癫癎儿童发作间期癫癎样放电特点初步研究

目的癫癎发作及癫癎样放电具有昼夜倾向,睡眠是诱发癫癎的重要因素之一,但睡眠触发癫癎的易感性在不同的睡眠时相对发作及发作间期癫癎样放电的影响并不相同。该文主要研究局灶性及全面性癫癎儿童发作间期睡眠和觉醒状态癫癎样放电的特点。方法采用24h动态脑电图、多导睡眠脑电图及长程视频脑电图仪描记48例原发性癫癎儿童的全夜睡眠(至少一个完整的睡眠周期)和睡眠前后觉醒状态的脑电资料,统计分析觉醒期及各睡眠期棘/尖波、棘/尖慢波等癫癎样波的放电指数的差异,比较局灶性和全面性发作类型癫癎儿童癫癎样放电阳性率的差异。结果局灶性癫癎儿童组发作间期脑电图癫癎样放电的阳性率高于全面性癫癎儿童组(16/25,64.0%)vs(9/25,36.0%),(P<0.05)。癫癎患儿非快速动眼睡眠各期(S1～S4)癫癎样波放电指数为21.13±19.96,19.59±17.76,22.85±18.99,20.37±16.63,均高于觉醒期的8.20±6.21(P<0.05)。非快速动眼睡眠期S3期癫癎样波放电指数明显高于快速动眼睡眠期(22.85±18.99vs12.91±10.95),(P<0.05)。结论局灶性癫癎儿童发作间期脑电图癫癎样放电的阳性率较全面性癫癎儿童癫癎样放电阳性率高。睡眠,尤其是非快速动眼睡眠期对癫癎儿童癫癎样放电具有易化作用。     

癫癎儿童发作间期癫癎样放电特点初步研究

目的：癫癎发作及癫癎样放电具有昼夜倾向,睡眠是诱发癫癎的重要因素之一,但睡眠触发癫癎的易感性在不同的睡眠时相对发作及发作间期癫癎样放电的影响并不相同。该文主要研究局灶性及全面性癫癎儿童发作间期睡眠和觉醒状态癫癎样放电的特点。方法：采用24 h动态脑电图、多导睡眠脑电图及长程视频脑电图仪描记48例原发性癫癎儿童的全夜睡眠（至少一个完整的睡眠周期）和睡眠前后觉醒状态的脑电资料,统计分析觉醒期及各睡眠期棘/尖波、棘/尖慢波等癫癎样波的放电指数的差异,比较局灶性和全面性发作类型癫癎儿童癫癎样放电阳性率的差异。结果：局灶性癫癎儿童组发作间期脑电图癫癎样放电的阳性率高于全面性癫癎儿童组（16/25,64.0%）vs（9/25,36.0%）,（P<0.05）。癫癎患儿非快速动眼睡眠各期（S1~S4）癫癎样波放电指数为21.13±19.96,19.59±17.76,22.85±18.99,20.37±16.63,均高于觉醒期的8.20±6.21（P<0.05）。非快速动眼睡眠期S3期癫癎样波放电指数明显高于快速动眼睡眠期（22.85±18.99 vs 12.91±10.95）,（P<0.05）。结论：局灶性癫癎儿童发作间期脑电图癫癎样放电的阳性率较全面性癫癎儿童癫癎样放电阳性率高。睡眠,尤其是非快速动眼睡眠期对癫癎儿童癫癎样放电具有易化作用。     

儿童偏头痛与癫癎相关性的研究

目的　探讨儿童偏头痛与癫的相关性及鉴别诊断。方法　对 32例偏头痛患儿 (其中 9例并临床癫发作 )进行脑电图及临床分析。结果　 32例中脑电图异常 1 8例 ,其中 1 4例为偏头痛 ,异常率为43 .75 % ,4例枕叶癫。 3例普通型头痛伴癫发作 ,脑电图 3次复查正常 ,2例复杂型偏头痛伴临床癫发作 ,脑电图均表现异常。结论　儿童偏头痛常常表现脑电图非特异性异常 ,甚至样放电 ,但以脑电图异常不能代替临床诊断。头痛性癫的诊断必须结合临床表现和脑电图检查结果综合判定 ,并与偏头痛进行鉴别。某些偏头痛可以伴癫发作 ,两者交叉重叠 ,极易混淆。对头痛性癫的诊断应慎重     

