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1.
J Child Neurol ; 39(7-8): 275-284, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-39246040

RESUMEN

INTRODUCTION: Studies suggest disparities in outcomes in minoritized children after severe traumatic brain injury. We aimed to evaluate for disparities in intracranial pressure-directed therapies and outcomes after pediatric severe traumatic brain injury. METHODS: We conducted a secondary analysis of the Approaches and Decisions for Acute Pediatric TBI (ADAPT) Trial, which enrolled pediatric severe traumatic brain injury patients (Glasgow Coma Scale score ≤8) with an intracranial pressure monitor from 2014 to 2018. Patients admitted outside of the United States were excluded. Patients were categorized by race and ethnicity (Hispanic, non-Hispanic Black, non-Hispanic White, and "Other"). We evaluated outcomes by assessing mortality and 3-month Glasgow Outcome Score-Extended for Pediatrics. Our analysis involved parametric and nonparametric testing. MAIN RESULTS: A total of 671 children were analyzed. Significant associations included older age in non-Hispanic White patients (P < .001), more surgical evacuations in "Other" (P < .001), and differences in discharge location (P = .040). The "other" cohort received hyperventilation less frequently (P = .046), although clinical status during Paco2 measurement was not known. There were no other significant differences in intracranial pressure-directed therapies. Hispanic ethnicity was associated with lower mortality (P = .004) but did not differ in unfavorable outcome (P = .810). Glasgow Outcome Score-Extended for Pediatrics was less likely to be collected for non-Hispanic Black patients (69%; P = .011). CONCLUSIONS: Our analysis suggests a general lack of disparities in intracranial pressure-directed therapies and outcomes in children after severe traumatic brain injury. Lower mortality in Hispanic patients without a concurrent decrease in unfavorable outcomes, and lower availability of Glasgow Outcome Score-Extended for Pediatrics score for non-Hispanic Black patients merit further investigation.


Asunto(s)
Lesiones Traumáticas del Encéfalo , Disparidades en Atención de Salud , Presión Intracraneal , Humanos , Lesiones Traumáticas del Encéfalo/terapia , Lesiones Traumáticas del Encéfalo/etnología , Lesiones Traumáticas del Encéfalo/mortalidad , Niño , Femenino , Masculino , Preescolar , Adolescente , Disparidades en Atención de Salud/etnología , Disparidades en Atención de Salud/estadística & datos numéricos , Presión Intracraneal/fisiología , Resultado del Tratamiento , Hispánicos o Latinos/estadística & datos numéricos , Etnicidad , Lactante , Negro o Afroamericano/estadística & datos numéricos , Escala de Consecuencias de Glasgow , Población Blanca/estadística & datos numéricos
5.
Dimens Crit Care Nurs ; 43(5): 231-238, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39074225

RESUMEN

BACKGROUND: Nursing interventions in the care of pediatric patients with severe traumatic brain injury (TBI) can have a direct effect on intracranial pressure (ICP), yet they have been largely underexplored. Early evidence is therefore needed to describe these relationships and to determine intervention that promotes neuroprotection and recovery. OBJECTIVES: The aim of this study was to examine nursing interventions within the first 72 hours of pediatric severe TBI and their effects on ICP. METHOD: This is a retrospective review of pediatric patients admitted for severe TBI using a quasi-experimental approach to assess nursing interventions and their association with the patients' ICP values prior to and after each intervention. RESULTS: Of the 56 patients who met the inclusion criteria, 3392 intervention events (range, 31-138 events per patient) were reported. Paired t tests conducted for each intervention type found a statistically significant relationship with suctioning and percent change in ICP values (P = .045). All other interventions showed no significant differences. DISCUSSION: Standard nursing interventions, specifically suctioning, in pediatric severe TBI may affect ICP and therefore neuroprotection. Further work is needed to better understand the role and timing of nursing interventions and their influence on cerebral hemodynamics so that future TBI guidelines consider nursing care and their impact on brain injury recovery.


