关键词： Pediatric   Neurology   Readmission   Predictors   Indicators  

摘要： Background Unplanned readmission is one of many measures of the quality of care of pediatric patients with neurological conditions. In this multicenter study, we searched for novel risk factors of readmission of patients with neurological conditions. Methods We retrieved hospitalization data of patients less than 18 years with one or more neurological conditions. This resulted in a total of 105,834 encounters from 18 hospitals. We included data on patient demographics, prior healthcare resource utilization, neurological conditions, number of other conditions/diagnoses, number of medications, and number of surgical procedures performed. We developed a random intercept logistic regression model using stepwise minimization of Akaike Information Criteria for variable selection. Results The most important neurological conditions associated with unplanned pediatric readmissions include hydrocephalus, inflammatory diseases of the central nervous system, sleep disorders, disease of myoneural junction and muscle, other central nervous system disorder, other spinal cord conditions (such as vascular myelopathies, and cord compression), and nerve, nerve root and plexus disorders. Current and prior healthcare resource utilization variables, number of medications, other diagnoses, and certain inpatient surgical procedures were associated with changes in odds of readmission. The area under the receiver operator characteristic curve (AUROC) on the independent test set is 0.733 (0.722, 0.743). Conclusions Pediatric patients with certain neurological conditions are more likely to be readmitted than others. However, current and prior healthcare resource utilization remain some of the strongest indicators of readmission within this population as in the general pediatric population.

关键词： Kynurenine   Neurotransmitter   Pterin   LC-MS/MS   Serum   CSF  

摘要： Concurrent measurement of tyrosine, tryptophan and their metabolites, and other co-factors could help to diagnose and better understand a wide range of metabolic and neurological disorders. The two metabolic pathways are closely related to each other through co-factors, regulator molecules and enzymes. By using high performance liquid chromatography coupled to electrospray ionization triple quadrupole mass spectrometry, we present a robust, selective and comprehensive method to determine 30 molecules within 20 min using a Waters Atlantis dC18. The method was validated according to the guideline of European Medicines Agency on bioanalytical method validation. Analytical performance met all the EMA requirements and the assay covered the relevant clinical concentrations. Linear correlation coefficients were all > 0.998. Intra-day and inter-day accuracy were between 80-119% and 81-117%, precision 1-19% respectively. The method was applied to measure TYR, TRP and their metabolites, and other neurologically important molecules in human serum and CSF samples. The assay can facilitate the diagnosis and is suitable for determination of reference values in clinical laboratories. (C) 2020 Elsevier B.V. All rights reserved.

关键词： Congenital heart disease   Aortic coarctation   Neurological complications   Stroke   Subarachnoid bleeding   Mortality  

摘要： Background: Patients with coarctation of the aorta (CoA) are at increased risk of periprocedural and late neurological complications. Based on a nationwide inpatient dataset, we assessed the prevalence and risk factors for neurological complications in this cohort. Method and Results: We included all CoA patients hospitalized in England between 1997 and 2015. The risk for procedural complications, subarachnoid bleeding and ischemic stroke were quantified, and risk factors investigated. Overall, 11,907 patients (60% male) with CoA were identified. The median age at first presentation was 0.6 years with a median follow-up period of 14.4 years. Throughout the study period, 8456 surgical or interventional procedures were performed in 6796 patients. The neurological complication rate for primary surgical or interventional repair in patients born after 1997 was 0.05% and 0.2%, respectively. During a total follow-up period of 146,295 patient-years, late neurological complications such as subarachnoid bleeding and cerebral infarction occurred in 225 patients (0.15%/patient-year). The median age for subarachnoid bleeding was 29 years and 57 years for ischemic stroke. While, arterial hypertension (p = .0014), current smoking (p = .02), dyslipidaemia (p = .007) and diabetes (p = .037) were significantly related to the risk of ischemic stroke, only arterial hypertension emerged as significant risk factor for subarachnoid bleeding. Conclusions: Neurological complication during primary CoA repair are extremely rare in the current era. In contrast, many CoA patients are afflicted by late complications such as subarachnoid bleeding and ischemic stroke at a relatively young age, emphasizing the need for meticulous and pro-active blood pressure control and reduction of cardiovascular risk factors. (C) 2020 Published by Elsevier B.V.