难治性癫癎治疗新进展

癫癎是以反复发作的神经元异常放电导致中枢神经系统功能失常为特征的疾病,为临床常见病和多发病之一,严重影响患者的日常生活.现就难治性癫癎的治疗方面综述如下. 一、药物治疗传统抗癎药对于大部分癫癎的治疗是有效的,但仍有20%～30%不能有效控制.近年来采用新型抗癎药治疗难治性癫癎,取得较好的临床疗效.常用药物如下:     

良性癫(癎)伴中央颞区棘波患儿的临床及脑电图特点

目的 探讨良性癫(癎)伴中央颞区棘波(BECTS)患儿的临床表现及脑电图特点.方法 对BECTS患儿64例的临床资料进行回顾性分析.诊断明确予抗癫(癎)药物治疗.其中26例治疗后0.5、1.0年复查脑电图,比较治疗前后脑电图异常放电部位、放电频度及波形变化.结果 发病高峰年龄为8～10岁;癫(癎)发作类型为局限性发作(33/64例)、局限性发作泛化全面性发作(21/64例)、强直阵挛性发作(10/64例).发作与睡眠密切相关,多在刚入睡不久(51/64例)或晨起欲醒时(26/64例).癫(癎)样波形为棘波(35/64例)、尖波(20/64例)、二者均有(9/64例).治疗前与治疗后多数(18/26例)脑电图异常放电是变化的,在一侧半球内变化7/26例,在双侧半球内变化6/26例,治疗0.5年时5/26例消失,1年后其中2/5例异常放电又出现.结论 儿童良性限局性癫(癎)与睡眠密切相关,发作形式以局限性发作及局限泛化全面性发作居多,癫(癎)波具有变化性、游走性甚至一过性消失特点.脑电图特征对该病有重要诊断价值.     

难治性癫癎的相关因素分析

目的探讨儿童难治性癫癎(IE)的临床相关因素。方法采用回顾性分析的方法对38例IE患儿(IE组)与40例药物治疗有效的癫癎患儿(对照组)的临床资料(包括起病年龄、发作频率、发作形式、脑电图表现、头颅影像学改变、智能情况、家族史、服药依从性,对首次用药治疗的反应情况等)进行对比分析。结果 IE组1岁前的发病率、发作频率、出现多种发作形式和伴智能障碍率、头颅影像学及脑电图异常率均较对照组增高,差异均有统计学意义(Pa<0.05);IE组首次应用抗癫癎药物治疗反应不良、联合多种药物控制发作及药物治疗依从性差的概率较对照组亦增高,差异均有统计学意义(Pa<0.05)。结论儿童IE与发病年龄小、发作频繁、症状性癫癎或癫癎综合征、多种发作形式共存、伴智能障碍、头颅影像学及脑电图异常改变率高、首次应用抗癫癎药物治疗的反应差、联合多种药物治疗、药物治疗的依从性差等诸多因素有关。     

甲基泼尼松龙冲击治疗Lennox-Gastaut综合征合并睡眠期癫癎性脑电持续状态

目的：LennoxGastaut综合征(LGS)为一种难治性的儿童癫癎综合征,部分患儿合并睡眠期癫癎性电持续状态(electricalstatusepilepticusduringsleep,ESES)增加了治疗的难度。该文观察了大剂量甲基泼尼松龙冲击治疗LGS合并ESES的近期疗效。方法：22例经视频脑电图或动态脑电图证实合并ESES的LGS患儿,给予甲基泼尼松龙冲击治疗以及口服泼尼松维持治疗。冲击治疗后两周评价临床发作并复查脑电图。结果：22例患者中,15例临床发作减少,16例癫癎样放电减少,未见严重不良反应。结论：甲基泼尼松龙冲击治疗可减少LGS患儿发作及脑电图放电,在抗癫癎药物治疗无效时可考虑使用。     

外伤性癫癎患儿脑电图及临床随访分析

目的　探讨小儿外伤性癫的脑电图及临床特征。方法　对我院治疗 15 6例患儿 ,行脑电图及临床追踪观察 ,并做回顾性分析。结果　男童发病率较高 (10 2∶5 4 )。婴幼儿及学龄前儿童发病率较高 (10 2∶5 4 )。脑电图样放电检出率较高。年龄越小 ,早期癫的发病率越高。 15 1例临床发作完全控制 ,余 5例发作次数明显减少。脑电图样放电消失较临床发作控制滞后 6个月～ 2年。结论　脑电图检查对本病的诊断、鉴别诊断和指导治疗有重要意义     