Asunto(s)
Lesiones Traumáticas del Encéfalo , Presión Intracraneal , Humanos , Lesiones Traumáticas del Encéfalo/enfermería , Estudios Retrospectivos , Niño , Masculino , Femenino , Adolescente , Preescolar , Enfermería de Cuidados Críticos
6.
JAMA Netw Open ; 7(6): e2414122, 2024 Jun 03.
Artículo en Inglés | MEDLINE | ID: mdl-38857050

RESUMEN

Importance: Neurological manifestations during acute SARS-CoV-2-related multisystem inflammatory syndrome in children (MIS-C) are common in hospitalized patients younger than 18 years and may increase risk of new neurocognitive or functional morbidity. Objective: To assess the association of severe neurological manifestations during a SARS-CoV-2-related hospital admission with new neurocognitive or functional morbidities at discharge. Design, Setting, and Participants: This prospective cohort study from 46 centers in 10 countries included patients younger than 18 years who were hospitalized for acute SARS-CoV-2 or MIS-C between January 2, 2020, and July 31, 2021. Exposure: Severe neurological manifestations, which included acute encephalopathy, seizures or status epilepticus, meningitis or encephalitis, sympathetic storming or dysautonomia, cardiac arrest, coma, delirium, and stroke. Main Outcomes and Measures: The primary outcome was new neurocognitive (based on the Pediatric Cerebral Performance Category scale) and/or functional (based on the Functional Status Scale) morbidity at hospital discharge. Multivariable logistic regression analyses were performed to examine the association of severe neurological manifestations with new morbidity in each SARS-CoV-2-related condition. Results: Overall, 3568 patients younger than 18 years (median age, 8 years [IQR, 1-14 years]; 54.3% male) were included in this study. Most (2980 [83.5%]) had acute SARS-CoV-2; the remainder (588 [16.5%]) had MIS-C. Among the patients with acute SARS-CoV-2, 536 (18.0%) had a severe neurological manifestation during hospitalization, as did 146 patients with MIS-C (24.8%). Among survivors with acute SARS-CoV-2, those with severe neurological manifestations were more likely to have new neurocognitive or functional morbidity at hospital discharge compared with those without severe neurological manifestations (27.7% [n = 142] vs 14.6% [n = 356]; P < .001). For survivors with MIS-C, 28.0% (n = 39) with severe neurological manifestations had new neurocognitive and/or functional morbidity at hospital discharge compared with 15.5% (n = 68) of those without severe neurological manifestations (P = .002). When adjusting for risk factors in those with severe neurological manifestations, both patients with acute SARS-CoV-2 (odds ratio, 1.85 [95% CI, 1.27-2.70]; P = .001) and those with MIS-C (odds ratio, 2.18 [95% CI, 1.22-3.89]; P = .009) had higher odds of having new neurocognitive and/or functional morbidity at hospital discharge. Conclusions and Relevance: The results of this study suggest that children and adolescents with acute SARS-CoV-2 or MIS-C and severe neurological manifestations may be at high risk for long-term impairment and may benefit from screening and early intervention to assist recovery.


Asunto(s)
COVID-19 , Hospitalización , Enfermedades del Sistema Nervioso , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica , Humanos , COVID-19/complicaciones , COVID-19/epidemiología , Niño , Femenino , Masculino , Preescolar , Hospitalización/estadística & datos numéricos , Adolescente , Estudios Prospectivos , Síndrome de Respuesta Inflamatoria Sistémica/epidemiología , Enfermedades del Sistema Nervioso/etiología , Enfermedades del Sistema Nervioso/epidemiología , Lactante , Índice de Severidad de la Enfermedad
8.
Semin Pediatr Neurol ; 49: 101117, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38677796

RESUMEN

Neuromonitoring is used to assess the central nervous system in the intensive care unit. The purpose of neuromonitoring is to detect neurologic deterioration and intervene to prevent irreversible nervous system dysfunction. Neuromonitoring starts with the standard neurologic examination, which may lag behind the pathophysiologic changes. Additional modalities including continuous electroencephalography (CEEG), multiple physiologic parameters, and structural neuroimaging may detect changes earlier. Multimodal neuromonitoring now refers to an integrated combination and display of non-invasive and invasive modalities, permitting tailored treatment for the individual patient. This chapter reviews the non-invasive and invasive modalities used in pediatric neurocritical care.


