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??Endoscopic diagnosis and treatment is the most common and reliable method in digestive system disease. As an advanced medical treatment method??digestive endoscopy has been widely used in pediatrics. Digestive endoscopy has positive effects on the diagnosis and treatment of pediatric digestive system diseases. Children have poor tolerance?? low ability of self-regulation and strain capacity??and rapid disease progression. The perioperative management must be done carefully. In the current review??preoperative assessment and preparation??management of surgical procedures??postoperative management were discussed.     

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??Abstract?? The treatment of SLE becomes standard in recent year. Serious complications caused by infection become the leading cause of death in SLE?? more than renal involvement and lupus encephalopathy. SLE co-infection mainly connects with the defects in humoral immunity and cellular immunity. And also connect with the disease activity?? the dose of hormone and immune inhibitor.     

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??Objective To determine the relationship between clinical features of bronchiolitis in children under 2 years old and lymphocyte subsets ratio. Methods Two hundred and sixty-eight hospitalized children in Children Hospital of Soochow University from January 2014 to September 2015 were enrolled in this study. Peripheral blood was collected and cellular immunity was detected by flow cytometry. Pathogens were tested and patients’ clinical data was collected. Results Bronchiolitis infants were identified in 11.84% of 2264 patients in corresponding period. Prevalence rate of pathogen was 57.84%??whose sequence was??from high to low?? respiratory syncytial virus??RSV??21.27%????mycoplasma pneumoniae??MP??16.42%????Haemophilus influenzae??10.07%????Streptococcus pneumoniae??8.96%??. CD3+??CD3+CD8+ ratio of the children with bronchiolitis and without wheezing patients was lower than healthy control group. The CD4+/CD8+ ratio was the highest in bronchiolitis group??which was the lowest in healthy control group. The CD3- CD19+ ratio was higher in bronchiolitis and no-wheezing group than in healthy control group??P??0.05??. Conclusion Lymphocyte subsets disorder in brochiolitis children was samilar to that in asthma patients. Children between 6 months and 1 year old were more likely to develop bronchiolitis than the other two groups. Bronchiolitis infants may have high expression tendency of B lymphocyte??especially those with allergic symptoms. RSV is still the most common pathogen in bronchiolitis.     

MECP2基因及MECP2相关疾病

<正>Andrea Rett首次于1966年报道一例青年女性在1~1.5岁之前正常发育,以后出现认知、运动倒退以及手的刻板动作,提出一类疾病"认知倒退、脑萎缩伴有高氨血症"~([1])。伴有高氨血症的类似病例因此受到关注,不幸的是血氨升高是实验室误差所致。直到1983年,Hagberg等~([2])在Annals of Neurology报道了类似发育倒退但没有高氨血症     

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先天性房性快速性心律失常在儿科临床上很少见。由于出生前胎心率就明显增快,易被误诊为宫内窘迫等,分娩后婴儿仍心动过速,经心电图等检查才能确立诊断。现结合近期诊治的2例临床资料,就本病的诊断和治疗等问题加以讨论。例1.患儿男,生后1d。足月第1产。胎心率明显增快(185～190/min),疑宫内窘迫行剖宫产,生后心率200/min。查体:发育良好,呼吸稍促,无发绀。两肺无干湿啰音?率200/min,律齐,心音有力,无杂音。腹部软,肝右肋下1cm,四肢温,不肿。住院诊治经过:于2004-09-08入院,住院后曾做心脏彩色多普勒超声心动图检查,心内结构及血流未见异…     

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例1.女,2岁6个月,因咳嗽、气促3个月于2004年10月1日入院。2004年7月初患麻疹肺炎后一直有咳嗽、气促入住当地医院,诊为迁延性肺炎,怀疑有肺结核或异物。住院期间3次痰培养分别为卡他球菌、大肠埃希菌、铜绿假单胞菌生长。肺功能检查为混合性通气功能障碍。纤维支气管镜检查为:(1)慢性支气管内膜炎;(2)左主支气管变窄(考虑漏斗胸所致)。胸部CT检查,示“双肺斑片状阴影,闭塞性细支气管炎?”,先后予仙力素、氟康唑、大蒜素、红霉素、复达欣、泰能等治疗,病情好转出院。出院后仍有咳嗽、气促,活动后喘息入我院。入院查体:体温37.6℃,脉搏156/m…     

