视频脑电图评估促肾上腺皮质激素治疗婴儿痉挛症的疗效

目的 分析促肾上腺皮质激素(adrenocorticotrophic hormone,ACTH)治疗婴儿痉挛症(infantile spasms,IS)前后视频脑电图变化的特点.方法 收集我院2005年1月至2009年12月住院的65例IS患儿的临床资料,分析其用ACTH治疗前后视频脑电图变化的特点.结果 65例患儿用ACTH治疗前IS发作间期脑电图背景均出现高峰失律现象;丛集性痉挛发作频繁出现,并可见同期特征性发作图形.ACTH治疗后,40例(61.5%,40/65)完全控制发作的患儿中,27例(67.5%,27/40)脑电图高峰失律背景消失或明显好转;另25例(38.5%,25/65)仍有发作的患儿中,仅5例(20.0%,5/25)脑电图高峰失律背景消失或明显好转.完全控制发作和仍有发作患儿的脑电图高峰失律背景消失或好转率比较差异有统计学意义(x2=13.888,P＜0.000).结论 IS患儿发作前视频脑电图监测是正确诊断IS的重要依据,ACTH治疗后视频脑电图监测是判断短期疗效的重要指标.     

常规剂量与小剂量促肾上腺皮质激素治疗婴儿痉挛的对照研究

目的：比较常规剂量与小剂量促肾上腺皮质激素（ACTH）治疗婴儿痉挛（West syndrome,WS）疗效及副作用的差异,寻找ACTH的最小有效剂量。方法：采用前瞻性随机对照研究,将30例WS（8例隐原性,22例症状性）随机分为常规剂量组（n=15）或小剂量组（n=15）。常规剂量组ACTH 50 IU/d×2周,随后2周减量至停药;小剂量组每日0.4 IU/kg×2周,若痉挛发作停止,随后2周减量至停药,若未完全控制或无效,加量至每日1 IU/kg 再用2周,随后用2周的时间逐渐减量至停药。比较ACTH两种不同剂量治疗WS的疗效及副作用。结果：常规剂量与小剂量治疗WS的近期疗效相似,有效率分别为53%及60%（P>0.05）,治疗后脑电图变化、痉挛复发及复发时间两组间差异无显著性（P>0.05）。8例完成12个月以上随诊,长期疗效两组间也无差异。ACTH对隐原性WS的有效率及脑电图高峰失律消失例数均高于症状性组（P<0.05）。常规剂量组副作用发生率(93%,14/15)明显高于小剂量组(20%,3/15),常规剂量组中1例出现轻度脑萎缩。结论：ACTH 50 IU/d与每日0.4 IU/kg两种剂量治疗WS的近期及远期疗效相似,对隐原性WS的疗效优于症状性,为避免副作用的发生,ACTH的使用应从小剂量（每日 0.4 IU/kg）开始。［中国当代儿科杂志,2009,11（6）：445-448］     

不同剂量促肾上腺皮质激素对婴儿痉挛症疗效和安全性的Meta分析

目的:评价不同剂量促肾上腺皮质激素（ACTH）治疗婴儿痉挛症（IS）的疗效及安全性。方法:检索PubMed、EMBASE、Ovid、ScienceDirect、中国期刊全文数据库、万方数据库、CiNii（国立情報学研究所論文情報ナビゲータ）和Medical Online等数据库,并手工检索会议论文汇编和专题论文集等,收集关于不同剂量ACTH治疗IS的RCT文献。依据随机分配方法、分配隐藏、盲法、结果数据的完整性、选择性报告研究结果及其他偏倚来源进行文献偏倚评价。采用Review Manager 5.0软件进行Meta分析,无法进行合并分析的资料进行描述性分析。结果:共检索到836篇相关文献,符合纳入标准的4篇RCT文献进入Meta分析。文献偏倚评价结果显示,1篇文献存在中度偏倚风险,3篇文献存在高度偏倚风险。Meta分析结果显示,不同剂量ACTH组痉挛发作缓解率（OR=1.09,95%CI：0.54~2.17,P=0.82）、EEG高度失律消失率（OR=1.04,95%CI：0.43~2.51,P=0.94）及复发率（OR=1.27,95%CI：0.64~2.54,P=0.50）差异均无统计学意义。进一步按症状性和隐原性IS行亚组分析,结果显示两亚组不同剂量ACTH上述疗效指标差异均无统计学意义。大剂量ACTH组高血压和体重增加等不良反应的发生率高于小剂量ACTH组。结论:现有证据提示,不同剂量ACTH治疗IS在痉挛发作缓解率、EEG高度失律消失率及复发率方面疗效相近。小剂量 ACTH所致不良反应更少。鉴于纳入Meta分析的RCT文献数量较少且质量较低,明确结论仍需进一步的RCT研究。     

