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Corrigendum to Cortical Gyrification Is Associated With the Clinical Phenotype in Tuberous Sclerosis Complex [Pediatric Neurology, Volume 161, December 2024, Pages 170-175]. 皮质回旋与结节性硬化症复合体的临床表型有关[《儿科神经病学》,第 161 卷,2024 年 12 月,第 170-175 页]的更正。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-11 DOI: 10.1016/j.pediatrneurol.2024.10.011
Nicolò Trevisan, Francesco Brunello, Fabio Sambataro, Gaia Biscalchin, Margherita Nosadini, Stefano Sartori, Concetta Luisi, Maria Federica Pelizza, Renzo Manara, Irene Toldo
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引用次数: 0
Pediatric Suprasellar Tumors: Unveiling the Mysteries of Craniopharyngioma and Germ Cell Tumors-Insights From Diagnosis to Advanced Therapeutics. 小儿星上肿瘤:揭开颅咽管瘤和生殖细胞瘤的神秘面纱--从诊断到先进治疗的启示。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-28 DOI: 10.1016/j.pediatrneurol.2024.10.016
Margaret Shatara, Mohamed S Abdelbaki

Background: Pediatric suprasellar tumors represent a unique and intricate challenge in the landscape of pediatric neuro-oncology.

Methods: We conducted an in-depth literature review, focusing on large clinical trials and major publications in pediatric suprasellar tumors, particularly craniopharyngiomas and germ cell tumors, to provide a comprehensive perspective on the challenges in the diagnosis, treatment, and molecular aspects of these tumors.

Results: Nestled within the critical confines of the suprasellar region, these tumors manifest against the backdrop of crucial growth and developmental processes. The suprasellar region, housing the pituitary gland and surrounding structures, plays a pivotal role in orchestrating hormonal regulation and growth. The emergence of tumors within this delicate terrain introduces a complex array of challenges, encompassing neurological, endocrinological, and developmental dimensions from damage to the hypothalamic-pituitary axis.

Conclusions: This article provides a thorough exploration of pediatric craniopharyngiomas and germ cell tumors, elucidating their clinical presentations, treatment modalities, and outcomes. The focused analysis aims to deepen our understanding of these tumors by offering insights for refined clinical management and improved patient outcomes.

背景:小儿鞍上肿瘤是小儿神经肿瘤学领域一个独特而复杂的挑战:小儿鞍上肿瘤是小儿神经肿瘤学领域一个独特而复杂的挑战:我们进行了深入的文献综述,重点关注小儿鞍上肿瘤(尤其是颅咽管瘤和生殖细胞瘤)的大型临床试验和主要出版物,以全面透视这些肿瘤在诊断、治疗和分子方面的挑战:这些肿瘤位于鞍上区域的重要范围内,是在关键的生长和发育过程中出现的。蝶鞍上区是垂体及其周围结构的所在地,在激素调节和生长过程中起着至关重要的作用。下丘脑-垂体轴受到损伤后,在这一微妙区域出现的肿瘤会带来一系列复杂的挑战,包括神经学、内分泌学和发育方面的挑战:本文对小儿颅咽管瘤和生殖细胞瘤进行了深入探讨,阐明了它们的临床表现、治疗方式和结果。重点分析旨在加深我们对这些肿瘤的认识,为完善临床管理和改善患者预后提供见解。
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引用次数: 0
Functional Syncope in Children and Adolescents: A Retrospective Cohort Study 儿童和青少年功能性晕厥:回顾性队列研究
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-22 DOI: 10.1016/j.pediatrneurol.2024.10.010
Imad T. Jarjour MD , Laila K. Jarjour MB, ChB, MPH , Katherine Tran MD , Danita Czyzewski PhD

Background

Functional syncope, or psychogenic pseudosyncope, is often under-recognized. We aimed to show that functional syncope may be diagnosed in most pediatric patients by the initial neurological consultation.

Methods

We reviewed the medical records of patients who were evaluated from 2006 to 2022 in clinic for apparent transient loss of consciousness (a-TLOC) and probable functional syncope. Inclusion criteria included the following: (1) one or more episodes of a-TLOC; (2) spontaneous recovery; (3) age <19 years; (4) patients collapse or lie immobile andunresponsive to verbal stimulation; (5) normal or mildly increased heart rate and blood pressure, if assessed; (6) no other medical causes; and (7) episodes occurred during tilt, electroencephalography, or electrocardiogram or were seen by the author on a smartphone video or in clinic. Patients meeting criteria 1 to 7 were classified as “definite” functional syncope and those meeting criteria 1 to 6 as “probable” functional syncope.

