Recent progress in genetic analysis and therapeutics provide patients and families with a hope for the cure of intractable diseases. The current age also enforces pediatricians to think over the complex and diverse decision-making processes and therapeutic options for each patient. Presumably, this situation will become more complex within the range of legal and ethical consensus for therapeutic options that pediatricians must consider as advocates for ill children. We cannot simplify or streamline the process for how they support the interests of children and what they should protect. When standing at the critical point of decision-making for a life-threatening condition, pediatricians need to develop agreement with their patients and families through persistent discussions without setting up a goal for agreement among them. Thus, translational research in pediatrics may represent the search for essence deeply embedded in bioethics, which may possibly lead us to untangling the conundrums in clinical practice.

  Objective: This study aimed to examine the management of patients with clusters of seizures accompanied by fever (SaF) via investigation of their clinical characteristics and laboratory data. Methods: Patients with clusters of SaF admitted to Toyooka Public Hospital between 2011 and 2021 were retrospectively included and divided into two groups : Group A (seizures repeated only twice) and Group B (seizures repeated thrice or more). Those who had epilepsy, underlying disorders associated with seizures, and convulsive status epilepticus were excluded. Results: Overall, 223 patients were included, with 169 in Group A and 54 in Group B. All included patients exhibited no abnormal neurological findings at the time of discharge. The length of hospital stay was longer in Group B than in Group A. Group B also had higher AST/ALT and lower blood glucose levels than Group A (P＜0.05). Seizure suppression rate after the administration of diazepam (DZP) suppository was 80% in patients who used DZP after second seizure and 90.9% in those who used it after three or more seizure clusters. The seizure suppression rate after the third seizure was not significantly different between those with and without DZP suppository use. Conclusions: Approximately one-fourth of children with SaF had three or more clusters of seizures. Although the serum AST/ALT level may be a useful marker for prediction of seizure cluster, further investigations are required owing to its low sensitivity and specificity. Moreover, the effect of DZP suppository for the purpose of suppressing seizure clusters remains unknown. Indications of hospitalization and intervention for patients with SaF should be considered depending on the access to the hospital, medical resources in each region, and burden on caregivers of patients.

  Objective: Professionals for children with neurodevelopmental disorders are shorthanded, and this leads to a long duration of contact with professionals. In this study, we examined the perspectives of general pediatricians and professionals on the real and ideal states of medical resources for children with neurodevelopmental disorders and discussed the appropriate medical resources and the problem of the long duration of contact with professionals. Methods: We performed the online survey, and a total of 808 pediatricians and psychiatrists participated in the survey. We tested the difference between perspectives of general pediatricians and professionals and the difference between the real and ideal states of medical resources. Results: For children with attention-deficit hyperactivity disorder (ADHD) or autism spectrum disorder (ASD) who did not need intervention, needed medication, and needed psychosocial intervention, there were significantly higher rates by which general pediatricians and professionals responded that the children attended the outpatient services for neurodevelopmental disorders in the actual situation than in the ideal situation. Professionals less frequently responded that children with ADHD/ASD who needed medication attended the general outpatient services and both general and neurodevelopmental disorders’ outpatient services on the actual situation than the ideal situation. Conclusions: We considered that the regular treatment of children with neurodevelopmental disorders was concentrated on the outpatient services for neurodevelopmental disorders in an actual setting. Professionals seemed to expect medication for children with neurodevelopmental disorders to be prescribed in the general outpatient services. Thus, it is necessary that feasible treatments are provided in the general outpatient services. We considered that such practice is associated with the appropriate allocation of medical resources and is an important factor to alleviate the problem of the long duration of contact with professionals.