Asunto(s)
Unidades de Cuidado Intensivo Pediátrico , Monitorización Neurofisiológica , Humanos , Niño , Monitorización Neurofisiológica/métodos , Electroencefalografía/métodos , Cuidados Críticos/métodos , Neuroimagen/métodos
9.
Brain Spine ; 4: 102731, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38510614

RESUMEN

Background: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) carries variability in arterial flow pulsatility (AFP). Research question: What changes in cerebral and somatic oxygenation, hemodynamics, and autonomic function are associated with AFP during VA-ECMO? Methods: This is a prospective study of children on VA-ECMO undergoing neuromonitoring. AFP was quantified by arterial blood pressure pulse amplitude and subcategorized: no pulsatility (<1 mmHg), minimal pulsatility (1 to <5 mmHg), moderate pulsatility (5 to <15 mmHg) and high pulsatility (≥15 mmHg). CVPR was assessed using the cerebral oximetry index (COx). Cerebral and somatic oxygenation was assessed using cerebral regional oximetry (rSO2) or peripheral oxygen saturation (SpO2). Autonomic function was assessed using baroreflex sensitivity (BRs), low-frequency high-frequency (LF/HF) ratio and standard deviation of heart rate R-R intervals (HRsd). Differences were assessed across AFP categories using linear mixed effects models with Tukey pairwise comparisons. Univariate logistic regression was used to explore risk of AFP with brain injuries. Results: Among fifty-three children, comparisons of moderate to high pulsatility were associated with reductions in rSO2 (p < 0.001), SpO 2 (p = 0.005), LF/HF ratio (p = 0.028) and an increase in HRsd (p < 0.001). Reductions in BRs were observed across comparisons of none to minimal (P < 0.001) and minimal to moderate pulsatility (p = 0.004). Comparisons of no to low pulsatility were associated with reductions in BRs (p < 0.001) and ABP (p < 0.001) with increases in SpO2 (p < 0.001) and HR (p < 0.001). Arterial ischemic stroke was associated with higher pulsatility (p = 0.0384). Conclusion: During VA-ECMO support, changes toward high AFP are associated with autonomic dysregulation and compromised cerebral and somatic tissue oxygenation.

10.
Front Pediatr ; 12: 1340385, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38410766

RESUMEN

Introduction: Hospitalized children diagnosed with SARS-CoV-2-related conditions are at risk for new or persistent symptoms and functional impairments. Our objective was to analyze post-hospital symptoms, healthcare utilization, and outcomes of children previously hospitalized and diagnosed with acute SARS-CoV-2 infection or Multisystem Inflammatory Syndrome in Children (MIS-C). Methods: Prospective, multicenter electronic survey of parents of children <18 years of age surviving hospitalization from 12 U.S. centers between January 2020 and July 2021. The primary outcome was a parent report of child recovery status at the time of the survey (recovered vs. not recovered). Secondary outcomes included new or persistent symptoms, readmissions, and health-related quality of life. Multivariable backward stepwise logistic regression was performed for the association of patient, disease, laboratory, and treatment variables with recovered status. Results: The children [n = 79; 30 (38.0%) female] with acute SARS-CoV-2 (75.7%) or MIS-C (24.3%) had a median age of 6.5 years (interquartile range 2.0-13.0) and 51 (64.6%) had a preexisting condition. Fifty children (63.3%) required critical care. One-third [23/79 (29.1%)] were not recovered at follow-up [43 (31, 54) months post-discharge]. Admission C-reactive protein levels were higher in children not recovered vs. recovered [5.7 (1.3, 25.1) vs. 1.3 (0.4, 6.3) mg/dl, p = 0.02]. At follow-up, 67% overall had new or persistent symptoms. The most common symptoms were fatigue (37%), weakness (25%), and headache (24%), all with frequencies higher in children not recovered. Forty percent had at least one return emergency visit and 24% had a hospital readmission. Recovered status was associated with better total HRQOL [87 (77, 95) vs. 77 (51, 83), p = 0.01]. In multivariable analysis, lower admission C-reactive protein [odds ratio 0.90 (95% confidence interval 0.82, 0.99)] and higher admission lymphocyte count [1.001 (1.0002, 1.002)] were associated with recovered status. Conclusions: Children considered recovered by their parents following hospitalization with SARS-CoV-2-related conditions had less symptom frequency and better HRQOL than those reported as not recovered. Increased inflammation and lower lymphocyte count on hospital admission may help to identify children needing longitudinal, multidisciplinary care. Clinical Trial Registration: ClinicalTrials.gov (NCT04379089).