Results

We identified 31 patients with a-TLOC: 26 (23 females) had functional syncope, aged six to 17 years, whereas five were excluded (two functional seizures, one temporal lobe epilepsy, one vasovagal syncope, and one asthma). The clinical features of 13 patients in each group (definite versus probable) were not different statistically. Episodes were prolonged (1 to 270 minutes, mean 58 minutes) and frequent (daily or weekly in 65%), with eyes closed in 71% and eye flutter in 27%. After mean follow-up of 15 months in 14 patients: episodes disappeared in 29%, decreased >50% in 36%, and remained the same in 36%.

Conclusions

Functional syncope can be diagnosed at the initial neurological consultation without additional diagnostic testing in most patients.
背景:功能性晕厥或精神性假性晕厥往往未被充分认识。我们的目的是证明大多数儿科患者的功能性晕厥可通过神经科初诊诊断出来:我们回顾了 2006 年至 2022 年期间因明显的一过性意识丧失(a-TLOC)和可能的功能性晕厥而接受门诊评估的患者的病历。纳入标准包括以下几点:(1) 一次或多次发作 a-TLOC;(2) 自发恢复;(3) 年龄 结果:我们发现了 31 名 a-TLOC 患者:26 人(23 名女性)患有功能性晕厥,年龄在 6 至 17 岁之间,5 人被排除在外(2 名功能性癫痫发作患者、1 名颞叶癫痫患者、1 名血管迷走性晕厥患者和 1 名哮喘患者)。每组 13 名患者的临床特征(明确与可能)在统计学上没有差异。发作时间长(1 至 270 分钟,平均 58 分钟),频率高(65% 的患者每天或每周发作一次),71% 的患者闭眼,27% 的患者眼球跳动。对 14 名患者进行平均 15 个月的随访后发现:29% 的患者发作消失,36% 的患者发作次数减少 50%以上,36% 的患者发作次数保持不变:大多数患者在初次神经科会诊时即可诊断出功能性晕厥,无需额外的诊断测试。
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引用次数: 0
Neurocognitive Evaluation of Patients With DiGeorge Syndrome 迪乔治综合征患者的神经认知评估。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-21 DOI: 10.1016/j.pediatrneurol.2024.10.012
Zuhal Karali MD , Yasin Karali MD , Sukru Cekic MD , Berfin Altinok MD , Muhittin Bodur MD , Mustafa Bostanci MD , Sara S. Kilic MD

Background

DiGeorge syndrome (DGS), the most common microdeletion syndrome, affects multiple organs, including the heart, the nervous system, and the immune system. In this study, we aimed to evaluate the clinical, laboratory, brain magnetic resonance imaging (MRI), and neurocognitive findings of our patients with DGS.

Methods

Clinical and laboratory data of 52 patients with DGS between June 2000 and March 2022 were evaluated retrospectively. Brain MRI and neuropsychologic tests were performed to assess the neurocognitive status of the patients.

Results

Fifty-two patients (28 males and 24 females) were included in our study. Fifteen of them died during the follow-up. All 37 patients who are alive had partial DGS. The median age of patients was 10 years and 7 months, and the median age at diagnosis was 5 years and 4 months. Bilateral conduction deceleration in the anterior visual pathways in six (20%) of 30 patients was determined by the visual evoked potentials. The auditory brainstem evoked potential test showed sensorineural hearing loss in 11 of 30 (36.6%) patients. Brain MRI disclosed brain parenchymal abnormalities in 18 of 25 (72%) patients. Impairments in executive functions, expressive language, and verbal memory were noted in 18 patients who were neuropsychologically assessed.