艾滋病2例

例1,女,5岁,因发热、腹泻、呕吐2个月入院。2个月前因发热、腹胀,在当地医院治疗,诊断为结核性腹膜炎。经异烟肼、利福平及头孢曲松钠治疗后腹水消失。体温不降,出现脓血便、呕吐。大便红细胞5~10个／HP,白细胞10~13个／HP。脑脊液细胞总数3600×106／L,中性0.86,淋巴     

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1病例介绍1.1病例1患儿男,6岁3个月,主因“反复双下肢痛并大小便困难半年,加重20d”。于2006-03-10入住我院脊柱外科。体格检查:轻度贫血貌,皮肤无瘀斑及出血点,肝脾淋巴结未及肿大,脊柱腰段生理弯曲变直,腰椎各向活动轻度受限,椎旁肌肉略紧张痉挛,L5/S1棘突间及棘突旁两侧1cm有压痛,并向腰大肌两旁放射。下肢各肌肌力无减弱,肌张力增高,下肢皮肤感觉无减退。屈颈试验(+)。双侧膝腱反射活跃,双侧跟腱反射活跃,肛周、会阴感觉及肛门反射减弱。双侧上肢肌力感觉关节活动正常,双侧Hoff-mann征(-),Barbanski征(+)。诊治经过:CT示骶管内偏左侧…     

口周肌阵挛伴失神2例临床和脑电图分析

目的探讨口周肌阵挛伴失神(POMA)的临床特点、脑电图特征及治疗。方法回顾2例POMA患儿的临床表现、脑电图特征及治疗效果,并结合文献进行分析。结果 2例患儿均以热性惊厥起病,发作表现为口周肌肉节律性抽动,伴有失神、意识障碍,发作次数较频繁,每次持续数秒至10 s。发作期脑电图特征为广泛性3 Hz棘慢波、慢波阵发,发作间期脑电图特征为中-极高波幅棘慢波、慢波散发或短阵发放。头颅影像学均正常,1例智力、运动发育稍落后,1例有癫痫家族史。2例患儿均予丙戊酸钠治疗,服药后均能控制发作,其中1例停药后复发。结论 POMA是以失神发作伴突出的口周肌阵挛为特征的特发性全面性癫痫综合征,视频脑电图对明确诊断有重要价值。     

肌阵挛失神癫癎临床及脑电图特征

目的 肌阵挛失神癫癎(epilepsy with myoclonic absences,EMA)是一种以肌阵挛失神(myoclonic absences,MA)为主要发作类型的儿童癫癎综合征.本研究旨在探讨EMA的临床及神经电生理特征.方法 对6例EMA患儿均进行视频脑电图(VEEG)监测,2例同步监测双侧三角肌肌电图(EMG).对EMA的临床、神经电生理特征、治疗及预后进行分析.结果 6例中女3例,男3例.起病年龄为2岁3个月～11岁,平均5岁2个月.5例以MA为惟一发作类型,1例以全身强直-阵挛发作起病,后转变为MA.MA临床表现为:不同程度意识改变,上肢为主的节律性肌阵挛抽搐、常伴强直性收缩、有时可见头及身体的偏斜或不对称抽搐,持续时间为2～30 s,发作突发突止,发作频繁时每日可达数次～30余次,过度换气常可诱发.所有患儿发作期EEG为双侧对称同步的3 Hz棘慢波节律性暴发,2例双侧三角肌EMG记录到与发作期放电频率一致的节律性肌电暴发,其中1例还记录到伴随的持续强直性肌电活动.所有患儿发作间期EEG均有醒睡各期全导棘慢波发放,部分存在少量局灶性放电.治疗主要采用丙戊酸单药或联合其他抗癫癎药.随访时年龄为6岁4个月～19岁,4例发作控制8个月～3年;1例因开始治疗晚、1例未及时正规治疗并伴病程中全身强直-阵挛发作,分别随访2年半及5年,目前仍有发作,且有智力受损.结论 EMA为一种少见的儿童癫癎性疾病,发作类型主要为MA.临床表现结合发作期VEEG及同步EMG可确诊MA.早期恰当的选用丙戊酸或联合其他抗癫癎药对EMA有效.治疗不及时或伴有全身强直-阵挛发作时预后相对不良.     

