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Quality of Life Dilemmas in Pediatric Patients with Epilepsy Residing in Long-Term Care Facilities 长期护理机构儿童癫痫患者的生活质量困境
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-22 DOI: 10.1055/s-0040-1714356
L. Tran
Abstract Children with chronic care or complex medical needs, such as epilepsy, depend on advocates to ensure their safety and well-being, a role typically filled by parents or extended family. Close participation to provide vital history and details is quintessential to help guide appropriate management decisions to ensure optimal neurodevelopmental outcome. However, when these vulnerable children are placed in the foster care system, their need for an advocate becomes even more paramount. Unfortunately, this can be lacking and can lead to a breakdown in their medical care. Their complex medical conditions often can be intimidating to most potential foster families and may result in placement in long-term care facilities. This, in turn, presents additional obstacles that can hinder optimal care such as lack of consistent, dedicated caretakers. This void not only impacts their outcome and medical care but also leads to excessive use of limited healthcare resources as well as unwanted adverse reactions that can be prevented if a reliable source of history is available. As a result, there is a need for dedicated resources to help provide devoted advocates for these vulnerable children.
患有慢性护理或复杂医疗需求(如癫痫)的儿童依靠倡导者来确保他们的安全和福祉,这一角色通常由父母或大家庭承担。密切参与提供重要的历史和细节是帮助指导适当的管理决策,以确保最佳的神经发育结果的精髓。然而,当这些弱势儿童被安置在寄养系统中时,他们对倡导者的需求变得更加重要。不幸的是,这可能是缺乏的,并可能导致他们的医疗崩溃。他们复杂的医疗状况往往会对大多数潜在的寄养家庭造成威胁,并可能导致他们被安置在长期护理机构。这反过来又带来了额外的障碍,如缺乏一致的、专门的护理人员,可能会阻碍最佳护理。这种空白不仅会影响他们的治疗结果和医疗护理,还会导致过度使用有限的医疗资源,以及如果有可靠的病史来源就可以预防的不必要的不良反应。因此,需要专门的资源来帮助为这些弱势儿童提供专门的倡导者。
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引用次数: 0
Repetitive Arm and Hand Movements (Complex Motor Stereotypy) in a Six-Year Boy 六岁男孩重复性手臂和手部动作(复杂运动刻板印象)
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-21 DOI: 10.1055/s-0040-1712162
R. Koul, R. Hora
Abstract Abnormal movements are not uncommon in children. Most of these abnormal movements can be defined by the available criteria. Abnormal movements are easily diagnosed in adults. Most of these abnormal movements are well categorized and diagnosed by the defined characteristics. Sometimes it is difficult to diagnose an unusual movement disorder in children, particularly if it is rare and there are no investigations to prove the diagnosis. Repetitive arm and hand movements (complex motor stereotypy) is one such disorder which is rare and difficult to diagnose unless seen before. In this case report, we described a 6-year-old boy with this disorder, in whom it took 6 years to diagnose.
异常运动在儿童中并不少见。大多数这些异常运动都可以用现有的标准来定义。成人的异常运动很容易被诊断出来。这些异常运动大多可以很好地分类并根据定义的特征进行诊断。有时很难诊断儿童的异常运动障碍,特别是如果它很罕见并且没有调查来证明诊断。重复的手臂和手部运动(复杂运动刻板印象)就是这样一种罕见且难以诊断的疾病,除非以前见过。在这个病例报告中,我们描述了一个患有这种疾病的6岁男孩,他花了6年的时间才诊断出来。
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引用次数: 0
Exploring the Role of Pediatric Neurology in Determinations of Futility, Identifying Vulnerability, and Participating in Shared Decision Making through Case-Based Analysis 通过案例分析,探索儿童神经病学在确定无效、识别脆弱性和参与共同决策中的作用
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-20 DOI: 10.1055/s-0040-1714354
Julio Quezada, J. Lantos
Abstract It is not uncommon for pediatric neurologists to encounter ethical dilemmas in clinical practice. These dilemmas not only require an exploration of the basic bioethical principles, but also considering that neurological disease can have impact on a person's self-identity. Patients with neurological disease are particularly vulnerable and at risk of having long-term sequelae of their nervous system and their individual selves. These important considerations can then raise the question of futility of treatment in a setting of uncertainty of outcome. In these complex situations, a pediatric neurologist plays an important role in shared decision making with both families and multidisciplinary teams. In this paper, we explore case-based scenarios that serve to demonstrate the unique character of ethics in neurology and the role of pediatric neurologists in finding solutions that are in a patient's best interest, have a minimal chance of harm, respect a patient's autonomy, and are just and promote justice.