Conclusions

It is important to keep in mind that patients with DGS may be accompanied by neurocognitive findings. Awareness of the potential for underlying psychiatric and neurodevelopment disorders is key to anticipatory guidance, optimization of therapies, and maximizing life quality.
背景:迪乔治综合征(DGS)是最常见的微缺失综合征,影响多个器官,包括心脏、神经系统和免疫系统。在这项研究中,我们旨在评估 DGS 患者的临床、实验室、脑磁共振成像(MRI)和神经认知结果:回顾性评估了 2000 年 6 月至 2022 年 3 月期间 52 例 DGS 患者的临床和实验室数据。结果:52 名 DGS 患者(28 名男性)的临床和实验室数据得到了回顾性评估,脑磁共振成像和神经心理学测试对患者的神经认知状况进行了评估:研究共纳入52名患者(28名男性,24名女性)。其中 15 人在随访期间死亡。37名存活的患者均患有部分DGS。患者的中位年龄为 10 岁零 7 个月,确诊时的中位年龄为 5 岁零 4 个月。根据视觉诱发电位确定,30 名患者中有 6 人(20%)的双侧视觉前通路传导减速。听觉脑干诱发电位测试显示,30 名患者中有 11 人(36.6%)出现感音神经性听力损失。脑磁共振成像显示,25 名患者中有 18 名(72%)出现脑实质异常。18名接受神经心理学评估的患者在执行功能、语言表达能力和言语记忆力方面均存在障碍:重要的是要记住,DGS 患者可能伴有神经认知结果。意识到潜在的精神和神经发育障碍是提供预期指导、优化疗法和最大限度提高生活质量的关键。
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引用次数: 0
New-Onset Refractory Status Epilepticus With Diffuse Cerebral Restricted Diffusion in Young Children: A Novel Clinical-Radiologic Presentation 新发难治性癫痫状态伴弥漫性脑弥散受限:一种新的临床放射学表现。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-18 DOI: 10.1016/j.pediatrneurol.2024.10.009
Hope M. Reecher BS , Niyati P. Mehta MD , Namrata D. Patel MD, MS , Rachel A. Sawdy DNP , Raquel Farias-Moeller MD

Background

New-onset refractory status epilepticus (NORSE) is a clinical presentation characterized by explosive-onset refractory status epilepticus (RSE) without evident etiology or active epilepsy, often leading to devastating epilepsy. There is heterogeneity in neuroradiographic findings for NORSE. We encountered a series of young patients with NORSE who had diffuse cerebral restriction in diffusion (DCRD) with similar radiographic appearances as acute encephalopathy with biphasic seizures and late restricted diffusion/acute leukoencephalopathy with restricted diffusion (AESD/ALERD). We explore clinical similarities and proposed pathophysiologic overlaps to highlight a novel clinical-radiologic presentation.

Methods

Retrospective review was completed for patients younger than five years meeting NORSE criteria and then screened for radiographic evidence of DCRD. Demographic, clinical, and outcome data were collected.

Results

Eleven patients met NORSE criteria, of whom seven displayed DCRD. Immunosuppressant management varied. All patients required multiple antiseizure medications and continuous infusions for RSE. Only one had an etiology identified (genetic). All but one patient developed diffuse, global, and progressive cerebral atrophy. Two patients died: one after prolonged seizure three years post-NORSE and another of unknown causes two months post-NORSE. Of five survivors, three have medically refractory epilepsy. Most survivors have severe disability.

Conclusions

We present a single-center case series of seven patients with NORSE and DCRD, akin to AESD/ALERD. Our patients differed clinically to AESD/ALERD in terms of seizure severity and poorer outcome. There is a need to develop biomarkers for specific NORSE phenotypes. The young child with NORSE and DCRD may represent a novel phenotype with a specific neuroradiographic signature that deserves further attention.
背景:新发难治性状态癫痫(NORSE)是一种临床表现,其特点是爆发性发作的难治性状态癫痫(RSE),无明显病因或活动性癫痫,通常会导致破坏性癫痫。NORSE 的神经放射学检查结果存在异质性。我们遇到了一系列年轻的 NORSE 患者,他们的弥漫性脑弥散受限(DCRD)与急性脑病伴双相癫痫发作和晚期弥散受限/急性白质脑病伴弥散受限(AESD/ALERD)的影像学表现相似。我们探讨了两者的临床相似性和病理生理学重叠,以突出一种新的临床放射学表现:方法:我们对符合 NORSE 标准的五岁以下患者进行了回顾性检查,然后筛选出 DCRD 的放射学证据。结果:11 名患者符合 NORSE 标准:结果:11 名患者符合 NORSE 标准,其中 7 人显示出 DCRD。免疫抑制剂的使用方法各不相同。所有患者都需要服用多种抗癫痫药物并持续输注 RSE。只有一名患者找到了病因(遗传)。除一名患者外,所有患者都出现了弥漫性、全身性和进行性脑萎缩。两名患者死亡:一名是在 NORSE 发生三年后长期癫痫发作后死亡,另一名是在 NORSE 发生两个月后死于不明原因。五名幸存者中,三人患有药物难治性癫痫。大多数幸存者严重残疾:我们介绍了一个由七名 NORSE 和 DCRD(类似于 AESD/ALERD)患者组成的单中心病例系列。我们的患者与 AESD/ALERD 的临床表现不同,他们的癫痫发作严重程度和预后较差。有必要针对特定的 NORSE 表型开发生物标记物。患有 NORSE 和 DCRD 的幼儿可能代表了一种具有特定神经放射学特征的新型表型,值得进一步关注。
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引用次数: 0
Diagnostic and Therapeutic Insights Into Pediatric Neurosarcoidosis: Observations From French Pediatric Rheumatology Centers 小儿神经肉芽肿病的诊断和治疗见解:法国小儿风湿病中心的观察。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-18 DOI: 10.1016/j.pediatrneurol.2024.10.007
Racha Tohme MD , Anca Tanase MD , Cécile Dumaine MD , Perrine Dusser MD , Homa Adle-Biassette MD, PhD , Veronique Despert MD , Albert Faye MD, PhD , Inès Mannes MD , Isabelle Melki MD, PhD , Isabelle Kone-Paut MD, PhD , Ulrich Meinzer MD, PhD , SOFREMIP, French Society for Rheumatology and Pediatric Inflammatory Diseases