11.
Pediatrics ; 153(2)2024 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-38263886

RESUMEN

Sickle cell disease (SCD) is a chronic hematologic disorder which causes progressive cerebral arteriopathy beginning in childhood. As a result, arterial ischemic stroke is a major cause of morbidity and mortality in SCD, and SCD is a leading cause of childhood stroke worldwide. Allogenic hematopoietic stem cell transplant (HSCT) may be curative for individuals with SCD. Long-term outcomes and effects are currently being studied. In this report, we describe a child with SCD who presented with arterial ischemic stroke at 6 years of age and was found to have a severe form of cerebral large vessel arteriopathy by catheter-directed angiography. The patient initially underwent revascularization surgery by indirect superficial temporal artery to middle cerebral artery bypass, and 1 year later, he underwent curative HSCT. Approximately 3 years after HSCT, repeat catheter-directed angiography revealed a striking reversal of cerebral large vessel arteriopathy. This article reveals a previously unrecognized and potentially beneficial effect of HSCT that may ameliorate cerebral large vessel arteriopathy and improve cerebrovascular health for children with SCD.


Asunto(s)
Anemia de Células Falciformes , Trasplante de Células Madre Hematopoyéticas , Accidente Cerebrovascular Isquémico , Accidente Cerebrovascular , Niño , Humanos , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/terapia , Trasplante de Células Madre Hematopoyéticas/efectos adversos
12.
Neurocrit Care ; 40(2): 759-768, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37697125

RESUMEN

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) provides lifesaving support to critically ill patients who experience refractory cardiopulmonary failure but carries a high risk for acute brain injury. We aimed to identify characteristics reflecting acute brain injury in children requiring ECMO support. METHODS: This is a prospective observational study from 2019 to 2022 of pediatric ECMO patients undergoing neuromonitoring, including continuous electroencephalography, cerebral oximetry, and transcranial Doppler ultrasound (TCD). The primary outcome was acute brain injury. Clinical and neuromonitoring characteristics were collected. Multivariate logistic regression was implemented to model odds ratios (ORs) and identify the combined characteristics that best discriminate risk of acute brain injury using the area under the receiver operating characteristic curve. RESULTS: Seventy-five pediatric patients requiring ECMO support were enrolled in this study, and 62 underwent neuroimaging or autopsy evaluations. Of these 62 patients, 19 experienced acute brain injury (30.6%), including seven (36.8%) with arterial ischemic stroke, four (21.1%) with hemorrhagic stroke, seven with hypoxic-ischemic brain injury (36.8%), and one (5.3%) with both arterial ischemic stroke and hypoxic-ischemic brain injury. A univariate analysis demonstrated acute brain injury to be associated with maximum hourly seizure burden (p = 0.021), electroencephalographic suppression percentage (p = 0.022), increased interhemispheric differences in electroencephalographic total power (p = 0.023) and amplitude (p = 0.017), and increased differences in TCD Thrombolysis in Brain Ischemia (TIBI) scores between bilateral middle cerebral arteries (p = 0.023). Best subset model selection identified increased seizure burden (OR = 2.07, partial R2 = 0.48, p = 0.013), increased quantitative electroencephalographic interhemispheric amplitude differences (OR = 2.41, partial R2 = 0.48, p = 0.013), and increased interhemispheric TCD TIBI score differences (OR = 4.66, partial R2 = 0.49, p = 0.006) to be independently associated with acute brain injury (area under the receiver operating characteristic curve = 0.92). CONCLUSIONS: Increased seizure burden and increased interhemispheric differences in both quantitative electroencephalographic amplitude and TCD MCA TIBI scores are independently associated with acute brain injury in children undergoing ECMO support.


Asunto(s)
Lesiones Encefálicas , Isquemia Encefálica , Oxigenación por Membrana Extracorpórea , Accidente Cerebrovascular Isquémico , Humanos , Niño , Oxigenación por Membrana Extracorpórea/efectos adversos , Oxigenación por Membrana Extracorpórea/métodos , Circulación Cerebrovascular/fisiología , Oximetría , Lesiones Encefálicas/etiología , Lesiones Encefálicas/terapia , Arteria Cerebral Media , Convulsiones , Estudios Retrospectivos
13.
Neurocrit Care ; 40(1): 65-73, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38062304

