Seminars in Pediatric Neurology最新文献

Long-stay patients are an impactful, vulnerable, growing group of inpatients in today's (and tomorrow's) tertiary hospitals. They can outlast dozens of clinicians that necessarily rotate on and off clinical service. Yet, care from such rotating clinicians can result in fragmented care due to a lack of continuity that insufficiently meets the needs of these patients and their families. Using long-stay PICU patients as an example, this focused review discusses the impact of prolonged admissions and how our fragmented care can compound this impact. It also argues that it is an ethical imperative to provide a level of continuity of care beyond what is considered standard of care and offers a number of strategies that can provide such continuity.

The recent emergence of promising therapies to treat neuromuscular diseases such as spinal muscular atrophy raises important questions regarding the ethical permissibility of allowing a parent to refuse these Food and Drug Administration-approved drugs. The 3 most recent drugs targeting spinal muscular atrophy have all been approved since 2019, lack long-term data regarding potential side-effects and long-term benefits, and are costly. Indeed, onasemnogene abeparvovec-xioi (Zolgensma) has been called the most expensive drug in the world. Contemporary analyses of innovative therapies, compassionate use medications, off-label usage, and emerging therapies tend to focus on the importance of informed consent in framing the ethical dimensions of these medications. This manuscript utilizes a narrative framework of “rescue” to explore the competing perspectives of optimistic physicians and parents, who may decline the therapies finding the benefit-burden profile does not weigh in favor of their use. Ultimately, this paper concludes that such refusal should be considered ethically permissible until such time as more long-term data are available for these medications and their cost has decreased substantially.

Since the concept of death by neurologic criteria (DNC) or “brain death” was articulated by the Harvard Ad Hoc Committee in 1968, efforts to establish and uphold DNC as equivalent to biologic death have been supported through federal and state legislation, professional guidelines, and hospital policies. Despite these endeavors, DNC remains controversial among bioethics scholars and clinicians and is not universally accepted by patient families and the public. In this focused review, we outline the current points of contention surrounding the diagnosis of DNC in pediatric patients. These include physiologic, legal, and philosophical inconsistencies in the definition of DNC, controversy regarding the components of the clinical exam, variability in clinical practice, and ethical concerns regarding justice and role of informed consent. By better understanding these controversies, clinicians may serve families grappling with the diagnosis of DNC more effectively, compassionately, and equitably.

The decisions around whether or not to provide tracheostomy and chronic mechanical ventilation to children with acute neurologic injury are difficult for medical providers and surrogate decision makers. Consideration of the 4 primary principles of medical ethics–autonomy, beneficence, non-maleficence, and justice–can provide a framework from which constructive discussions can form. Determination of the goals of care is a good first step in navigating these complex decisions. A shared decision model should be used, including education of decision-makers by medical providers and appropriate recommendations based on the stated goals of care. In this paper, 2 illustrative cases are discussed highlighting the utility of this decision-making framework.

Pediatric organ transplantation remains a life-saving therapy, with donated organs being absolutely scarce resources. Efforts to both increase pediatric organ donation authorization by families of children declared dead by neurologic criteria and mitigate perception of conflicts of interest have resulted in frequent exclusion of physicians from this process. This article provides of focused review of pediatric organ donation in the setting of brain death, explores the breadth of consequences of physician exclusion in donation authorization requests, and provides an ethical framework defending physician involvement in the organ donation process for this patient population.

Advancements in genetic testing in the healthcare setting, most recently genomic sequencing, has enhanced our ability to diagnose genetic conditions. These advances include increased accessibility and affordability of genomic technologies. With expanded use comes the potential for significant ethical challenges for clinicians, particularly considering the implications of testing a child for one condition and incidentally finding a different condition or health risk. In this focused review, we address various ethical considerations from informed consent to the rights of a child undergoing genetic testing.