Clinical heterogeneity in eyelid myoclonia, with absences, and epilepsy

Eyelid myoclonia with absences should always be considered in the investigation of children with epilepsy.     

颈部肌阵挛伴失神发作2例报告并文献复习

目的探讨儿童颈部肌阵挛伴失神发作的临床特征。方法回顾分析2例以颈部肌阵挛伴失神为发作类型的儿童癫痫的临床和脑电图特征,并复习相关文献。结果 2例均为男孩,起病年龄分别为7岁9个月、9岁10个月,出生史及生长发育史无异常,无遗传性疾病及癫痫家族史。头颅影像无异常。视频脑电图(VEEG)显示背景节律无异常,发作间期睡眠期记录到前头部或广泛性棘波、棘慢波,发作均表现为节律性摇头运动伴头颈向一侧偏转,伴意识障碍,持续约6～13 s;发作期脑电图为广泛性极高波幅3 Hz左右棘慢波节律爆发,一侧胸锁乳突肌伴有与棘波同步的约50 ms节律性肌电爆发且伴有强直电位,过度换气能诱发发作,无光敏感。基因检测无异常。患儿经丙戊酸钠治疗后发作控制。文献检索国外共报道经VEEG证实为颈部肌阵挛伴失神发作患儿4例,起病年龄为4岁9月～12岁,1种或2种抗癫痫药物治疗4个月～3年后发作控制。结论颈部肌阵挛伴失神发作是一种具有独立脑电-临床特征的全面性癫痫发作类型。     

Eyelid myoclonia with absences occurring during the clinical course of cryptogenic myoclonic epilepsy of early childhood

We describe a 15-year-old boy with learning difficulties and eyelid myoclonia with absences (EMA). Myoclonic jerks of the extremities and trunk occurred 9 years before the onset of EMA, when the patient was 6. At that time, we diagnosed him with cryptogenic myoclonic epilepsy of early childhood, because he manifested mainly myoclonic jerks with generalized 3- to 6-Hz spike/polyspike-and-slow-wave complexes on EEG, normal neurological examination, good response to antiepileptic drugs, and no evidence of previous brain damage. This is an unusual case showing that myoclonic epilepsy of early childhood can evolve to EMA. Although the question of whether EMA is a seizure type or an epilepsy syndrome remains controversial, our case suggests that EMA is a seizure type during the clinical course of a particular kind of myoclonic epilepsy.     

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目的探讨额叶癫痫临床症状和视频脑电图(VEEG)特征及其与睡眠的相关性。方法 2010年10月至2011年1月中国医科大学附属盛京医院小儿神经内科确诊的27例额叶癫痫患儿,对VEEG监测期间的55次发作进行分析,发作特点及脑电图特征分别由有经验的临床医师和脑电图医师进行双盲分析。结果 22例中6例(22.2%)仅于清醒时发作,11例(50.0%)仅于睡眠时发作,清醒、睡眠无有发作5例(27.8%)55次癫痫发作表现复杂,运动症状突出,主要形式表现为过度运动性自动症23次(41.8%),伴不同程度意识障碍及发声16次(29.1%),姿势性强直发作15次(27.3%),口咽自动症14次(25.5%),先兆10次(18.2%),局部阵挛发作8次(14.5%),偏转性强直6次(10.9%),临床下放电5次(9.1%),发作后朦胧4次(7.3%),额叶失神3次(5.5%)。发作间期脑电图能定位于额叶者19例(70.4%)。51次监测到临床发作,脑电图表现为发作时或发作前瞬间脑电图改变为额区起源的局灶性痫样波发放。结论额叶癫痫是一组临床表现复杂多样的癫痫综合征,发作间期及发作前瞬间脑电图改变具有重要的定位诊断意义,睡眠时...