RESUMEN

BACKGROUND: The fundamental gap obstructing forward progress of evidenced-based care in pediatric and neonatal disorders of consciousness (DoC) is the lack of defining consensus-based terminology to perform comparative research. This lack of shared nomenclature in pediatric DoC stems from the inherently recursive dilemma of the inability to reliably measure consciousness in the very young. However, recent advancements in validated clinical examinations and technologically sophisticated biomarkers of brain activity linked to future abilities are unlocking this previously formidable challenge to understanding the DoC in the developing brain. METHODS: To address this need, the first of its kind international convergence of an interdisciplinary team of pediatric DoC experts was organized by the Neurocritical Care Society's Curing Coma Campaign. The multidisciplinary panel of pediatric DoC experts proposed pediatric-tailored common data elements (CDEs) covering each of the CDE working groups including behavioral phenotyping, biospecimens, electrophysiology, family and goals of care, neuroimaging, outcome and endpoints, physiology and big Data, therapies, and pediatrics. RESULTS: We report the working groups' pediatric-focused DoC CDE recommendations and disseminate CDEs to be used in studies of pediatric patients with DoC. CONCLUSIONS: The CDEs recommended support the vision of progressing collaborative and successful internationally collaborative pediatric coma research.


Asunto(s)
Investigación Biomédica , Elementos de Datos Comunes , Recién Nacido , Humanos , Niño , Estado de Conciencia , Coma/diagnóstico , Coma/terapia , Trastornos de la Conciencia/diagnóstico , Trastornos de la Conciencia/terapia
14.
J Clin Neurophysiol ; 41(3): 214-220, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37195275

RESUMEN

PURPOSE: Brain tissue hypoxia is associated with poor outcomes after pediatric traumatic brain injury. Although invasive brain oxygenation (PbtO 2 ) monitoring is available, noninvasive methods assessing correlates to brain tissue hypoxia are needed. We investigated EEG characteristics associated with brain tissue hypoxia. METHODS: We performed a retrospective analysis of 19 pediatric traumatic brain injury patients undergoing multimodality neuromonitoring that included PbtO 2 and quantitative electroencephalography(QEEG). Quantitative electroencephalography characteristics were analyzed over electrodes adjacent to PbtO 2 monitoring and over the entire scalp, and included power in alpha and beta frequencies and the alpha-delta power ratio. To investigate relationships of PbtO 2 to quantitative electroencephalography features using time series data, we fit linear mixed effects models with a random intercept for each subject and one fixed effect, and an auto-regressive order of 1 to model between-subject variation and correlation for within-subject observations. Least squares (LS) means were used to investigate for fixed effects of quantitative electroencephalography features to changes in PbtO 2 across thresholds of 10, 15, 20, and 25 mm Hg. RESULTS: Within the region of PbtO 2 monitoring, changes in PbtO 2 < 10 mm Hg were associated with reductions of alpha-delta power ratio (LS mean difference -0.01, 95% confidence interval (CI) [-0.02, -0.00], p = 0.0362). Changes in PbtO 2 < 25 mm Hg were associated with increases in alpha power (LS mean difference 0.04, 95% CI [0.01, 0.07], p = 0.0222). CONCLUSIONS: Alpha-delta power ratio changes are observed across a PbtO 2 threshold of 10 mm Hg within regions of PbtO 2 monitoring, which may reflect an EEG signature of brain tissue hypoxia after pediatric traumatic brain injury.


Asunto(s)
Lesiones Traumáticas del Encéfalo , Hipoxia Encefálica , Humanos , Niño , Estudios Retrospectivos , Oxígeno , Lesiones Traumáticas del Encéfalo/complicaciones , Lesiones Traumáticas del Encéfalo/diagnóstico , Hipoxia , Hipoxia Encefálica/etiología , Encéfalo , Electroencefalografía
15.
Neurocrit Care ; 39(3): 578-585, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37606737