摘要小儿神经科医师在临床实践中遇到伦理困境并不罕见。这些困境不仅需要探索基本的生物伦理原则,还需要考虑到神经系统疾病可能对一个人的自我认同产生影响。患有神经系统疾病的患者尤其容易受到伤害,他们的神经系统和个人自我都有长期后遗症的风险。这些重要的考虑可能会在结果不确定的情况下提出治疗无效的问题。在这些复杂的情况下,儿科神经科医生在与家庭和多学科团队共同决策方面发挥着重要作用。在本文中,我们探讨了基于案例的场景,以展示神经病学伦理的独特特征,以及儿科神经学家在寻找符合患者最佳利益的解决方案方面的作用,伤害的可能性最小,尊重患者的自主权,公正和促进正义。
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引用次数: 0
Social and Economic Challenges to Implementing the Ketogenic Diet: A Case Series 实施生酮饮食的社会和经济挑战:一个案例系列
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-15 DOI: 10.1055/s-0040-1713908
Sloan Lynch, Catherine N. Barry, L. Douglass
Abstract The ketogenic diet (KD) is a powerful therapeutic tool that can reduce seizure activity in patients with refractory epilepsy. However, dietary implementation can be difficult for patients and families due to the time and resource intensive nature of the diet. These challenges are particularly pronounced in economically disadvantaged or socially unstable patient populations. The first case of this series describes the successful implementation of KD in a low-income, homeless patient through the use of innovative KD recipes and access to social programs offered through Boston Medical Center. The second case illustrates the importance of consistent parental support to the success of KD and describes mechanisms used to improve familial support within a nonunified household. Collectively, these cases demonstrate ways by which health care providers can make this powerful dietary treatment accessible to low-resource patients.
生酮饮食(KD)是一种有效的治疗工具,可以减少难治性癫痫患者的癫痫发作活动。然而,由于饮食的时间和资源密集性,饮食的实施对患者和家属来说可能很困难。这些挑战在经济上处于不利地位或社会不稳定的患者群体中尤为明显。本系列的第一个案例描述了通过使用创新的KD食谱和波士顿医疗中心提供的社会项目,在一个低收入、无家可归的病人中成功实施KD。第二个案例说明了持续的父母支持对KD成功的重要性,并描述了在非统一家庭中用于改善家庭支持的机制。总的来说,这些案例表明,卫生保健提供者可以通过各种方式使这种强大的饮食治疗对资源不足的患者有效。
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引用次数: 0
Ethical, Social, and Economic Challenges in Managing a Child with Dravet Syndrome in a Developing Country 在一个发展中国家,管理患特拉韦特综合症儿童的伦理、社会和经济挑战
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-15 DOI: 10.1055/s-0040-1714065
S. Gulati, R. Sinha, Priyanka Madaan
Abstract Epilepsy is a chronic neurological disorder with a significant impact on sociocultural and economic aspects. The management of epilepsy in low-middle income countries (LMICs) is influenced by many factors such as disease burden, poverty, educational status, the reflection of the disease as a social stigma, diverse religious beliefs, and treatment expense. Despite the improvement in educational and social parameters, the stigmatization of the disease is still evident in many LMICs. The associated comorbidities and neurodevelopmental disorders further add to the cost and stigmatization. The pediatric neurologists/epileptologists in LMICs are encountered by distinctive ethical, social, and economic dilemmas during the patient care and management of epilepsy. This article discusses the various ethical dilemmas in a child with Dravet syndrome in a developing country.