Background

The diagnosis and management of neurosarcoidosis (NS) in pediatric patients remain challenging, with limited case documentation to guide clinicians. Most existing reports focus on initial presentations. This study aimed to outline the clinical features, management, and medium-term outcomes of pediatric NS

Methods

In this retrospective, multicentric, observational study, we collected data from pediatric patients followed in French pediatric rheumatology centers with a diagnosis of NS between January 2001 and June 2023.

Results

We identified 11 patients diagnosed with NS, comprising eight girls and three boys. The mean age at diagnosis of sarcoidosis was 10 (5 to 15) years, and the mean age of diagnosis of NS was 11.5 (5 to 17) years. Predominant neurological symptoms included headache (nine of 11 patients), papilledema (6 of 11 patients), facial palsy (two patients), seizures (one patient), and motor deficit (two patients). Nine of 11 patients had eye involvement, which consisted of granulomatous and bilateral uveitis. All patients exhibited meningitis, with cerebrospinal fluid white blood cell counts ranging from 6 to 70 cells/mm3. Six individuals presented neurological abnormalities on imaging, detailed in this study. Treatment primarily involved corticosteroids, methotrexate, and tumor necrosis factor alpha (TNF-alpha) inhibitors. Biologics targeting TNF-alpha were necessary to achieve remission in eight of 11 patients. In two patients who did not receive this treatment initially, it was required later in the course of evolution.

Conclusions

This study enhances understanding of the clinical course of pediatric NS and supports the early use of TNF-alpha biologics for improved management in affected children.
背景:儿科神经肉芽肿病(NS)的诊断和治疗仍具有挑战性,指导临床医生的病例资料有限。现有的大多数报告都集中在最初的表现上。方法:在这项回顾性、多中心观察研究中,我们收集了2001年1月至2023年6月期间在法国儿科风湿病学中心随访的诊断为NS的儿科患者的数据:我们发现了11名确诊为NS的患者,其中包括8名女孩和3名男孩。确诊为肉样瘤病的平均年龄为10(5至15)岁,确诊为NS的平均年龄为11.5(5至17)岁。主要的神经系统症状包括头痛(11 名患者中的 9 人)、乳头水肿(11 名患者中的 6 人)、面瘫(2 名患者)、癫痫发作(1 名患者)和运动障碍(2 名患者)。11 名患者中有 9 名眼部受累,包括肉芽肿性和双侧葡萄膜炎。所有患者均表现为脑膜炎,脑脊液白细胞计数从6到70个/立方毫米不等。六名患者在影像学检查中出现神经系统异常,详见本研究。治疗主要包括皮质类固醇、甲氨蝶呤和肿瘤坏死因子α(TNF-α)抑制剂。在11名患者中,有8名患者需要使用针对TNF-α的生物制剂才能获得缓解。有两名患者最初没有接受这种治疗,但在后来的演变过程中需要接受这种治疗:这项研究加深了人们对小儿NS临床病程的了解,并支持尽早使用TNF-α生物制剂来改善患儿的治疗。
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引用次数: 0
Alice in Wonderland Syndrome in Children With Severe Acute Respiratory Syndrome SARS-CoV-2 Infection: A Case Series of Two Patients in an Italian Hospital 严重急性呼吸综合征 SARS-CoV-2 感染儿童的爱丽丝梦游仙境综合征:意大利一家医院两名患者的病例系列。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-18 DOI: 10.1016/j.pediatrneurol.2024.10.008
Susanna Staccioli MD, PhD , Rosanna Mariani MD, PhD , Sarah Bompard MD , Nicole Olivini MD , Claudia Fanfoni MD , Gianluca Mirra MD , Eleonora Bisozzi TNFP , Andrea Campana MD , Donatella Lettori MD, PhD

Background

Alice in Wonderland syndrome (AIWS) is a disorienting neurological condition that affects human perception to the senses of vision, hearing, touch, and sensation and the phenomenon of time. Herein we report two pediatric cases of AIWS temporally related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.

