目的 探讨热性惊厥患儿血清电解质和血糖的变化及其临床意义。 方法 选取2009 年6月－2010 年12月儿科住院的呼吸道感染并发热性惊厥患儿38例和呼吸道感染无惊厥患儿42例，分别作为观察组和对照组，测定和比较两组患儿血清电解质和血糖值。 结果 观察组血清钠离子浓度为（133.05 ± 1.74）mmol/L、氯离子浓度为（100.37 ± 1.79）mmol/L；对照组血清钠离子浓度为（142.19 ± 1.85）mmol/L、氯离子浓度为（104.57 ± 1.55）mmol/L，差异均有统计学意义（P＜0.01）；观察组和对照组血糖浓度依次为（6.93 ± 0.87）、（5.12 ± 0.55）mmol/L，差异有统计学意义（P＜0.01）。观察组在治疗后的血清钠离子、氯离子浓度分别为（140.89 ± 2.68）、（103.29 ± 1.94）mmol/L，均高于发生惊厥时的浓度（P＜0.01）；观察组在治疗后的血糖浓度为（5.31 ± 0.68）mmol/L，明显低于发生惊厥时，差异有统计学意义（P＜0.01）。 结论 婴幼儿发生热性惊厥时存在血钠、血氯水平降低和血糖升高，在热性惊厥患儿的治疗中应纠正血钠水平和高血糖。Objective To explore the clinical significance of the changes in serum electrolytes and blood glucose in the children with febrile convulsion. Methods Thirty-eight children with respiratory infection combined with febrile convulsion and 42 children with single respiratory infection diagnosed between June 2009 and December 2010 were selected as the observation group and control group, respectively. Serum electrolytes and blood glucose concentration were assayed and compared between the two groups. Results The concentrations of serum sodium and chloride were (133.05 ± 1.74) mmol/L and (100.37 ± 1.79) mmol/L in the observation group, while (142.19 ± 1.85) and (104.57 ± 1.55) mmol/L in the control group; the differences between the two groups were significant (Plt;0.01). The concentrations of blood glucose were (6.93 ± 0.87) mmol/L in the observation group and (5.12 ± 0.55)mmol/L in the control group; the difference was significant (Plt;0.01). After the treatment, the serum concentrations of sodium and chloride were (140.89 ± 2.68) and (103.29 ± 1.94)mmol/L in the observation group, which were higher than those before treatment (Plt;0.01). After treatment, the blood glucose concentration was (5.31 ± 0.68）mmol/L in the observation group, which was lower than that before the treatment (Plt;0.01). Conclusion Hyponatremia, low serum chlorine and hyperglycemia occurre in the febrile convulsion in children, which should be corrected in the treatment of febrile convulsion.
ObjectiveUsing Quality in prognosis studies (QUIPS) analysis, this paper systematically reviewed the factors influencing the poor outcome of children with convulsive status epilepticus (CSE).MethodsTo longitudinal cohort studies on the prognostic evaluation of CSE mortality and mobidity in children.The retrieval time was from January 2008 to November 2019, and three system reviewers PUBMED, EMBASE, COCHRANE and other databases were used to search for literatures related carried out literature extraction and quality evaluation. According to the QUIPS analysis method, the included literatures were scored, the quality grade was divided, and the analysis variables of medium/high quality literatures with statistical significance were selected to draw a conclusion.ResultsQUIPS analysis was used to assess the literature quality, 17 medium/high quality literatures were included, and the factors with statistical significance (P<0.05) mentioned at least twice or more in≥2 medium/high quality literatures were selected, which were considered as important risk factors affecting prognosis.These factors include: etiology, age, duration of convulsion, refractory CSE, neuroimaging abnormalities.ConclusionFive risk factors indicating poor outcome of CSE in children were summarized. Due to the heterogeneity of various literature studies, Meta-analysis has not been completed, so it has certain limitations.
