50例新生儿缺血缺氧性脑病临床及CT分析

缺血缺氧性脑病（HIE）是围产期新生儿最常见的中枢神经系统病变，不仅严重威胁新生儿的生命健康，而且是伤残儿童最常见的病因之一．我院儿科于1988年-1994年6月共收治新生地缺血缺氧性脑病50例，其中25例做了脑脊液检查，全部病例做了头颅CT检查·现就其临床及CT结果分析报告如下。资料与方法一、HIE诊断标准[1-5]：（1）有宫内或生后窒息（Arrar评分1分钟wt3分·5分钟wt5分）或有引起缺血缺氧性的严重原发病；（2）有意识障碍及肌张力改变持续在生后24小时以上；（3）有惊厥或呼呗节律不整及脑水肿表现；（4）CT呈现2个区域（脑叶…     

新生儿缺血缺氧性脑病产科病因分析

目的 分析新生儿缺血缺氧性脑病的产科病因.方法 选取2012年1 月～2014年12月在本院出生的60例患有缺血缺氧性脑病新生儿为观察对象,其中有30例新生儿进行产科产前干预,30例新生儿未进行产科产前干预,另以同时期60例健康新生儿为参照对象.比较缺血缺氧性脑病新生儿而与健康新生儿、未行产科产前干预和行产科产前干预的缺血缺氧性脑病新生儿相关致病因素.结果 缺血缺氧性脑病新生儿而与健康新生儿在羊水污染、胎盘异常、产程异常、脐带异常、妊高症方面所占比重的差异有统计学意义,P＜0.05.未行产科产前干预和行产科产前干预的缺血缺氧性脑病新生儿在Apgar评分、脐血流、脉搏血氧饱和度、生物物理评分等方面的差异有统计学意义,P＜0.05.结论 羊水污染、胎盘异常、产程异常、妊高症等是新生儿缺血缺氧性脑病的相关致病因素,Apgar评分＜7、脐血流＞3、脉搏血氧饱和度≤30％、生物物理评分≤5等是新生儿缺血缺氧性脑病的产科致病因素.     

新生儿缺血缺氧性脑病的护理

目的 总结37例新生儿缺血缺氧性脑病(HIE)的护理体会,以提高临床治愈率。方法 分析37例HIE患儿的临床资料。结果 本组患儿治愈率达91.9%。结论 指出保持呼吸道通畅、有效吸氧;维持全身各器官良好灌注和血糖水平;积极控制颅内高压及惊厥;加强基础护理及心理护理等是提高HIE患儿治愈率的关键。     

新生儿缺血缺氧性脑病危险因素的logistic回归分析

目的分析新生儿缺血缺氧性脑病危险因素。方法采用回顾性研究方法,对202例新生儿缺血缺氧性脑病的相关因素进行logistic回归分析。结果危险因素按OR值排序,依次为5m in Apgar评分<4分、1m in Apgar评分<4分、宫内窘迫、新生儿肺透明膜病、颅内出血、产程延长。结论产科因素或围生因素是缺血缺氧性脑病发生的主要原因,加强围生期保健,提高产科技术,提高新生儿窒息复苏技术。对于降低新生儿缺血缺氧性脑病的发生率有重要意义。     

新生儿缺血缺氧性脑病免疫功能研究

目的对新生儿缺血缺氧性脑病免疫功能进行探讨,为其治疗提供理论依据.方法 IL-2、sIL-2R、T淋巴细胞亚群、免疫球蛋白和补体C3检测,分别采用E LISA、APAAP、单向免疫扩散试验和火箭电泳法进行.结果 HIE患儿的IgM和C3补体的浓度 ,CD3和CD4的百分率和IL-2水平与对照组比较,明显降低(P＜0.01),而sIL-2R 水平明显升高,表明HIE患儿存在明显的体液和细胞免疫功能异常.结论 HIE患儿免疫功能紊乱 ,易继发感染.为加强隔离消毒和防治感染提供理论的依据.     