儿童癫癎的诊断和治疗

目的研究癫癎诊断和治疗的现状。方法采用回顾性分析方法,对5年间450例门诊和住院的癫癎患儿资料进行发作类型与年龄的关系、脑电图及药物的疗效分析。结果450例中全面性发作63.1%(284例),其中大发作33.3%(150例);局限性发作16.7%(75例);其他类型发作20.2%(91例)。本组68.2%为6岁以下儿童,其中<3岁占73%。1岁以内发作类型最常见为婴儿痉挛(WS),占该年龄组的41.1%(46/112例)。失神发作见于学龄期儿童,占本组病例的2.4%,Lennox-Gastaut综合征在9岁前各年龄组均可发生,热性惊厥转变为大发作见于学龄期儿童。癫癎发作的诱因主要为发热,有家族史占各种诱因的4.7%。癫癎的病程最长13年。脑电图(EEG)异常率达74.9%,其中普通EEG示癎样放电为50.5%,加做video-EEG或24h动态EEG后阳性率达62.8%。65.7%无影像学异常改变。120例(26.7%)诊断后未治疗或服药后未复诊。330例中单药治疗组总疗效76.5%,联合用药组为55.6%,两者之间疗效存在显著差异(P<0.05),而单药治疗组与WS组疗效比较无显著性差异(P>0.05)。结论癫癎治疗以单药治疗为主,但WS联合用药效果较佳。癫癎治疗滥用药现象较严重,有待规范化治疗。     

癫癎夜间发作患儿癎样放电及睡眠结构特征分析

目的：研究癫癎夜间发作患儿的癎样放电及其睡眠结构特征。方法：选取2009年12月至2011年6月接受夜间12 h视频录像脑电图及整夜多导生理参数睡眠监测仪监测的癫癎夜间发作患儿54例为病例组,对其脑电图癎样放电及睡眠结构进行研究。选取同期年龄、性别与病例组相匹配的正常儿童40例为对照组。结果：（1）病例组患儿均出现癎样放电及次数不等的临床发作,其癎样放电与临床发作均以非快速眼动睡眠期的S1、S2期最多。（2）与对照组比较,病例组睡眠结构中S1期和S2期所占百分比明显增高,而(S3+S4)期和快速眼动睡眠期所占百分比明显降低,差异有统计学意义（P<0.01）。结论：夜间发作性癫癎患儿在非快速眼动睡眠期更易出现癎样放电及临床发作,且伴有明显睡眠结构紊乱。     

早产儿视网膜病的危险因素分析及随访调查

研究早产儿视网膜病(retinopathy of prematurity,ROP)的发生率、高危因素、治疗与随访情况。方法对2005年7月-2007年12月温州医学院附属第一医院NICU收治的符合ROP筛查标准的早产儿,于生后2周开始由资深眼科医师开始行间接眼底镜检查眼底,并进行随访。结果434例早产儿中ROP的发生率为5.5%(24/434例),24例ROP中Ⅰ期19例,Ⅱ期3例,Ⅲ期2例。Ⅲ期阈值病变者行激光光凝治疗,全部患儿均恢复正常。对434例早产儿行单因素分析得出,胎龄、出生体重、住院时间、吸氧、吸氧浓度、吸氧时间、呼吸暂停、新生儿肺透明膜病(RDS)、肺表面活性剂(PS)的应用、机械通气、输血、光疗时间、感染与ROP的发生有相关性(P<0.05)。Logistic回归分析显示胎龄、出生体重、胎数、吸氧时间、光疗时间、代谢性酸中毒、母亲妊高症、颅内出血是影响ROP发生的主要因素。结论早产是ROP的根本原因,防治各种并发症、合理的氧疗是预防ROP的关键。建立完善有效的ROP筛查制度,早期发现、早期治疗ROP,可改善ROP的预后。     

术中判断肠活力三种方法的比较

术中准确地评价肠活力是外科医师面临的一个普遍问题。应用兔肠缺血模型，比较静脉荧光素、表面血氧测定和激光多普勒三种技术判断肠活力的精确性。结果：诊断效率静脉荧光素法为７８％，表面血氧测定为６８％，激光多普勒为９５％。后者精确度显著优于前二者，且操作简便、迅速，具有临床应用前景。     

Spectrum of clinical presentations of vein of Galen aneurysm

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.     