促皮质素与生酮饮食治疗新发婴儿痉挛症的随机对照研究

目的 通过促皮质素(ACTH)与生酮饮食(KD)治疗新发婴儿痉挛症(IS)的随机对照研究,比较二者的疗效、安全性、耐受性,证实KD能否作为IS的首选治疗方法.方法 将不同时间收治的30例IS新发病例随机分为2组,每组15例.分别给予KD及ACTH治疗,于治疗2周及1、3、6、12个月观察和对比二者的疗效、脑电图改善情况、不良反应及治疗保留率情况.结果 治疗2周,ACTH组与KD组无发作比例分别为71.4%(10/14例)与33.3%(5/15例),ACTH组优于KD组(P=0.039),ACTH治疗有效病例起效时间为(6.00±4.10) d,而KD治疗有效病例起效时间为(9.31±3.59) d,ACTH组起效快于KD组(P<0.05),但治疗3、6、12个月,ACTH组无发作比例分别为50.0%、35.7%、28.6%;KD组无发作比例分别为85.7%、81.8%、75.0%.KD组均优于ACTH组(P=0.045,0.025,0.043),同时脑电图的高峰节律紊乱背景的改善情况与发作控制情况基本平行.1 a后,ACTH组与KD组治疗保留率分别为26.7%(4/15例)及40%(6/15例),二者差异无统计学意义(P=0.227).观察期间,ACTH组与KD组分别出现19例次与16例次的不良反应,但经对症处理后均能很快好转.结论 ACTH治疗IS起效较KD快,近期疗效优于KD治疗,但其停药后复发率逐渐增高,远期疗效不及KD治疗,提示KD可考虑作为IS的首选治疗方案.     

泼尼松治疗婴儿痉挛症有效性及HPA轴机制分析

目的 通过研究泼尼松治疗前后婴儿痉挛症(infantile spasm,IS)的发作、脑电图的改变及下丘脑-垂体-肾上腺(hypothalamus-pituitary-adrenal,HPA)轴功能的改变,探讨泼尼松治疗婴儿痉挛症的有效性、HPA轴在IS发病机制中的作用,阐明泼尼松控制痉挛发作的HPA轴相关机制.方法 共收集30例符合标准IS病例(IS组),与30例健康婴幼儿(对照组)对比,对30例IS病例在泼尼松治疗前后进行发作次数记录、EEG监测、HPA轴功能检测,采用化学发光法检测血清皮质醇、促肾上腺皮质激素(adreno-cortico-tropic-hormone,ACTH)水平;利用酶联免疫吸附法检测血清促肾上腺皮质激素释放激素(corticotro-phin Releasing hormone,CRH)水平.结果 用药前IS组血清CRH水平均较正常对照组高,且差异有统计学意义(P<0.05),而IS组血皮质醇、ACTH与正常对照组比较无明显差别,差异无统计学意义(P>0.05);平均每日发作串数、平均每日发作总次数分别与CRH呈正相关(P<0.05);应用泼尼松后,经泼尼松治疗后30例IS病例有19例发作控制,11例发作未控制,有18例脑电高度失律完全缓解,12例脑电高度失律未完全缓解,治疗后每日发作串数及每日发作总次数均较治疗前明显下降,差异有统计学意义(P<0.05),治疗后的DQ较治疗前DQ改善,有统计学意义(P<0.05),病程是泼尼松治疗效果的主要影响因素,病程越长,治疗效果越差,差异有统计学意义(P<0.05).治疗后的CRH、皮质醇、ACTH较治疗前明显下降,差异有统计学意义(P<0.05).结论 泼尼松能有效控制婴儿痉挛症的发作,早期治疗效果更好,IS患儿存在HPA轴功能紊乱,泼尼松能调节HPA轴功能紊乱起到控制痉挛发作的效果.     