  Myelomeningocele (MMC) is the most severe form of spina bifida that presents a variety of symptoms based on the spinal cord lesions of MMC, hydrocephalus and Chiari II malformation. Management of respiratory complications is important for prolonging the life expectancy of patients with MMC during childhood. Recent studies indicate a high prevalence of sleep-related breathing disorders (SRBD) in MMC patients. We encountered an 8-year-old girl with MMC located in the thoracic to sacral spine, who underwent neurosurgery at 6 and 7 years of age for treatment of Chiari II malformation. We under-recognized SRBD in MMC and did not perform routine respiratory evaluation. Respiratory evaluation was finally performed because of choking secondary to difficulty in expectoration and oxygen desaturation at 8 years of age. An interview and the results of a sleep questionnaire were insufficient to detect SRBD. A portable monitor showed loss of breathing effort during apnea, 3% oxygen desaturation index (ODI) of 70.9 and apnea hypopnea index (AHI) of 12, indicating that the patient had central sleep apnea syndrome. Non-invasive positive pressure ventilation therapy resulted in a 3%ODI of 1 and AHI of 1. Her respiratory status was stable thereafter. Routine evaluation of SRBD is important in the health management of MMC. Although there are comprehensive guidelines regarding SRBD in the care of people with spina bifida overseas, there is no such guideline in Japan. For adequate health care management, comprehensive guidelines for MMC are required in Japan as well.

  Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headache onset and multifocal segmental cerebral vasoconstriction. This syndrome is seen predominantly in women between 20 and 50 years old. Although its prognosis is good, there are various complications of RCVS, including intracerebral hemorrhaging, subarachnoid hemorrhaging (SAH), and posterior reversible encephalopathy syndrome. Previously healthy 7- and 12-year-old boys developed cortical SAH after a thunderclap headache. Cranial computed tomography angiography and cranial magnetic resonance angiography indicated multifocal segmental cerebral vasoconstriction. RCVS was diagnosed from the clinical course and these neuroimaging findings. One was treated with calcium channel blockers, and neither had neurological sequelae. RCVS should be considered in the differential diagnosis of pediatric patients complaining of thunderclap headaches or developing cortical SAH. It is important to perform neuroimaging studies serially for these patients.

  We describe the case of an 8-year-old Nepalese boy who was admitted to the intensive care unit (ICU) of our hospital with a second-degree burn. One day after admission, the patient developed a fever, followed by diarrhea, conjunctival hyperemia, and systemic erythema. On the third day, after developing disturbed consciousness and clustered convulsions, the patient suddenly developed severe hypotension and multiple organ failure. Tracheal intubation was performed, and both antibiotics and immunoglobulin were administered. Toxic shock syndrome toxin 1-producing methicillin-resistant Staphylococcus aureus was isolated from the burn wound culture, confirming a diagnosis of toxic shock syndrome (TSS). However, after recovering from the acute shock, the patient developed persistently disturbed consciousness, ataxia, action tremor, and systemic muscle weakness predominantly in the lower limb. Mild atrophy of the cerebrum and cerebellum on brain MRI, hypoperfusion of the parietal lobe on brain SPECT, and abnormal peripheral nerve conduction studies in the lower extremities were revealed. These findings led to a diagnosis of acute encephalopathy and ICU-acquired weakness associated with TSS. Although the muscle weakness improved at discharge, the ataxia and action tremor remained. Furthermore, higher brain dysfunction and borderline intellectual functioning persisted. Recent studies have reported TSS patients with encephalopathy. The present case illustrates the variation in clinical presentation, brain imaging, and neurological prognosis of encephalopathy associated with TSS. We suggest that an appropriate evaluation of possible acute encephalopathy, with mid-and long-term neurological follow-up, is necessary for patients with TSS.

  A 2-year-old girl experienced prolonged consciousness disturbance followed by status epilepticus with a fever from coronavirus disease 2019 (COVID-19). A brain MRI revealed cerebral subcortical reduced diffusion known as bright tree appearance, based on which she was diagnosed with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). She was treated with intravenous high-dose methylprednisolone and subsequent intramuscular thyrotropin-releasing hormone. AESD should be recognized as a differential diagnosis in patients with febrile status epilepticus associated with COVID-19.