癫痫是一种慢性神经系统疾病,对社会文化和经济产生重大影响。中低收入国家的癫痫管理受到许多因素的影响,如疾病负担、贫困、教育状况、该病作为一种社会耻辱的反映、不同的宗教信仰和治疗费用。尽管教育和社会条件有所改善,但在许多中低收入国家,对这种疾病的污名化仍然很明显。相关的合并症和神经发育障碍进一步增加了成本和污名化。中低收入国家的儿科神经科医生/癫痫学家在对癫痫患者的护理和管理过程中遇到了独特的伦理、社会和经济困境。这篇文章讨论了发展中国家患德拉韦特综合症儿童的各种伦理困境。
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引用次数: 2
Electrocardiography Parameters Changes in Epilepsy and Febrile Convulsion Children Compared with Controls 癫痫和热性惊厥患儿与对照组比较的心电图参数变化
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-07-08 DOI: 10.1055/s-0040-1713907
N. Noori, Alireza Teimouri, A. Khajeh
Abstract Epilepsy and febrile convulsion are the most common neurological diseases with significant effect on cardiac functions. This study aimed to evaluate electrocardiography parameters alterations in epilepsy and febrile convulsion children compared with controls. In this comparison study, 270 children analyzed who shared equally in epilepsy, febrile convulsion, and healthy that aged from 0.5 to 5 years. The participants were collected from Ali ibn Abi Talib Hospital of Zahedan, Iran. Epilepsy confirmed based on definition of having at least two unprovoked seizures in 24 hours. Febrile convulsion was confirmed based on its definition by the International League against Epilepsy. Healthy children selected from those referred to the hospital with fever and without any underline diseases. Electrocardiography was performed by a pediatric cardiologist. Data were analyzed using SPSS 19 with p < 0.05 significant level. Heart rate was higher in epilepsy (129.64 ± 27.63) compared with control (108.78 ± 26.01) and febrile convulsion (125.79 ± 25.38; X 2 = 28.701, p < 0.001). S wave in lead V1 was higher in controls (0.72 ± 0.36) compared with epilepsy (0.58 ± 0.45) and febrile convulsion (0.58 ± 0.36). QT dispersion and QTc dispersion levels were higher in epilepsy than febrile convulsion children that both were higher than controls. Concluded that R in aVL, LV mass (LVM), QT dispersion, and QTc dispersion were higher significantly in epilepsy compared with febrile convulsion children. To maintain a good strategic treatment in patients with epilepsy and febrile convulsion, there is a need to assess alternations in ECG parameters, especially QT interval changes that lead to better comprehensive autonomic changes.
癫痫和热性惊厥是最常见的神经系统疾病,对心功能有显著影响。本研究旨在评估癫痫和热性惊厥患儿与对照组相比的心电图参数改变。在这项比较研究中,分析了270名年龄在0.5至5岁之间的儿童,他们在癫痫、热性惊厥和健康方面具有相同的特征。参与者来自伊朗扎黑丹的Ali ibn Abi Talib医院。根据24小时内至少有两次非诱发性癫痫发作的定义确诊为癫痫。热性惊厥是根据国际抗癫痫联盟的定义确定的。从那些转诊到医院的有发烧和没有任何突出疾病的健康儿童中挑选。由儿科心脏病专家进行心电图检查。数据采用SPSS 19分析,p < 0.05显著水平。癫痫组心率(129.64±27.63)高于对照组(108.78±26.01)和热惊厥组(125.79±25.38);x2 = 28.701, p < 0.001)。对照组V1导联S波(0.72±0.36)高于癫痫组(0.58±0.45)和热惊厥组(0.58±0.36)。癫痫患儿QT离散度和QTc离散度水平高于热惊厥患儿,两者均高于对照组。结论癫痫病患儿aVL R、左室质量(LVM)、QT离散度、QTc离散度明显高于热惊厥患儿。为了对癫痫和热性惊厥患者保持良好的治疗策略,有必要评估心电图参数的改变,特别是QT间期的改变是否能导致更好的全面自主神经变化。
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引用次数: 3
Neuroglobin and Prolactin As Potential Biomarkers for Differentiating Epileptic versus Nonepileptic Paroxysmal Disorders in Children 神经球蛋白和催乳素作为鉴别儿童癫痫性与非癫痫性发作性疾病的潜在生物标志物
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-06-19 DOI: 10.1055/s-0040-1713153
M. Mahgoob, M. Moussa
Abstract At least 20% of patients referred to pediatric epilepsy centers with the suspicion of epileptic seizures actually have other conditions. Neuroglobin is a new globin member which is highly expressed in the central and peripheral nervous systems. In this article, we aimed to evaluate usefulness of neuroglobin to differentiate between epilepsy and other conditions that mimic epilepsy. Our study was conducted on 90 children divided into three groups: 30 patients with epileptic seizures, 35 children with nonepileptic paroxysmal disorder, and 25 apparently healthy, age and sex-matched children as a normal control. Complete blood count, blood chemistries including random blood glucose, calcium, sodium, in addition to serum prolactin, and neuroglobin were performed for all children. This study showed a significant increase of both serum neuroglobin and prolactin levels in epileptic group compared with nonepileptic paroxysmal disorder and control groups (p < 0.01). Serum neuroglobin showed 95% sensitivity and 95.7% specificity in the diagnosis of generalized seizures. Serum neuroglobin may be a promising novel marker to differentiate epileptic versus nonepileptic disorders in children in the emergency setting, when history and clinical presentation are equivocal.