ObjectiveTo analyze the clinical characteristics and corresponding genetic features of epilepsy related to fever sensitivity. MethodsRetrospectively review 29 children with epilepsy related to fever sensitivity who were diagnosed and treated in the Department of Pediatric Neurology of the Third Affiliated Hospital of Zhengzhou University from January 2017 to December 2022, with complete clinical data and underwent molecular genetic testing. Fill in the clinical data registration form in detail, and retrospectively summarize their clinical characteristics, electroencephalogram (EEG) manifestations, neuroimaging examinations, the selection of antiepileptic drugs, curative effects, and evaluate and follow up the developmental indicators. ResultsAmong the 29 children with epilepsy related to fever sensitivity, there were 13 males (44.8%) and 16 females (55.2%); 10 cases (34.5%) were Dravet syndrome, 3 cases (10.3%) were genetic epilepsy with febrile seizures plus (GEFS+), and 1 case (3.4%) was PCDH19 gene-related epilepsy. The age of onset ranged from 2 to 25 months. Among them, 19 cases (65.5%) had an onset age of 2 to 12 months, and 10 cases (34.5%) had an onset age greater than 12 months. In 1 case of GEFS+ child, all seizures occurred after fever, and in the other 28 children, afebrile seizures were present. The interval between the first febrile seizure and the appearance of afebrile seizures was 0.09 to 116 months; the age of appearance of afebrile seizures was 5 to 134 months. There were 6 cases (20.7%) with a single seizure type, and 23 cases (79.3%) with 2 or more seizure types. There were 24 cases (82.8%) with generalized tonic-clonic seizures, 9 cases (31.0%) with generalized tonic seizures, 18 cases (62.1%) with focal seizures, 4 cases (13.8%) with absence seizures, and 1 case (3.4%) with spasm seizures. 10 cases (34.5%) of children had status epilepticus, and 13 cases (44.8%) had cluster seizures. 16 cases (55.2%) of children had a positive family history, among which 8 cases (27.6%) had a family history of febrile seizures, and 11 cases (37.9%) had a family history of afebrile seizures/epilepsy; during the initial visit and follow-up, 22 cases (75.9%) were found to have developmental delays of varying degrees. Pathogenic/suspected pathogenic gene variants/copy number variants clearly related to epilepsy were detected in 20 cases, with a detection rate of 68.9%, including SCN1A gene variants (11 cases), GABRB2 gene variants (1 case), GABRG2 gene variants (1 case), PCDH19 gene variants (1 case), SPTBN1 gene variants (c.1081_c.1097delAACTTGGAAGTGCTGCTinsCA, 1 case), ASNS gene variants (c.146G>A, 1 case), copy number variants in the 4p16.3 region (3 cases), and copy number variants in the 16p11.2 region (1 case). Among them, the gene variants of SPTBN1 and ASNS are novel gene variants that have not been previously reported in China for epilepsy related to fever sensitivity. ConclusionEpilepsy related to fever sensitivity mostly occurs in infancy, with diverse seizure patterns, varying degrees of severity of clinical symptoms, often accompanied by status epilepticus and cluster seizures, and mostly combined with developmental delays of varying degrees. This study found that the gene variants of SPTBN1 and ASNS, which have not been previously reported in China, may be rare pathogenic genes for epilepsy related to fever sensitivity.
目的 研究利多卡因对海马的神经毒性是否会对大鼠空间学习记忆能力产生影响，并探讨大鼠空间学习能力的变化与海马CA3区锥体细胞数目的相关性。 方法 将成年Wistar雄性大鼠随机分为基础值组（n=7）和利多卡因惊厥组（n=40）。基础值组大鼠静脉给予生理盐水后使用Y迷宫测定大鼠的空间学习能力。利多卡因惊厥组大鼠尾静脉持续输注利多卡因造成惊厥，待大鼠恢复正常运动以后放入鼠笼重新饲养。并于惊厥后第1、3、5、7天从中随机抓取大鼠测试其空间学习能力以及组织学改变。根据对应天数将利多卡因惊厥组的40只大鼠随机细分为Day-1、Day-3、Day-5、Day-7亚组，每亚组10只。所有大鼠在测定空间学习能力之后立即处死，取出大脑并做石蜡包埋，冠状面切片后进行组织学检测，显微镜下评估海马CA3区锥体细胞状态。 结果 ① 基础值组和Day-1、Day-3、Day-5、Day-7亚组大鼠的Y迷宫穿梭次数分别为（25.2 ± 3.7）、（27.1 ± 8.1）、（36.9 ± 9.9）、（38.7 ± 10.6）、（40.6 ± 16.3）次，除Day-1亚组与基础值组比较差异无统计学意义（P＞0.05）外，其余各亚组与基础值组差异均有统计学意义（P＜0.05）；② 与基础值组单位面积（10.3 ± 4.5）个（异常锥体）细胞比较，利多卡因惊厥组大鼠海马CA3区异常锥体细胞数增加，Day-1、Day-3、Day-5、Day-7亚组计数值分别为13.0 ± 7.2、15.6 ± 5.0、19.6 ± 8.1、18.1 ± 5.1，且与大鼠Y迷宫穿梭次数呈正相关（r=0.711，P＜0.05）。 结论 利多卡因引起的惊厥使成年大鼠海马依赖性空间学习能力下降，利多卡因的神经毒性引起的海马异常锥体细胞增多可能是造成这一现象的一种原因。
Objective To investigate the diagnosis and treatment of status epilepticus in hospitals of different levels and the knowledge of status epilepticus in clinical physicians, in order to better guide clinical education in the future. Methods From August 2014 to August 2015, a questionnaire was designed and used to investigate the general situation of the hospital, the diagnosis of status epilepticus and the clinical practice among trainee doctors and students in the epilepsy training class in the Neurological Intensive Care Unit and the Department of Neurology of West China Hospital, Sichuan University. The results of the investigation were statistically analyzed. Results Ninety questionnaires were distributed, and all the questionnaires were retrieved with validity. The number of investigated physicians was 42 (46.7%) from the Department of Neurology, 6 (6.7%) from the Department of Neurosurgery, 30 (33.3%) from the Intensive Care Unit and 12 (13.3%) from other departments. Twenty-seven (30.0%) physicians were from class Ⅲ grade A hospitals, 31 (34.4%) from class Ⅲ grade B hospitals, and 32 (35.6%) from class Ⅱ grade A hospitals. All the class Ⅲ hospitals and 53.1% of class Ⅱ hospitals had electroencephalograph monitoring facilities. The proportion of status epilepticus patients ranged from 0.5% to 10.0% in different hospitals. There were great differences in the identification and treatment of convulsive status epilepticus among different hospitals. Conclusions Status epilepticus is a common emergency. Questionnaire survey is an effective means to reflect the difference in identifying and treating the emergency among different departments and hospitals. It can guide clinical education and promote the identification and treatment of the emergency more accurately in doctors of all levels.