不同剂量托莫西汀治疗注意缺陷多动障碍44例临床分析

目的探讨不同剂量托莫西汀治疗注意缺陷多动障碍(ADHD)的疗效和安全性。方法将2008年11月至2009年10月广州医学院荔湾医院心理门诊及住院的44例7～16岁ADHD患儿分为大剂量组21例[剂量由0.5mg(/kg·d)增至1.2mg(/kg·d)];小剂量组23例[0.5mg(/kg·d)];均每日晨顿服,共8周。在治疗前和治疗8周末,以中文版SNAP-Ⅳ家长评定量表进行疗效评定,采用治疗副反应量表(TESS)评估其治疗过程中的副反应。结果治疗后两组SNAP-Ⅳ家长评定量表总分均较治疗前下降(P0.01)。治疗8周后,大、小剂量组有效率分别为76.1%和60.3%,差异有统计学意义(P0.05);完全缓解率分别为47.6%和26.1%,差异有统计学意义(P0.01)。两组均未见严重副反应,大、小剂量组常见的副反应为食欲减退(28.6%、13.0%,P0.01),恶心(9.5%、8.7%,P0.05),瞌睡/疲倦(19.0%、8.7%,P0.05),头晕(4.8%、4.3%,P0.05),腹痛(9.6%、4.3%,P0.05)和体重下降(23.8%、8.7%,P0.01)。结论大剂量组托莫西汀治疗儿童ADHD的疗效优于小剂量组,且安全性均较好,未出现严重副反应。     

泼尼松龙与促肾上腺糖皮质激素治疗婴儿痉挛症的Meta分析

目的 比较泼尼松龙与促肾上腺糖皮质激素（ACTH）治疗婴儿痉挛症（IS）的疗效和安全性。方法 通过检索Cochrane Library、Embase、PubMed、中国生物医学文献数据库、中国知网和万方数据库等中英文数据库，收集比较泼尼松龙与ACTH治疗IS的临床研究，进行文献筛选、资料提取和质量评价，采用Review Manager 5.3软件进行Meta分析。结果 根据文献纳入、排除标准，最终纳入5项研究。Meta分析结果显示，泼尼松龙治疗组和ACTH治疗组痉挛缓解率、痉挛缓解时间、并发感染率和易激惹率的比较差异均无统计学意义（P > 0.05），而ACTH治疗组的EEG高峰节律紊乱消失率高于泼尼松龙组（P < 0.05）。结论 现有证据未显示采用泼尼松龙和ACTH治疗IS的临床疗效有差异，但ACTH在稳定EEG上更具优势；感染、易激惹等不良反应的发生在两种疗法中也未见差异。     

大剂量甲泼尼龙对婴儿痉挛幼鼠脑组织的保护作用

目的 婴儿痉挛症(infantile spasms,IS)是婴儿期最常见的难治性癫痫性脑病,但相关动物实验缺乏,该研究通过构建IS动物模型,探讨大剂量甲泼尼龙对IS幼鼠脑组织的影响.方法 将生后10 d(P10)SD大鼠随机分为对照组、模型Ⅰ组和模型Ⅱ组,每组30只.模型Ⅰ组和模型Ⅱ组分别在P10、P13腹腔注射N-甲基-D-天冬氨酸(N-methyl-D-aspartate,NMDA)诱发癫痫发作,模型Ⅱ组在P13应用NM-DA前连用3 d甲泼尼龙.观察幼鼠癫痫发作并评分,原位末端标记法(TUNEL)检测凋亡情况,免疫组织化学法测Bax、Bcl-2蛋白表达、实时荧光定量PCR检测海马组织Bcl-2、Bax和caspase-3 mRNA的表达.结果 (1)P13模型Ⅰ组癫痫发作率83.3%(25/30),模型Ⅱ组均未出现发作,模型Ⅱ组较模型Ⅰ组癫痫发作率明显降低;(2)模型Ⅱ组凋亡细胞数(14.37±2.02)少于模型Ⅰ组(25.67±1.52),多于对照组(9.00±2.50),差异均有统计学意义(均P<0.05);(3)模型Ⅱ组Bax蛋白及mRNA表达量(44.55±3.58,2.35±1.01)明显低于模型Ⅰ组(58.05±4.62,3.27±0.95),高于对照组(28.90±5.14,1.68±0.50),差异均有统计学意义(均P<0.05);(4)模型Ⅱ组caspase-3mRNA表达量(5.99±1.75)低于模型Ⅰ组(7.88±1.60),高于对照组(3.60±1.70),差异均有统计学意义(均P<0.05).结论 大剂量甲泼尼龙能减轻NMDA致IS幼鼠癫痫发作;大剂量甲泼尼龙对NMDA致IS幼鼠具有脑保护作用,这种保护作用可能与其减轻癫痫发作,减少细胞凋亡有关.     