至少有20%的儿童癫痫中心疑似癫痫发作的患者实际上有其他疾病。神经红蛋白是在中枢和周围神经系统中高度表达的一种新的珠蛋白成员。在这篇文章中,我们的目的是评估神经红蛋白对区分癫痫和其他类似癫痫的疾病的有用性。我们的研究将90名儿童分为三组:30名癫痫发作患者,35名非癫痫性发作性障碍儿童,25名表面健康,年龄和性别匹配的正常对照儿童。对所有儿童进行全血细胞计数、血液化学成分(包括随机血糖、钙、钠、血清催乳素和神经球蛋白)检测。研究结果显示,癫痫发作组血清神经球蛋白和催乳素水平较非癫痫发作性发作障碍及对照组显著升高(p < 0.01)。血清神经球蛋白诊断全局性癫痫的敏感性为95%,特异性为95.7%。当病史和临床表现不明确时,血清神经球蛋白可能是一种有希望的新标志物,用于区分儿童癫痫与非癫痫性疾病。
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引用次数: 0
Ethical Issues in Pediatric Regulatory Studies Involving Placebo Treatment 涉及安慰剂治疗的儿科监管研究中的伦理问题
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-06-04 DOI: 10.1055/s-0040-1712147
K. Rose, D. Neubauer, J. Grant-Kels
Abstract Separate pediatric studies for antiepileptic drugs (AEDs) emerged with general separate drug approval in children and were defined by the U.S. Food and Drug Administration (FDA) as <17 years and by the European Union (EU) as <18 years. These administrative age limits are necessary in pediatrics, but they correspond variably with the physiological maturity of young patients and are not helpful for therapeutic decisions or as study inclusion criteria. AEDs are often effective for partial onset seizures (POS) in 2 to 17-year-olds as well as in ≥18-year-olds, if dosed correctly. Separate pediatric AED studies assume no difference between the legal and the physiological meaning of the word “child.” While the FDA now accepts efficacy of AEDs in POS in children ≥2 years, the EU still requires separate “pediatric” studies. For retigabine it waived all pediatric studies after having required 20 such studies over several years. We feel the current regulation creates a situation where many studies in children are done unnecessarily; we question the ethics of such an approach, which in our view, is morally wrong. Critical publications contributed to the FDA's shift of opinion for AEDs in POS but did not address the blur of different meanings of the word “child.”
美国食品药品监督管理局(FDA)和欧盟(EU)分别将儿童抗癫痫药物(aed)的独立儿科研究定义为<17年和<18年。这些行政年龄限制在儿科是必要的,但它们与年轻患者的生理成熟程度相对应,对治疗决策或作为研究纳入标准没有帮助。如果剂量正确,aed通常对2至17岁以及≥18岁的部分发作性癫痫发作(POS)有效。单独的儿科AED研究认为“孩子”这个词的法律意义和生理意义没有区别。虽然FDA现在接受aed在POS≥2岁儿童中的疗效,但欧盟仍然需要单独的“儿科”研究。对于瑞加滨,在几年内要求进行20项此类研究后,它放弃了所有儿科研究。我们认为目前的规定造成了一种情况,即许多儿童研究都是不必要的;我们质疑这种做法的伦理性,在我们看来,这种做法在道德上是错误的。关键的出版物促成了FDA对POS中aed的看法转变,但没有解决“儿童”一词不同含义的模糊。
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引用次数: 10
Convulsive Status Epilepticus in Children: A Prospective Observational Study from India 儿童惊厥癫痫持续状态:一项来自印度的前瞻性观察研究
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-06-04 DOI: 10.1055/s-0040-1712544
Adhi Arya, C. Azad, V. Mahajan, V. Guglani
Abstract Convulsive status epilepticus (CSE) is one of the commonest and life threatening pediatric neurological emergencies. Only few studies on pediatric status epilepticus (SE) are available from the Indian subcontinent. The aim was to study the etiology and immediate outcome of CSE in children getting admitted in pediatric emergency during the study period and to study association of various clinical presentations with immediate outcome. A prospective observational study was conducted in tertiary care institute of Northern India. Children aged between 1 month and 18 years with CSE were enrolled and followed up till discharge. Primary outcome was kept as hospital survival/mortality; those who survived were further graded on the basis of disability at discharge. A total of 81 patients with CSE were assessed for primary outcome. The mortality rate was found to be 15% (12/81); 83% deaths were due to acute central nervous system (CNS) infection. Ten percent of children (8/81) had disability at discharge, five had mild, and three had moderate disability. The neurocysticercosis was the most common etiology seen in 23% (19/81) of the children followed by febrile SE in 20% (13/81) of the study participants. CSE responded to first-line antiepileptic drugs (AED) in 15% children (12/81). Refractory status was seen in 13.5% (11/81) cases. Prehospital treatment was received only in 15% patients. Young age, low Glasgow coma scale score at admission, and requirement of critical care support were the factors found to be significantly associated with mortality. CSE has a high mortality especially in young children and acute CNS infections are the most common cause of it.