高压氧改善围产期小鼠缺氧性脑病病理变化

临床用高压氧对新生儿缺氧性脑病进行早期治疗,效率高,疗效好,但治疗机理尚不太清楚,本文通过高压氧治疗围产期鼠缺氧性脑病,用免疫组织化学等方法探讨如下。     

新生儿缺血缺氧性脑病治疗前后脑干听觉诱发电位对比

本室对近两年来32例被确诊为新生儿缺血缺氧性脑病（HIE）的新生儿分别在急性期、治疗2周后检测脑干听觉诱发电位（BAEP），比较检测结果，现报告如下。     

新生儿缺血缺氧性脑病的脑电地形图观察

目的：观察新生儿缺血缺氧性脑病(HIE)早期脑电地形图(BEAM)的变化及其意义。方法：应用常规脑电地形图对65例HIE进行了检查。结果：63例患(96％)出现BEAM异常，并且BEAM以背景活动异常为主。结论：BEAM为HIE早期观察脑功能变化的敏感指标。     

血清生物标志物在缺血缺氧性脑病新生儿中的研究进展

治疗性低温疗法作为低氧缺血性脑病(hypoxic ischemic encephalopathy,HIE)治疗标准的广泛引入,给临床医生带来了越来越大的压力,要求他们对发生的低氧损伤(hypoxic injury,HI)的程度和随之而来的脑病的严重程度做出早期和准确的评估.然而,目前还没有任何一种基于血液的标志物足以检测HI或预测预后.许多炎症蛋白、神经元特异性蛋白和MicroRNA表达可预测HIE病情变化,这些变化在出生的几小时至几天内迅速演变.将临床数据与生化检测结果相结合是目前改善新生儿HIE的检测和预测结局的最可能途径.本文总结了目前对HIE血清生物标志物的研究,显示了其预测HIE预后的潜力.     

缺血缺氧性脑病对新生儿外周血髓鞘碱性蛋白水平变化的Meta分析

目的:系统分析髓鞘碱性蛋白(MBP)在不同程度缺血缺氧性脑病(HIE)新生儿血清水平变化。方法:计算机检索中国知网、万方数据、PubMed、Embase、Web of Science和Cochrane Library等数据库,从建库至2016-11-25收录的有关HIE新生儿MBP血清水平变化的研究。对符合纳入和排除标准的随机或半随机对照研究进行数据提取和质量评估后,用RevMan5.3软件对文献数据进行Meta分析。结果:共检索文献156篇,最终纳入6篇。6篇文献结果数据均完整,对结局指标均有报道,不存在选择性结果报道,其它潜在偏倚风险不确定。Meta分析显示,与对照组相比,轻度、中度和重度HIE均可引起新生儿外周血MBP水平升高,且重度中度轻度。轻度HIE新生儿MBP水平合并标准化均数差(SMD)=1.82(95%CI:0.65-2.99,P0.01),中度HIE新生儿MBP水平SMD=4.02(95%CI:1.55-6.49,P0.01),重度HIE新生儿MBP水平SMD=6.06(95%CI:3.35-8.78,P0.01)。中度与轻度HIE相比,MBP水平SMD=2.46(95%CI:0.71-4.22,P0.01);重度与轻度HIE相比,MBP水平SMD=4.85(95%CI:2.59-7.10,P0.01);重度与中度HIE相比,MBP水平SMD=2.94(95%CI:1.21-4.67,P0.01)。纳入6项研究存在明显发表偏倚。结论:不同程度HIE新生儿外周血MBP水平升高可为其临床诊断提供帮助。     

伴发脑病的成人全面性癫癎电一临床分析

目的对伴发脑病的全面性癫癎成年病人作电生理学与临床症状学分析。方法：入选标准：①18岁以上明确诊断全面性癫癎的患者;②行简易智能精神状态检查量表（MMSE）及日常生活活动能力量表（ADL）检查明确有精神发育迟滞及不同程度残障的癫癎患者,进行了长时间视频脑电图＋肌电多导仪（以下简称长时间V-EEG＋EMG多导仪）监测,记录间歇期EEG及发作时同步V—EEG＋EMG。结果：符合条件的6例患者共记录到64次临床发作：轴肌强直发作19次,短暂性强直发作8次,不典型失神发作1次,全面性强直阵挛发作2次,全面性强直阵挛发作2次,轴肌肌阵挛发作20次,眼睑肌阵挛10次,右侧上肢肌阵挛发作1次,无法分类的发作1次。6例患者中有5例为中度,1例为重度精神发育迟滞。结论：年龄依赖性癫癎性脑病患者发病早期的诊断及癫癎发作分型并进行长期的电生理学临床症状学随访到成年是必要的。     