RESUMEN

BACKGROUND: Electroencephalography (EEG) has long been recognized as an important tool in the investigation of disorders of consciousness (DoC). From inspection of the raw EEG to the implementation of quantitative EEG, and more recently in the use of perturbed EEG, it is paramount to providing accurate diagnostic and prognostic information in the care of patients with DoC. However, a nomenclature for variables that establishes a convention for naming, defining, and structuring data for clinical research variables currently is lacking. As such, the Neurocritical Care Society's Curing Coma Campaign convened nine working groups composed of experts in the field to construct common data elements (CDEs) to provide recommendations for DoC, with the main goal of facilitating data collection and standardization of reporting. This article summarizes the recommendations of the electrophysiology DoC working group. METHODS: After assessing previously published pertinent CDEs, we developed new CDEs and categorized them into "disease core," "basic," "supplemental," and "exploratory." Key EEG design elements, defined as concepts that pertained to a methodological parameter relevant to the acquisition, processing, or analysis of data, were also included but were not classified as CDEs. RESULTS: After identifying existing pertinent CDEs and developing novel CDEs for electrophysiology in DoC, variables were organized into a framework based on the two primary categories of resting state EEG and perturbed EEG. Using this categorical framework, two case report forms were generated by the working group. CONCLUSIONS: Adherence to the recommendations outlined by the electrophysiology working group in the resting state EEG and perturbed EEG case report forms will facilitate data collection and sharing in DoC research on an international level. In turn, this will allow for more informed and reliable comparison of results across studies, facilitating further advancement in the realm of DoC research.


Asunto(s)
Investigación Biomédica , Elementos de Datos Comunes , Humanos , Trastornos de la Conciencia/diagnóstico , Trastornos de la Conciencia/terapia , Recolección de Datos , Electrofisiología
16.
Crit Care Med ; 51(12): 1740-1753, 2023 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-37607072

RESUMEN

OBJECTIVES: To address areas in which there is no consensus for the technologies, effort, and training necessary to integrate and interpret information from multimodality neuromonitoring (MNM). DESIGN: A three-round Delphi consensus process. SETTING: Electronic surveys and virtual meeting. SUBJECTS: Participants with broad MNM expertise from adult and pediatric intensive care backgrounds. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two rounds of surveys were completed followed by a virtual meeting to resolve areas without consensus and a final survey to conclude the Delphi process. With 35 participants consensus was achieved on 49% statements concerning MNM. Neurologic impairment and the potential for MNM to guide management were important clinical considerations. Experts reached consensus for the use of MNM-both invasive and noninvasive-for patients in coma with traumatic brain injury, aneurysmal subarachnoid hemorrhage, and intracranial hemorrhage. There was consensus that effort to integrate and interpret MNM requires time independent of daily clinical duties, along with specific skills and expertise. Consensus was reached that training and educational platforms are necessary to develop this expertise and to provide clinical correlation. CONCLUSIONS: We provide expert consensus in the clinical considerations, minimum necessary technologies, implementation, and training/education to provide practice standards for the use of MNM to individualize clinical care.


Asunto(s)
Competencia Clínica , Adulto , Niño , Humanos , Consenso , Técnica Delphi , Encuestas y Cuestionarios , Estándares de Referencia
17.
Orphanet J Rare Dis ; 18(1): 225, 2023 08 03.
Artículo en Inglés | MEDLINE | ID: mdl-37537625

RESUMEN

BACKGROUND: Mutations in the NMDA receptor are known to disrupt glutamatergic signaling crucial for early neurodevelopment, often leading to severe global developmental delay/intellectual disability, epileptic encephalopathy, and cerebral palsy phenotypes. Both seizures and movement disorders can be highly treatment-refractory. RESULTS: We describe a targeted ABA n-of-1 treatment trial with intrathecal MgSO4, rationally designed based on the electrophysiologic properties of this gain of function mutation in the GRIN1 NMDA subunit. CONCLUSION: Although the invasive nature of the trial necessitated a short-term, non-randomized, unblinded intervention, quantitative longitudinal neurophysiologic monitoring indicated benefit, providing class II evidence in support of intrathecal MgSO4 for select forms of GRIN disorders.


Asunto(s)
Discapacidad Intelectual , Magnesio , Humanos , Discapacidad Intelectual/genética , Magnesio/metabolismo , Mutación/genética , Proteínas del Tejido Nervioso/genética , Receptores de N-Metil-D-Aspartato/genética , Convulsiones/genética , Estudios de Casos Únicos como Asunto
18.
World J Crit Care Med ; 12(3): 116-129, 2023 Jun 09.
Artículo en Inglés | MEDLINE | ID: mdl-37397588

RESUMEN

Acute neurologic injuries represent a common cause of morbidity and mortality in children presenting to the pediatric intensive care unit. After primary neurologic insults, there may be cerebral brain tissue that remains at risk of secondary insults, which can lead to worsening neurologic injury and unfavorable outcomes. A fundamental goal of pediatric neurocritical care is to mitigate the impact of secondary neurologic injury and improve neurologic outcomes for critically ill children. This review describes the physiologic framework by which strategies in pediatric neurocritical care are designed to reduce the impact of secondary brain injury and improve functional outcomes. Here, we present current and emerging strategies for optimizing neuroprotective strategies in critically ill children.