Outcome of the Treatment of Inborn Errors of Metabolism

The use of specific dietary restrictions, cofactor administration, mobilisation of insoluble substances, environmental modifications, product replacement and selective enzyme inhibition are now established for the treatment of some inborn errors of metabolism. There is no generally accepted application for enzyme administration, cytopharmacology (manipulation of the cytoskeleton) or for cell transplantation except for bone marrow transplantation in disorders where the bone marrow is primarily at fault. The other uses of bone marrow transplantation which have been proposed require further evaluation. Results of recent research suggest that the scope of this approach is gradually being widened. There is also scope for development in the field of organ transplantation taking advantage of recent technical¹ and immunological progress. The treatment of inborn errors of metabolism by genetic modification is not yet on a practical clinical level; more laboratory and animal studies are needed before this can be attempted in man. Adenosine deaminase deficiency appears to be the disease in which this will be first attempted using a retroviral vector to insert the gene into the genome of pluripotential bone marrow cells.     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

主动脉缩窄的治疗研究进展

主动脉缩窄(coarctation of aorta,CoA)是主动脉的局限性狭窄,狭窄最常见于峡部,是一种常见的先天性心血管畸形,占所有先天性心脏病的5％ ～8％.缩窄导致上肢血压升高,下肢血压降低,并可引起心功能下降,未经治疗的CoA预后不佳.治疗方式包括外科治疗及经皮介入治疗,不同治疗方式的并发症发生率不同,术后长期监测其并发症、心功能是评估预后的重要指标.该文对主动脉缩窄的治疗方式及术后并发症、心功能情况的研究进展作一综述.     

早产儿脑病研究进展

早产儿脑病是复杂的原发性脑损伤和继发性脑发育异常疾病.过去几年对该病有了较为深刻的认识,该文就最新的有关早产儿脑病概念演变,脑损伤后的细胞分子机制和损伤后发育障碍,尤其是活化小胶质细胞介导的少突胶质细胞损伤机制、神经/髓鞘受损和丘脑、板层下神经元及大脑皮层的受损机制或发育成熟障碍进行综述,以便对该病有更深刻的认识.     

�����뼤���ٴ�Ӧ��ע���

??Inhaled corticosteroids??ICS?? are the most effective medicine for chronic airway inflammation nowdays. Atomization inhalation has been widely applied in clinics because of its efficacy??fewer side-effects and convenience. Here??we focused on some points which should be paid attention to??including how to choose appropriate patients??how to ensure the effectiveness of inhaled steroids and how to reduce possible side-effects.     

Treatment of retinopathy of prematurity

Retinopathy of prematurity is a potentially blinding disorder of premature infants. Retinal ablation of the avascular retina originally described using cryotherapy but now most commonly undertaken with laser photocoagulation, reduces the unfavourable structural outcomes and improves the functional visual acuity outcome. The CRYO-ROP study showed the long-term benefit of treatment of threshold disease compared with no treatment, however even with cryoablation 44.4% of treated eyes had a visual acuity of 6/60 or worse at 10 year follow-up. The ETROP study of earlier treatment for high-risk pre-threshold disease, rather than treatment at threshold, has shown that pre-threshold treatment of type 1 disease produces a significantly improved outcome. Despite treatment some infants develop retinal detachment for which various surgical treatments have been described, although not always with a good functional outcome. Future treatment modalities may include the use of anti-VEGF therapies.     

Epidemiology of respiratory distress of newborns

The present prospective study was conducted to find out the incidence, etiology and outcome of respiratory distress (RD) in newborns. All newborns (n=4505), delivered at this hospital over a period of 13 months, were observed for respiratory problems. Relevant antenatal, intranatal and neonatal information was noted. Cases were investigated for the cause of respiratory distress and followed up for the outcome. The overall incidence of RD was 6.7% Preterm babies had the highest incidence (30.0%) followed by post-term (20.9%) and term babies (4.2%). Transient tachypnea of newborn (TTN) was found to be the commonest (42.7%) cause of RD followed by infection (17.0%), meconium aspiration syndrome (10.7%), hyaline membrane disease (9.3%) and birth asphyxia (3.3%). TTN was found to be common among both term and preterm babies. While Hyaline membrane disease (HMD) was seen mostly among preterms, and Meconium aspiration syndrome (MAS) among term and post-term babies. Overall case fatality ration for RD was found to be 19%, being highest for HMD (57.1%), followed by MAS (21.8%) and infection (15.6%). Our results indicate that RD is a common neonatal problem. TTN accounts for a large proportion of thhese cases. MAS and infection also contribute significantly and are largely preventable. Without adequate ventilatory support HMD and MAS carry high mortality.