MT, Al-Rumayyan AR, Al-Tuwaijri WA, et al. The response patterns of infantile spasms to treatments in 156 patients: Hormonal therapy with intravenous synthetic ACTH appears promising[J]. Neurosciences (Riyadh), 2022, 27(1): 40-44.
[23]
Shi XY, Zou LP, Yang G, et al. Prenatal stress exposure hypothesis for infantile spasms[J]. Med Hypotheses, 2012, 78(6): 735-737.
[24]
Basu T, Maguire J, Salpekar JA. Hypothalamic-pituitary-adrenal axis targets for the treatment of epilepsy[J]. Neurosci Lett, 2021, 746: 135618.
[25]
张艳, 姬辛娜, 陈倩. 促肾上腺皮质激素治疗婴儿痉挛症的临床和基础研究进展[J]. 中华神经医学杂志, 2022, 21(4): 420-424.
[26]
Sumanasena SP, Wanigasinghe J, Arambepola C, et al. Developmental profile at initial presentation in children with infantile spasms[J]. Dev Med Child Neurol, 2019, 61(11): 1295-1301.
[27]
Riikonen R. Infantile spasms: outcome in clinical studies[J]. Pediatr Neurol, 2020, 108: 54-64.
[28]
Bitton JY, Desnous B, Sauerwein HC, et al. Cognitive outcome in children with infantile spasms using a standardized treatment protocol. A five-year longitudinal study[J]. Seizure, 2021, 89: 73-80.
[29]
Chourasia N, Yuskaitis CJ, Libenson MH, et al. Infantile spasms: assessing the diagnostic yield of an institutional guideline and the impact of etiology on long-term treatment response[J]. Epilepsia, 2022, 63(5): 1164-1176.
[30]
Daida A, Hamano SI, Hayashi K, et al. Comparison of adrenocorticotropic hormone efficacy between aetiologies of infantile spasms[J]. Seizure, 2021, 85: 6-11.
[31]
Dang HD, Zou LP, Tian JH, et al. Etiologic classification of infantile spasms using positron emission/magnetic resonance imaging and the efficacy of adrenocorticotropic hormone therapy[J]. Eur J Nucl Med Mol Imaging, 2020, 47(6): 1585-1595.
[32]
Jain P, Sahu JK, Horn PS, et al. Treatment of children with infantile spasms: a network meta-analysis[J]. Dev Med Child Neurol, 2022, 64(11): 1330-1343.
[33]
Demarest ST, Shellhaas RA, Gaillard WD, et al. The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium[J]. Epilepsia, 2017, 58(12): 2098-2103.
[34]
钟金花, 廖建湘, 胡雁, 等. 促肾上腺皮质激素治疗婴儿痉挛症的疗效分析[J]. 第三军医大学学报, 2012, 34(22): 2257-2259.
[35]
O'Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study[J]. Epilepsia, 2011, 52(7): 1359-1364.
[36]
Surana P, Symonds JD, Srivastava P, et al. Infantile spasms: Etiology, lead time and treatment response in a resource limited setting[J]. Epilepsy Behav Rep, 2020, 14: 100397.
[37]
Sehgal R, Gulati S, Sapra S, et al. Prognostic utility of clinical epilepsy severity score versus pretreatment hypsarrhythmia scoring in children with west syndrome[J]. Clin EEG Neurosci, 2017, 48(4): 280-287.
[38]
Zhang Y, Yu L, Zhou Y, et al. Prognostic utility of hypsarrhythmia scoring in children with West syndrome after ketogenic diet[J]. Clin Neurol Neurosurg, 2019, 184: 105402.
2024年第49卷第8期
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doi: 10.11855/j.issn.0577-7402.0212.2024.0229
  • 接收时间：2023-03-13
  • 首发时间：2025-11-21
  • 出版时间：2024-08-28
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  • 收稿日期：2023-03-13
  • 录用日期：2023-07-18
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Medical Science Research Project of Chengdu(2019018)
成都市医学科研课题(2019018)
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    电子科技大学医学院附属妇女儿童医院.成都市妇女儿童中心医院神经内科，四川成都 611731

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2种不同金属材料的力学参数
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Number of
genus		 种数
Number of
species		 占总种数比例
Percentage of
total species (%)		 属
Genus		 种数
Number of
species		 占总种数比例
Percentage of total
species (%)
鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
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