Prognostic assessment in patients with hepatic encephalopathy

Hepatic encephalopathy (HE) is a common complication of liver failure that is associated with poor prognosis. However, the prognosis is not uniform and depends on the underlying liver disease. Acute liver failure is an uncommon cause of HE that carries bad prognosis but is potentially reversible. There are several prognostic systems that have been specifically developed for selecting patients for liver transplantation. In patients with cirrhosis the prognosis of the episode of HE is usually dictated by the underlying precipitating factor. Acute-on-chronic liver failure is the most severe form of decompensation of cirrhosis, the prognosis depends on the number of associated organ failures. Patients with cirrhosis that have experienced an episode of HE should be considered candidates for liver transplant. The selection depends on the underlying liver function assessed by the Model for End-stage Liver Disease (MELD) index. There is a subgroup that exhibits low MELD and recurrent HE, usually due to the coexistence of large portosystemic shunts. The recurrence of HE is more common in patients that develop progressive deterioration of liver function and hyponatremia. The bouts of HE may cause sequels that have been shown to persist after liver transplant.     

Hashimoto encephalopathy with fulminant myocarditis

There are very limited data concerning the association of lymphocytic thyroiditis with cardiomyopathy, and there is only one published report of fatal cardiomyopathy associated with autoimmune thyroiditis. An association of Hashimoto encephalopathy (HE) with autoimmune myocarditis has not yet been reported. Here we describe a case of a 31-year-old man with autoimmune thyroiditis complicated by HE, who succumbed to autoimmune myocarditis. This association raises the possibility that HE and myocarditis may share a common pathogenetic mechanism in some cases.     

一氧化碳中毒后迟发性脑病患者的智能与脑电图分析

目的：观察一氧化碳中毒后迟发性脑病(DEACMP)患者的智力与EEG恢复的关系及其在临床上的指导意义。方法：对66例DEACMP患者定期进行长谷川痴呆量表、常识记忆注意测验、日常生活能力量表和中国修订韦氏成人智力量表、EEG等检测。结果：急性期DEACMP患者的智力与EEG的异常表现一致，均为100％异常，而恢复期EEG较智力恢复明显快。结论：智能及EEG检测对临床诊治DEACMP有较大的指导价值，恢复期智力检测对判断患者恢复情况意义更大，长期坚持治疗及锻炼均能不同程度地恢复患者的智能。     

Brain mapping analysis in patients with hepatic encephalopathy

Summary Topographical analysis of cerebral electrical activity was performed in 44 patients with hepatic encephalopathy. These patients were classified in 5 groups according to clinical criteria. Eight healthy subjects were used as a control group. All were studied in an awake, eyes closed, condition and some [Control Group (CG), Group 0 (G0), Group 1 (G1) and Group 2 (G2)] also in an awake, eyes open, condition. The awake, eyes closed, maps showed marked differences in the power spectral density (PSD) of the different bands, when comparing normal subjects with patients with several degrees of hepatic encephalopathy. These differences were related to the degree of clinical involvement, mainly in the alpha and delta PSD bands. The combination of a decreased alpha PSD, increased delta PSD, and decreased mean dominant frequency (MDF) allowed a clear discrimination between the different clinical groups. The differences observed between awake, eyes closed, and awake, eyes open, conditions were especially helpful to discriminate between CG subjects and G0, G1 and G2 patients.     

De novo variants in RHOBTB2, an atypical Rho GTPase gene,cause epileptic encephalopathy

By whole exome sequencing, we identified three de novo RHOBTB2 variants in three patients with epileptic encephalopathies (EEs). Interestingly, all three patients showed acute encephalopathy (febrile status epilepticus), with magnetic resonance imaging revealing hemisphere swelling or reduced diffusion in various brain regions. RHOBTB2 encodes Rho‐related BTB domain‐containing protein 2, an atypical Rho GTPase that is a substrate‐specific adaptor or itself is a substrate for the Cullin‐3 (CUL3)‐based ubiquitin ligase complex. Transient expression experiments in Neuro‐2a cells revealed that mutant RHOBTB2 was more abundant than wild‐type RHOBTB2. Coexpression of CUL3 with RHOBTB2 decreased the level of wild‐type RHOBTB2 but not the level of any of the three mutants, indicating impaired CUL3 complex‐dependent degradation of the three mutants. These data indicate that RHOBTB2 variants are a rare genetic cause of EEs, in which acute encephalopathy might be a characteristic feature, and that precise regulation of RHOBTB2 levels is essential for normal brain function.