Case presentation

An eight-year-old-girl without history of migraine or epilepsy experienced some episodes of visual distortions (micropsia, macropsia, and teleopsia) and misperception of sound, sometimes associated with headache. The onset of symptoms began at the occurrence of fever (38°C), during SARS-CoV-2 infection. Another six-year-old girl, with no history of migraine or epilepsy, experienced short-term episodes of visual (metamorphopsia) and color disturbance (chromatopsia), during an otherwise asymptomatic SARS-CoV-2 infection. In both cases, clinical examination was unremarkable; surface electroencephalography showed normal findings, without any correlation between visual phenomena and cortical activity; and brain magnetic resonance was normal. The patients were given symptomatic treatment, consisting of anti-inflammatory drugs on demand. The frequency of episodes decreased progressively following a negative SARS-CoV-2 test, with full remission in a few weeks. At the moment of hospital admission, none of the patients had completed the two-dose vaccination schedule for SARS-CoV-2.

Conclusion

Based on our clinical experience, we believe SARS-CoV-2 may be responsible for AIWS, in addition to other neurological symptoms more frequently documented in the literature. Pathogenesis is multifactorial and arises from the activation of inflammatory pathways. We therefore suggest also searching for SARS-CoV-2, among other viruses linked with AIWS, in children presenting with visual and/or auditory hallucinations, even as isolated symptoms.
背景介绍爱丽丝梦游仙境综合征(Alice in Wonderland Syndrome,AIWS)是一种神经迷失症,会影响人的视觉、听觉、触觉、感觉和时间现象。在此,我们报告了两例与严重急性呼吸系统综合征冠状病毒 2(SARS-CoV-2)感染在时间上相关的儿童 AIWS 病例:病例介绍:一名八岁女孩,无偏头痛或癫痫病史,出现过一些视觉扭曲(小视、大视野和远视)和声音错觉,有时伴有头痛。这些症状是在感染 SARS-CoV-2 期间发烧(38°C)时开始出现的。另一名六岁女孩没有偏头痛或癫痫病史,在感染 SARS-CoV-2 期间出现了短期的视觉(偏视)和颜色障碍(色觉)。两例患者的临床检查均无异常;表面脑电图显示正常,视觉现象与大脑皮层活动之间无任何关联;脑磁共振检查正常。患者接受了对症治疗,包括按需服用抗炎药物。SARS-CoV-2 检测呈阴性后,发病频率逐渐减少,几周后完全缓解。入院时,所有患者均未完成两剂 SARS-CoV-2 疫苗接种:根据我们的临床经验,我们认为除了文献中更常见的其他神经系统症状外,SARS-CoV-2 也可能是导致 AIWS 的原因。发病机制是多因素的,源于炎症通路的激活。因此,我们建议在出现视觉和/或听觉幻觉(即使是孤立的症状)的儿童中也要寻找 SARS-CoV-2,以及其他与 AIWS 相关的病毒。
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引用次数: 0
Effect of Piracetam and Iron Supplementation on Heart Rate Variability in Children With Breath-Holding Spells: Effective Treatment or Placebo? 吡拉西坦和铁补充剂对憋气儿童心率变异性的影响:有效治疗还是安慰剂?
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-12 DOI: 10.1016/j.pediatrneurol.2024.10.005
Prateek Kumar Panda DM, Indar Kumar Sharawat DM
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引用次数: 0
Horner Syndrome Secondary to Rapid Enlargement of a Mediastinal Lymphatic Malformation 继发于纵隔淋巴畸形快速增大的霍纳综合征。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-12 DOI: 10.1016/j.pediatrneurol.2024.10.006
Elizabeth Pickup MD , Kyle Spagnolo DO , Alexandra B. Kornbluh MD
Horner syndrome is a clinical triad of ptosis, miosis, and anhidrosis, which commonly prompts urgent neuroimaging. The differential diagnosis of new-onset Horner syndrome in children includes neurological emergencies. We report here a case of a 13-month-old male infant with acute-onset Horner syndrome, subsequently found to have a large mediastinal lymphatic malformation compressing the trachea and neck vasculature.
霍纳综合征是一种由上睑下垂、瞳孔缩小和无汗症组成的临床三联征,通常需要进行紧急神经影像学检查。儿童新发霍纳综合征的鉴别诊断包括神经系统急症。我们在此报告了一例 13 个月大的急性霍纳综合征男婴,随后发现其纵隔淋巴畸形压迫气管和颈部血管。
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引用次数: 0
Racial, Ethnic, and Socioeconomic Disparities in Pediatric Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder 小儿水肿素-4 阳性神经脊髓炎谱系障碍的种族、民族和社会经济差异。
IF 3.2 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-10-11 DOI: 10.1016/j.pediatrneurol.2024.09.028
Kelsey E. Poisson MD , Linda Nguyen MD, PhD , Paul S. Horn PhD , Andrew F. Beck MD, MPH , Helen Wu MD, PhD , Kris Wesselkamper MD , Jayne M. Ness MD, PhD , Yolanda S. Wheeler PhD, CRNP, CPNP-AC, MSCN