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目的对促肾上腺皮质激素(ACTH)治疗的婴儿痉挛(IS)患儿进行追踪随访,了解其预后并评价影响预后的因素。方法(1)对北京大学第一医院儿科1993年1月至2003年12月住院的53例ACTH治疗的IS患儿的临床随访资料进行回顾性研究。(2)ACTH治疗方法:采用ACTH静滴,25~40IU/d,共4周。结果(1)对53例ACTH治疗患儿随访6个月至10年,ACTH治疗发作完全缓解的31例,随访2年以上,共有16例复发,复发率为51·6%,其中于停用ACTH后2~3个月复发者占50·0%(8/16),复发形式以肌阵挛发作、痉挛发作、部分运动性发作为主;(2)临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关(病程≤2个月者、ACTH近期疗效好的临床发作预后好),与发病年龄、病因无关;(3)智力预后与ACTH开始治疗时的病程、病因、发作预后有关(病程≤2个月者、隐原性者、发作完全缓解者智力预后好),与发病年龄、ACTH近期疗效无关。结论(1)IS患儿临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关。(2)智力预后与ACTH开始治疗时的病程、病因、发作预后有关。     

不同剂量泼尼松联合托吡酯治疗婴儿痉挛的疗效及安全性北大核心CSCD

目的 评价不同剂量泼尼松联合托吡酯（TPM）治疗婴儿痉挛（IS）的临床疗效和安全性,为IS的用药提供新的选择.方法 收集2011年5月至2012年12月就诊于江西省儿童医院神经内科的56例IS患儿,随机分为对照组和试验组（各28例）.对照组予泼尼松[2 mg/（kg·d）,2次/d]口服2周,试验组予口服泼尼松（40 mg/d,每6h1次）2周,2组均添加TPM,初始1 mg/（kg·d）,1次/d或2次/d,于2周内调至维持量3 ～5 mg/（kg·d）,2次/d.2周时如果发作控制则2组激素均开始减量,在5周内逐渐减量直至停药,总疗程7周.如2周后仍有发作,2组激素均按初始剂量延长2周,再减量,总疗程达9周.所有患儿于治疗前和治疗2周后及疗程结束（7周或9周）时进行视频脑电图（VEEG）监测和痉挛发作控制情况评估,并记录药物不良反应,Gesell发育测试于治疗前和治疗6个月后（仅对发作缓解6个月以上者）进行.所有患儿接受2～18个月的随访.结果 1.治疗2周时,试验组和对照组中IS患儿痉挛发作完全控制率分别为75.00％（21/28例）和28.57％（8/28例）,差异有统计学意义（x2=12.087,P=0.001）;同期高度失律缓解率分别为60.71％（17/28例）和21.43％（6/28例）,差异有统计学意义（x2=8.928,P=0.003）.疗程结束时,试验组和对照组中IS患儿痉挛发作完全控制率分别为67.86％（19/28例）和35.71％（10/28例）,差异有统计学意义（x2=5.793,P=0.016）.同期高度失律缓解率分别为57.14％（16/28例）和14.29％（4/28例）,差异有统计学意义（x2=11.200,P=0.001）.2.不良反应：试验组和对照组中不良反应均以体质量或食欲增加为最常见,2组不良反应出现率分别为82.14％（23/28例）和67.86％（19/28例）,2组间比较差异无统计学意义（x2=1.524,P=0.217）.2组中均无一例因药物不良反应而退出治疗.3.试验组和对照组中IS患儿复发率分别为31.82％（7/22例）和72.73％（8/11例）,2组比较差异有统计学意义（x2 =4.950,P=0.026）;试验组中9例痉挛发作缓解6个月以上患儿治疗前后发育商比较,均值增大,但差异无统计学意义（t =2.271,P=0.053）.结论 大剂量泼尼松联合TPM治疗IS疗效明显优于常规剂量泼尼松联合TPM.     