惊厥性癫痫持续状态(CSE)是最常见和危及生命的儿科神经急症之一。只有少数研究儿童癫痫持续状态(SE)可从印度次大陆。目的是研究在研究期间儿科急诊科收治的儿童发生CSE的病因和直接预后,并研究各种临床表现与直接预后的关系。在印度北部三级保健研究所进行了一项前瞻性观察研究。年龄在1个月至18岁的CSE患儿入组并随访至出院。主要结局保存为医院生存/死亡率;那些幸存下来的人在出院时根据残疾程度进一步分级。共有81例CSE患者进行了主要结局评估。死亡率为15% (12/81);83%的死亡是由于急性中枢神经系统(CNS)感染。10%的儿童(8/81)在出院时残疾,5例为轻度残疾,3例为中度残疾。23%(19/81)的儿童最常见的病因是神经囊虫病,其次是20%(13/81)的发热性SE。15%的儿童(12/81)对一线抗癫痫药物(AED)有反应。13.5%(11/81)的病例出现难治性状态。只有15%的患者接受院前治疗。年龄小、入院时格拉斯哥昏迷评分低、需要重症监护支持是与死亡率显著相关的因素。CSE的死亡率很高,特别是在幼儿中,急性中枢神经系统感染是最常见的原因。
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引用次数: 3
Should Off Label Antiepileptic Drugs Be Used for Treatment of Infancy and Childhood Epilepsy? Discussions Based on a Dravet Syndrome Case Report 婴幼儿癫痫治疗是否应使用标签外抗癫痫药物?基于草稿综合征病例报告的讨论
IF 0.2 Q4 PEDIATRICS Pub Date : 2020-05-28 DOI: 10.1055/s-0040-1712498
M. Vințan
Abstract Epilepsy represents a burdensome neurological disorder with higher incidence before the age of 18 years. The treatment is medical and involves long-term administration of antiepileptic drugs (AED). There are known high-resistant syndromes with onset in infancy and childhood, Dravet syndrome, being one of them. It is a well-known fact that early seizures treatment prevents associated comorbidities, cognitive and motor disabilities, and improve long-term prognosis. There are several AEDs available but not all of them are approved for use in infants. This is due to the need for additional toxicology studies at this age and for development of suitable formulations. A 14-month-old girl with Dravet syndrome was presented here. Prompt diagnosis was made based on clinical features and confirmed by the genetic tests. She partially responded to valproate and clobazam but continued to have prolonged febrile seizures. We added stiripentol after consulting reports of studies in infants younger than 2 years and after obtaining family consent. She responded well with decrease in episodes of status epilepticus and improvement in psychomotor development and stiripentol was tolerated well. Off label use of certain AEDs can benefit infants when there are no major pharmacokinetic differences in comparison to older children.
癫痫是一种负担沉重的神经系统疾病,18岁前发病率较高。治疗是医学上的,包括长期服用抗癫痫药物(AED)。有一些已知的高耐药综合征,发病于婴儿期和儿童期,德拉韦综合征就是其中之一。众所周知,早期癫痫治疗可以预防相关的合并症、认知和运动障碍,并改善长期预后。有几种可用的aed,但并不是所有的都被批准用于婴儿。这是由于需要在这个年龄进行额外的毒理学研究和开发合适的配方。一个14个月大的女孩患有德拉韦综合征。根据临床特征及时诊断,并经基因检测证实。她对丙戊酸盐和氯巴唑有部分反应,但持续出现长时间的发热性惊厥。我们在查阅了2岁以下婴儿的研究报告并征得家属同意后加入了斯曲妥醇。她的反应良好,癫痫持续状态发作减少,精神运动发展改善,斯立哌丁醇耐受性良好。当与年龄较大的儿童相比,没有主要的药代动力学差异时,非标签使用某些aed可以使婴儿受益。
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引用次数: 0
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Journal of Pediatric Epilepsy
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