Background

Only 5% of aquaporin-4-positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD) cases emerge during childhood. Poorer outcomes have been suggested in black/African American (AA) adults with NMOSD; however, conflicting and limited data exist for pediatric-onset NMOSD. This study evaluates racial, ethnic, and socioeconomic disparities in pediatric AQP4+ NMOSD outcomes.

Methods

Thirty-eight pediatric patients with AQP4+ NMOSD cared for at three pediatric tertiary care centers between 2009 and 2021 were identified. Patient addresses connected to socioeconomic measures available from the US Census. Demographic characteristics, pertinent clinical outcomes, and health care utilization in the two years following diagnosis were captured.

Results

Compared with non-Hispanic White children, Black/AA children had a significantly higher Expanded Disability Status Scale (EDSS) (2.46 vs 0.33, P = 0.003), 2.37 more hospital admissions (P = 0.002), and 28.40 additional inpatient days (P = 0.002) in the two years following their NMOSD diagnosis. Additionally, children with public insurance had higher relapse rates than those with private insurance (P = 0.046). At two years and at the most recent follow-up, a significantly higher EDSS was correlated with children living in census tracts with a lower median income, higher deprivation index, and higher proportion of population on assisted income, in poverty, and with vacant housing (all P < 0.05).

Conclusions

We identified racial, ethnic, and socioeconomic disparities in clinical outcomes and health care utilization in pediatric AQP4+ NMOSD. Further prospective and household-level data are needed to dissect the interplay of genetics, structural racism, and social determinants of health so that interventions to optimize care and outcomes for this population may be developed.
背景:只有5%的水通道蛋白-4阳性神经脊髓炎视网膜频谱障碍(AQP4+ NMOSD)病例在儿童期发病。有研究表明,黑人/非裔美国人(AA)成人 NMOSD 患者的预后较差;然而,关于儿童期 NMOSD 的数据却相互矛盾且有限。本研究评估了儿科 AQP4+ NMOSD 结果的种族、民族和社会经济差异:方法:确定了 2009 年至 2021 年间在三家儿科三级护理中心接受护理的 38 名 AQP4+ NMOSD 儿科患者。患者地址与美国人口普查提供的社会经济数据相关联。结果显示:与非西班牙裔白人相比,儿童NMOSD患者的社会经济状况要好得多:与非西班牙裔白人儿童相比,黑人/美国黑人儿童的残疾状况扩展量表(EDSS)明显更高(2.46 vs 0.33,P = 0.003),在确诊 NMOSD 后的两年中,入院次数增加 2.37 次(P = 0.002),住院天数增加 28.40 天(P = 0.002)。此外,参加公共保险的儿童的复发率高于参加私人保险的儿童(P = 0.046)。在两年后和最近一次随访中,EDSS 明显较高的儿童与居住在收入中位数较低、贫困指数较高、领取补助收入的人口比例较高、贫困以及住房空置的人口普查区相关(所有 P 均为结论):我们发现了小儿 AQP4+ NMOSD 在临床结果和医疗保健使用方面的种族、民族和社会经济差异。我们需要更多的前瞻性和家庭层面的数据,以剖析遗传学、结构性种族主义和健康的社会决定因素之间的相互作用,从而制定干预措施,优化这一人群的护理和治疗效果。
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引用次数: 0
期刊
Pediatric neurology
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