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??Abstract??Objective??To explore the effects of methylprednisolone ??MP?? of different doses on vascular endothelial growth factor ??VEGF?? of early acute lung injury??ALI????providing a theoretical basis for clinical treatment. Methods??Totally 48 cases who met ALI diagnostic criteria from PICU were randomly divided into 3 groups?? group ALI?? group HALI ??treated by high-dose MP???? group LALI ??treated by low-dose MP??. Eight heathy individuals were selected as control group??NC??. Results????1??As compared with group NC?? the serum VEGF and blood lactate were significantly higher??while oxygenation index was significantly lower than in other groups ??P <0.05??. ??2??Contents of serum VEGF?? blood lactate of group HALI and LALI stepped significantly down??but oxygenation index stepped significantly up than group ALI after 24 h??P < 0.05???? ??3??Between group HALI and LALI??the serum VEGF??blood lactate??oxygenation index at 24 h?? 48 h and 72 h had no significant differences??P > 0.05??. ??4??Compared with group ALL?? ARDS??acute respiratory distress syndrome?? patients within 72h were stepped up significantly than group HALI and LALI??P < 0.05????but there were no significant differences between the two groups??P > 0.05??. Conclusion??The expression of VEGF is increased in early ALI?? low-dose MP can inhibit the progression of ALI by inhibiting the expression of serum VEGF.     

Epidemiologic surveillance of nosocomial infections in a Pediatric Intensive Care Unit of a developing country

Background

Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan.

Methods

All patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration.

Results

Fifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 ± 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH.

Conclusion

Vigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger studies from developing countries are required to validate the therapeutic trends observed in this study.     

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目的探讨晚发型癫痫性痉挛患儿的临床和脑电图(EEG)特征、治疗反应及预后。方法对北京大学第一医院儿科2000年6月至2007年8月59例晚发型癫痫性痉挛患儿的临床和EEG资料进行回顾性研究,其中37例有随访,随访时间13～90个月,末次随访年龄为2岁6个月至12岁2个月(中位数60个月)。结果59例患儿癫痫起病年龄为12～98个月,中位年龄22个月。以痉挛发作起病36例(61.0%),以其他发作类型起病23例(39.0%)。17例(28.8%)在病程中有部分性发作,22例(37.3%)合并其他全面性发作形式,20例(33.9%)病程中仅有痉挛发作。EEG14例(23.7%)为典型高度失律;2例(3.4%)为一侧性高度失律;43例(72.9%)无典型的高度失律。总计22例(37.3%)表现为局灶性或一侧性临床、影像学和(或)EEG异常。44例(74.6%)为症状性癫痫,围生期脑损伤、中枢神经系统感染、脑结构异常为主要病因。14例(23.7%)符合晚发型West综合征,7例(11.9%)符合Lennox-Gastaut综合征。促肾上腺皮质激素(ACTH)治疗后近期无发作率32.0%,有效率58.0%,复发率为61...     

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??Objective??To investigate the efficacy and safety of propofol used as anesthesia and deep sedation during flexible bronchoscopy in children. Methods??The clinical data of 206 children with atelectasis who underwent flexible bronchoscopic alveolar lavage in the Endoscopy Room of the Respiratory Department of Hebei Children’s Hospital from January 2016 to January 2017 were retrospectively analyzed. Children for ASA??/?? level were divided into two groups according to the sedation method :there were 106 cases in the propofol group??2 mg/kg?? and 100 cases in the midazolam group??0.1 mg/kg??. To compare the onset time of anesthetict??heart rate??HR????respiratory rate??RR????mean arterial pressure??MAP????percutaneous oxygen saturation??SPO2?? before and after anesthesia induction??T0??T1????during endoscopy placement??T2??and after awakening??T3?? at 4 time points??operative duration??the waked duration of postoperation??the rate of adverse reactions??hiccups??respiratory depression??in the operation and Ramsay score between two groups. All data were analyzed by SPSS 20.0 statistical software. Results????1??There were statistically significant differences at the onset time of anestheticts??operative duration and the rate of side-effects between the two groups??P??0.05??. There was no statistical differences in awake time??P??0.05??.??2??There was no significant difference in MAP or SPO2 at time points of T0??T1??T2??T3??P??0.05????whereas the difference in HR and RR at time points of T1??T2??T3 between the two groups was statistically significant??P??0.05??. ??3??The sedative effect of propofol group was significantly better than that of midazolam group in Ramsay sedative scoring. Conclusion??Propofol used for anesthesia and deep sedation works fast??safely and effectively in flexible bronchoscopy for children??the time to gain consciousness is short??the operation time is short and there is fewer side effects??which is worth promoting.     