The development of status myoclonus (SM) in a postcardiac arrest patient has historically been thought of as indicative of not only a poor neurologic outcome but of neurologic devastation. In many instances, this may lead clinicians to initiate conversations about withdrawal of life sustaining therapies (WLST) regardless of the time from return of spontaneous circulation (ROSC). Recent studies showing a percentage of patients may make a good recovery has called into question whether a self-fulfilling prophecy has developed where the concern for a poor neurologic outcome leads clinicians to prematurely discuss WLST. The issue is only further complicated by changing terminology, lack of neuro-axis localization, and limited data regarding association with electroencephalogram (EEG) characteristics, all of which could aid in the understanding of the severity of neurologic injury associated with SM. Here we review the initial literature reporting SM as indicative of poor neurologic outcome, the studies that call this into question, the various definitions of SM and related terms as well as data regarding association with EEG backgrounds. We propose that improved prognostication on outcomes results from combining the presence of SM with other clinical variables (eg EEG patterns, MRI findings, and clinical exam). We discuss the ethical implications of using SM as a prognostic tool and its impact on decisions about life-sustaining care in children following cardiac arrest. We advocate for prognostication efforts to be delayed for at least 72 hours following ROSC and thus to treat SM in those early hours and days.

Pediatric transplant centers are faced with the difficult task of maximizing the benefit of organs donated for transplantation while also ensuring that all patients undergoing transplant evaluation are fairly considered for this life-saving therapy. Children with neurodevelopmental disabilities are a complex patient population that on occasion may face the need for a solid organ transplant. Several concerns exist regarding transplantation in this population, yet standard transplant inclusion and exclusion criteria do not exist. Here we explore important factors regarding organ transplantation for children with neurodevelopmental disorders, including patient outcomes, quality of life considerations, and the fundamental ethical principles underlying this complex medical decision-making.

Rehabilitation for pediatric stroke survivors can maximize a child's potential through each developmental stage of life. Timely diagnosis and referral to a rehabilitation specialist may harness opportunities to maximize brain plasticity, to help children adapt and learn, and to participate in and enjoy daily life to their capability. The aim of this focused review is to explore current rehabilitation models and evidence-based interventions for pediatric stroke survivors in Low- and Middle- Income Countries (LMICs) and to provide recommendations for future research and focused areas of improvement.There are several published pediatric stroke guidelines from the American Heart Association (AHA), Canada, Australia, and the United Kingdom (UK) which provide specific recommendations for rehabilitation, although the suggested intervention and services vary. There are no current guidelines developed in or contextually adapted for LMICs, although the current pediatric stroke guidelines emphasize the need to develop interventions that fit the cultural and environmental contexts. The World Health Organization (WHO) Rehabilitation 2030 initiative acknowledges profound unmet rehabilitation needs around the world, especially in LMICs. According to the WHO, LMICs have less than ten skilled rehabilitation professionals per one million people. Enhancing the understanding of rehabilitation services in LMICs could lead to nationally supported workforce education initiatives targeted to expand the number of locally trained therapy providers. This could improve access to and delivery of quality rehabilitation interventions to pediatric stroke survivors in these settings.

Pediatric stroke is a condition that often results in life-long motor, cognitive, or sensory deficits for children. The purpose of this focused review is to compile the most recent literature on pediatric stroke neuromotor interventions and summarize evidence for use by rehabilitation providers and researchers. Terms including stroke, pediatric, and neuromotor were searched with appropriate MeSH terms. Information was collected regarding interventions conducted and outcome measures used for each article. Interventions and outcome measures were organized based on ICF components (Body Structure and Function, Activity, Participation, and Environmental Factors). 16 articles were included after full-text screens. From these 16 articles, a large majority of them included some form of neuromodulation as a part of intervention. Results identified a potentially problematic gap between domains addressed by interventions and measured by outcomes, with a need to include more expansive outcome measures in research studies. There are several areas of potential growth in pediatric stroke literature. Research studies should be precise when describing included samples. As interventions for pediatric stroke shift toward neuromodulation and other neurologic treatments, there is a need for well-defined populations, both clinically in the community as well as in research studies. There is also a need for US guidelines for rehabilitation after pediatric stroke. Overall, the trend in the literature seems to suggest that combining some form of neuromodulatory technique with existing recommended rehabilitation technique (ex: CIMT) may promote overall recovery for children after stroke, though further research is needed.