关键词： 北京大学出版社   文献计量学   《中文核心期刊要目总览》   核心期刊   神经病学   精神病学   专家评审   中华神经医学杂志  

摘要： 《中华神经医学杂志》主编:我们谨此郑重通知:依据文献计量学的原理和方法,经研究人员对相关文献的检索、统计和分析,以及学科专家评审,贵刊《中华神经医学杂志》入编《中文核心期刊要目总览》2017年版(即第8版)之“神经病学与精神病学”类的核心期刊。该书由北京大学出版社出版。

关键词： 文献计量学   《中文核心期刊要目总览》   核心期刊   神经病学   精神病学   专家评审   原理和方法   《中华老年心脑血管病杂志》  

摘要： 《中华老年心脑血管病杂志》主编:我们谨此郑重通知:依据文献计量学的原理和方法,经研究人员对相关文献的检索、统计和分析,以及学科专家评审,贵刊《中华老年心脑血管病杂志》入编《中文核心期刊要目总览》2017年版(即第8版)之“神经病学与精神病学”类的核心期刊。

关键词： 神经病学   临床思维   四步法   诊断流程   临床技能   教学质量  

摘要： 神经病学具有较高的理论性和逻辑性,涉及内容广泛、病种繁多,学习难度大。如何科学培养临床思维是神经病学临床教学中的重要内容。本科室采用四步法来提升临床思维。第一步树立定位诊断和定性诊断的基本观念,复习基础知识;第二步改变教科书模式,以患者为中心,强调诊断流程,将基础知识对接到临床;第三步在临床工作中注重临床技能培训,进行手把手教学;第四步将理论与技能结合,从典型病例入手,多模式教学,培养临床思维。通过上述策略,以提高神经病学的临床教学质量。

关键词： 神经系统疾病   神经病学   基础应用研究   创新基金项目   健康促进   (中国)   基金会   青年  

摘要： 为进一步推动我国神经病学临床与基础应用研究,提高神经系统疾病诊断和治疗水平,造福广大神经系统疾病患者,由中国健康促进基金会发起、卫材(中国)药业有限公司捐助的神经病学青年创新基金项目于2020年6月正式启动。

关键词： 科学技术协会   办刊宗旨   科研成果   科研人员   神经病学   中国医师协会   学术类期刊   神经疾病  

摘要： 《中国现代神经疾病杂志》为国家新闻出版广电总局和国家科学技术部2001年正式批准,由国家卫生健康委员会主管,中国医师协会、天津市科学技术协会、天津市神经科学学会及天津市环湖医院联合主办,国内外公开发行的神经病学及相关学科的学术类期刊。读者对象主要为广大神经内外科临床医师及相关科研人员。本刊办刊宗旨是:贯彻党和国家有关卫生工作的方针政策,贯彻理论与实践、普及与提高相结合的办刊方针;报道我国神经病学领域的科研成果及临床诊疗经验,促进国内外神经病学的学术交流。

关键词： Kufor-Rakeb   ATP13A2   Nonsense-mediated decay   Iron   NBIA  

摘要： Background: Kufor-Rakeb Syndrome (KRS) is an autosomal recessive disease characterized by Parkinsonism, pyramidal signs, dementia, and supranuclear gaze palsy. KRS is caused by mutations in ATP13A2 producing a transmembrane protein responsible for the regulation of intracellular inorganic cations. Objective: Two siblings born to a Turkish family of consanguineous marriage had mixed neurological presentations with the presence of hypointense images on T2-weighted MRI and were pre-diagnosed as having autosomal recessive spastic paraparesis or ataxia. We aimed to identify the disease-causing mutation by wholeexome sequencing and elucidate the underlying molecular mechanism of the causative mutation. Methods: Prussian blue staining was conducted for the detection of cellular iron accumulation. Disease-causing mutation in ATP13A2 was detected by whole-exome sequencing. Expression levels of ATP13A2 mRNA and protein were assessed by qRT-PCR and Western Blot. Results: Iron deposits in the patients' fibroblasts were detected by Prussian blue staining. Novel homozygous mutation c.1422_1423del:p.P474fs was detected in the ATP13A2. As this mutation caused a premature termination codon (PTC), the expression of mutant ATP13A2 mRNA through qRT-PCR analysis was found to be degraded by nonsense-mediated decay and this prevented the expression of ATP13A2 protein in the patients' fibroblasts. Conclusions: Novel frameshift mutation causing a PTC in ATP13A2 lead to degradation of ATP13A2 mRNA by NMD. Iron accumulation due to the absence of ATP13A2 protein in the patient's fibroblasts and hypointense areas on T2-weighted images may expand the spectrum of KRS to consider it as neurodegeneration with brain iron accumulation disorders.

关键词： 中华医学会   中华神经科杂志   防治指南   神经病学   中国脑血管病   脑血管病学   重庆市  

摘要： 由中华医学会神经病学分会、中华医学会神经病学分会脑血管病学组和中华医学会中华神经科杂志编辑委员会组织的中国脑血管病防治指南巡讲,于2020年11月21日在重庆市举行。主要来自重庆市的近90名代表现场参加了此次指南巡讲。