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??Abstract??Objecive??To compare the efficacy and safety of 10-day sequential treatment??including rabeprazole?? amoxicillin and clavulanate for oral suspension??clarithromycin?? and 7-day traditional trigeminy therapy ??including omeprazole?? amoxicillin and clarithromycin?? for the eradication of helicobacter pylori??HP??in children. Methods??From Oct.2006 to Aug.2009??123 children with gastropathy with Hp infection??in Luoyang Locomotive Hospital of Henan??were divided into two groups.The control group ??41?? was treated with traditional trigeminy therapy for 7 days??while the treatment group??82??was treated with 10-day sequential treatment.Cure rates of the ulcer healing and Hp eradication were assessed after treatment course of 7-10 days??while the symptom scores and the side effect were evaluated and investigated after the treatment. Results The healing rate of ulcer in control group was 40%??2/5????which was 55.5%??5/9?? in the treatment group??the difference being statistically significant??P??0.05??. The Hp eradication rate was 92.68%??76/82??and 70.73%??29/41??in treatment group and control group?? respectively .There was significant statistical difference??P??0.05??. The total symptom score of treatment group before treatment was??56.89±4.32???? and after treatment it was ??14.96±2.13????while that of control group before treatment was ??58.75 ± 5.12?? and ??21.42 ± 3.78?? after treatment??P??0.05 or P??0.01????the change of treatment group being more significant??P??0.01??. The side effect rate of treatment group was lower than that of control group ??6.1% VS 17.1%??respectirely??P??0.05??. Conclusion??The 10-day sequential treatment??including rabeprazole??amoxicillin and clavulanate for oral suspension and clarithromycin??is much more efficient with lower side effect than 7-day traditional trigeminy therapy for Hp eradication in children with gastropathy with Hp infection.     

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??Abstract??Objective To study the clinical and neuro-electrophysiological features of transient epileptic seizures by using polygraphic channel VEEG—EMG monitoring. Methods The information of 51 epilepsy children with rapid falling??nodding or limb shaking in video electroencephalogram was collected in the Department of Pediatric Neurology?? Peking University First Hospital from June 2012 to March 2013.The clinical features??EEG and EMG patterns were analyzed retrospectively. Results Totally 745 seizures were found in 51 patients. The seizures included 391 epileptic spasms??52.5%????138 myoclonic seizures??18.5%????117 atonic seizures??15.7%????61 negative myoclonus??8.2%????27 tonic seizures??3.6%??and 11 myoclonic atonic seizures??1.5%??.Different clinical manifestations were with different clinical-EEG-EMG patterns.According to the duration of EMG changes??patients were divided into EMG-burst group and EMG-static group. Conclusions Epileptic seizures with rapid falling, nodding or limb shaking are most affected by epileptic spasms??myoclonic seizures??atonic seizures??negative myoclonus??tonic seizures and myoclonic atonic seizures.Their common feature is rapid and transient??which makes them difficult to be diagnosed only by illness history.But by performing VEEG-EMG??we can accurately identify the types of seizures??which will be helpful in the diagnosis and treatment.     

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目的了解单次小剂量(0.4g/kg)静脉输注免疫球蛋白(IVIG)提升初发免疫性血小板减少性紫癜(ITP)患儿血小板至安全范围(≥30×109/L)的作用。方法研究对象为北京大学第一医院儿科2008-04-01—2011-04-01收治初发ITP患儿62例,其中2008-04-01—2009-10-01收治的30例为激素组,初始接受常规剂量醋酸泼尼松治疗;2009-10-02—2011-04-01就诊的32例为IVIG组,初始接受0.4g/(kg·d)IVIG治疗1～5d,每天复查血常规,血小板升至安全范围则规范停用。比较两组治疗第1、3、5天时血小板升至安全范围比例及长期随访结果。结果治疗前,激素组和IVIG组血小板中位值分别是10×109/L和6×109/L。治疗1d后两组血小板升至安全范围的比例分别是3.33%和43.75%,差异有统计学意义(P<0.01)。随访7～42个月后激素组和IVIG组分别有3.45%和3.23%血小板未升至正常(≥100×109/L)。所有患儿均无颅内出血发生及死亡。结论单次小剂量IVIG可使近半数初治ITP患儿血小板升至≥30×109/L相对安全范围,明显高于常规剂量醋酸泼尼松疗效。     