19.
Pediatr Neurol ; 146: 1-7, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37356227

RESUMEN

BACKGROUND: Pediatric neurocritical care (PNCC) has emerged as a field to care for children at the intersection of critical illness and neurological dysfunction. PNCC fellowship programs evolved over the past decade to train physicians to fill this clinical need. We aimed to characterize PNCC fellowship training infrastructure and curriculum in the United States and Canada. METHODS: Web-based survey of PNCC fellowship program leaders during November 2019 to January 2020. RESULTS: There were 14 self-identified PNCC fellowship programs. The programs were supported by Child Neurology and/or Pediatric Critical Care Medicine divisions at tertiary/quaternary care institutions. Most programs accepted trainees who were board-eligible or board-certified in child neurology or pediatric critical care medicine. Clinical training consisted mostly of rotations providing PNCC consultation (n = 13) or as a provider on the pediatric intensive care unit-based neurointensive care team (n = 2). PNCC-specific didactics were delivered at most institutions (n = 13). All institutions provided training in electroencephalography use in the intensive care unit and declaration of death by neurological criteria (n = 14). Scholarly activity was supported by most programs, including protected time for research (n = 10). CONCLUSIONS: We characterized PNCC fellowship training in the United States and Canada, which in this continuously evolving field, lays the foundation for exploring standardization of training going forward.


Asunto(s)
Cuidados Críticos , Becas , Niño , Humanos , Estados Unidos , Encuestas y Cuestionarios , América del Norte , Curriculum , Educación de Postgrado en Medicina
20.
Neurology ; 101(5): e546-e557, 2023 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-37295955

RESUMEN

BACKGROUND AND OBJECTIVES: The objective of this study was to determine patient-specific factors known proximate to the presentation to emergency care associated with the development of refractory convulsive status epilepticus (RSE) in children. METHODS: An observational case-control study was conducted comparing pediatric patients (1 month-21 years) with convulsive SE whose seizures stopped after benzodiazepine (BZD) and a single second-line antiseizure medication (ASM) (responsive established status epilepticus [rESE]) with patients requiring more than a BZD and a single second-line ASM to stop their seizures (RSE). These subpopulations were obtained from the pediatric Status Epilepticus Research Group study cohort. We explored clinical variables that could be acquired early after presentation to emergency medical services with univariate analysis of the raw data. Variables with p < 0.1 were retained for univariable and multivariable regression analyses. Multivariable logistic regression models were fit to age-matched and sex-matched data to obtain variables associated with RSE. RESULTS: We compared data from a total of 595 episodes of pediatric SE. Univariate analysis demonstrated no differences in time to the first BZD (RSE 16 minutes [IQR 5-45]; rESE 18 minutes [IQR 6-44], p = 0.068). Time to second-line ASM was shorter in patients with RSE (RSE 65 minutes; rESE 70 minutes; p = 0.021). Both univariable and multivariable regression analyses revealed a family history of seizures (OR 0.37; 95% CI 0.20-0.70, p = 0.0022) or a prescription for rectal diazepam (OR 0.21; 95% CI 0.078-0.53, p = 0.0012) was associated with decreased odds of RSE. DISCUSSION: Time to initial BZD or second-line ASM was not associated with progression to RSE in our cohort of patients with rESE. A family history of seizures and a prescription for rectal diazepam were associated with a decreased likelihood of progression to RSE. Early attainment of these variables may help care for pediatric rESE in a more patient-tailored manner. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that patient and clinical factors may predict RSE in children with convulsive seizures.


Asunto(s)
Epilepsia Refractaria , Estado Epiléptico , Humanos , Niño , Anticonvulsivantes/uso terapéutico , Estudios de Casos y Controles , Estudios Retrospectivos , Estado Epiléptico/tratamiento farmacológico , Benzodiazepinas/uso terapéutico , Convulsiones/tratamiento farmacológico , Epilepsia Refractaria/tratamiento farmacológico , Diazepam/uso terapéutico
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