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??Abstract??Objective??To study the clinical features of hand-foot-mouth disease ??HFMD?? caused by enterovirus 71 ??EV71?? infection. Methods Clinical data of hospitalized children with hand-foot-mouth disease caused by EV71 infection from May 2010 to August 2010 were analyzed retrospectively. The difference of clinical manifestation and results of auxiliary examination between intensive HFMD group and serious HFMD group were compared. Results??High fever and nontypical skin rash showed significant difference between intensive group and serious group??P = 0.002??P = 0.000??respectively???? 120 EV71-positived HFMD cases ??99.17%?? showed neurological impairments. The major neurological features included fatigue ??84.30%????frequent vomiting ??65.30%????limb tremble ??60.33%?? and sleep disorders ??53.72%??. The rate of abnormal knee reflex was 52.07% in physical examination. The incidence of vomiting??P = 0.001????unconsciousness ??P = 0.000????abnormal muscular tension??P = 0.000????abnormal heart rate ??P = 0.000????dysarteriotony ??P = 0.000????capillary refill time being more than 3 seconds ??CRT > 3 s?? ??P = 0.000????tachypnea or dyspnea ??P = 0.000?? and pulmonary exudative lesion in chest X-ray ??P = 0.000?? was morefrequent in serious group compared with intensive group??There were 51 cases ??42.15% ?? with a peripheral blood WBC count of more than 12 × 109/L or less than 4×109/L??52 cases ??42.98% ?? with blood glucose level of more than 6 mmol/L and cardiac troponin I elevated in 22 cases ??18.18%??. The above three indexes were significantly different between two groups ??P < 0.000??respectively??. Conclusion??HFMD caused by EV71 infection often shows neurological impairments. High fever??nontypical skin rash??frequent vomiting??unconsciousness??abnormal muscular tension??abnormal heart rate??dysarteriotony??CRT > 3s??tachypnea or dyspnea??and pulmonary exudative lesion are risk factors of serious HFMD. Early identification and correct treatment are the key to the rescue of serious HFMD.     

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??Objective??To compare the effect of metoprolol with that of midodrine in the treatment of vasovagal syncope??VVS?? using a systematic review. Methods??The databases of PubMed??Cochrane Library??ScienceDirect??CBM??CNKI and Wanfang were searched by computer. The search time was from the construction of databases to Nov. 2017. According to the Cochrane systematic review??we screened the data on the effects of metoprolol and/or midodrine on VVS??both English and Chinese randomized controlled trials??RCTs?? were screened. Meta-analysis was performed using RevMan 5.3 software. Efficacy of metoprolol group and/or midodrine group was compared. Results??The symptom improvement rate??4 RCTs showed that metoprolol group was superior to the basic treatment group??P??0.01????4 RCTs suggested that the midodrine group was better than the basic treatment group??P??0.01???? and 2 RCTs showed there was no significant difference between metoprolol group and midodrine group??P??0.63????HUTT negative rate??6 RCTs showed metoprolol group was superior to the basic treatment group??P??0.01????3 RCTs indicated that the efficacy of midodrine group was better than the basic treatment group??P??0.01????and 3 RCTs indicated there was no significant difference between metoprolol group and midodrine group??P??0.05????the recurrence syncope rate??3 RCTs showed that metoprolol group was better than the basic treatment group??P??0.01????4 RCTs indicated that the effect of midodrine group was better than that of the basic treatment group??P??0.01????and 2 RCTs showed that there was no significant difference between the metoprolol group and midodrine group??P??0.51????adverse reaction rate??3 RCTs showed there was no significant difference between the metoprolol group and midodrine groups??P??0.14??. Conclusion??Both metoprolol and midodrine can improve the clinical symptoms of patients with VVS??increase the number of negative HUTT test after treatment??and reduce the number of recurrence of syncope. However??there is no significant difference in efficacy indicators.Therefore??large-scale??multicenter RCTs are still needed to investigate the efficacy of drug treatment for VVS.