A Case of Anorexia Nervosa with Focal Cortical Dysplasia.

Anorexia nervosa (AN) is a fatal condition associated with extreme underweight and undernutrition. It is more common in young females, with a female-to-male ratio of 10 : 1. Focal cortical dysplasia (FCD) is characterized by dysplasia of the cerebral cortex and is a common cause of pharmacoresistant epilepsy. However, FCD associated with AN has never been reported. We report the first case of AN in a 12-year-old male diagnosed with FCD-type 2 on head magnetic resonance imaging (MRI). He became concerned about lower abdominal distention and began reducing his food intake. He was admitted to our hospital after weight loss of 10 kg in a 1 year. Head MRI showed a localized high-signal area from the cortex to the white matter of the fusiform gyrus near the left hippocampus, with no associated decreased blood flow or electroencephalography (EEG) abnormalities. These findings were characteristic of FCD type II. In males with AN, the search for underlying disease is particularly important. The pathophysiology of the association between AN and FCD is unclear. However, both conditions are reportedly associated with autism spectrum disorder. Further cases are needed to clarify whether FCD is associated with eating disorders.

Effectiveness of ultrasound-guided peripheral arterial cannulation in neonates, including very low birth weight infants who are conventionally difficult-to-cannulate: a case series.

The effectiveness of ultrasound-guided peripheral arterial cannulation (UGPAC) in children has been increasingly been reported. However, to the best of our knowledge, there have been no reports of UGPAC in neonates, including very low birth weight infants (VLBWIs). In this study, we aimed to retrospectively review the results of UGPAC in neonates, including VLBWIs, and assess its effectiveness. This case series was conducted in a tertiary neonatal intensive care unit (NICU) in Japan. We included neonates aged below 28 days who underwent UGPAC in our NICU between April 2021 and October 2022. We extracted the following data from medical records and analysed it retrospectively: patient age (days), postconceptional age, patient weight at the time of cannulation, number of punctures using the conventional technique before ultrasound guidance was performed and number of punctures with the ultrasound-guided technique until successful cannulation. A total of 27 UGPACs were performed in 19 neonates, including 14 cannulations in 10 VLBWIs. In infants weighing > 1500 g and VLBWIs, the success rate within the first three punctures was 100% (13/13) and 79% (11/14), respectively. Overall, 41% (11/27) of UGPACs were performed following failed punctures using conventional methods, with a 100% success rate within the first three attempts. In all cases, no apparent adverse events, such as hypothermia, were noted.  Conclusions: Our results suggest that UGPAC had a high success rate in neonates, including VLBWIs. Further studies are required to compare the effectiveness of UGPAC with conventional methods in neonates. What is Known: • The use of ultrasound guidance for arterial cannulation is recommended in children. • Ultrasound-guided peripheral arterial cannulation (UGPAC) in neonates, including very low birth weight infants (VLBWIs), has not been reported. What is New: • UGPAC in neonates, including VLBWIs, was performed with a high success rate; approximately 40% of UGPACs were performed after the failure of the conventional methods. • This study suggested the effectiveness of UGPAC in neonates, including VLBWIs.

General Anesthesia With Remimazolam and Peripheral Nerve Blocks Is Useful for Femoral Surgery in Patients With Severe Aortic Stenosis: A Case Report.

Traditional intravenous anesthetics and opioid analgesics are susceptible to inducing hemodynamic instability. Herein, we describe a case of open reduction and internal fixation of a femoral neck fracture in a patient with severe aortic stenosis. General anesthesia was accomplished using remimazolam, an intravenous anesthetic devoid of hemodynamic instability properties, in combination with a peripheral nerve block. During the surgical procedure, the need for circulatory agonist was reduced to a single dose, and satisfactory pain management was achieved. This approach represents an alternative method for patients with circulatory risk undergoing femoral surgery.

Verifying the Japanese version of the Healthy Aging Brain Care Monitor self-report tool for evaluating post-intensive care syndrome.

BACKGROUND: Post-intensive care syndrome (PICS) requires the use of multiple assessment tools because it affects multiple domains: Cognitive, Functional, and Behavioural/Psychological. Therefore, this study translated the self-report (SR) version of the Healthy Aging Brain Care Monitor (HABC-M), spanning multiple domains, into Japanese and analysed its reliability and validity in a post-intensive care setting. METHODS: Patients aged 20 years or older and admitted to the adult intensive care unit from August 2019 to January 2021 were included and surveyed by questionnaire. The 21-item Dementia Assessment Sheet for the Regional Comprehensive Care System was used to validate cognitive and physical aspects, and the Patient Health Questionnaire-9, the Generalized Anxiety Disorder-7, and the Post Traumatic Stress Disorder Checklist for Diagnostic and Statistical Manual of Mental Disorders 5th edition were used to validate emotional aspects. Reliability was assessed by Cronbach's alpha, and congruent validity by correlation analysis. Multivariate linear regression models were used to identify potential factors for PICS. RESULTS: A total of 104 patients (mean age: 64 ± 14 years) with 3 median mechanical ventilation days (interquartile range: 2-5) were enrolled. The Cognitive domain of the HABC-M SR was highly correlated with memory and disorientation (r = 0.77 for each), while the Functional domain was highly correlated with Instrumental Activities of Daily Living Scale (r = 0.75-0.79). The Behavioural/Psychological domain highly correlated with the Patient Health Questionnaire-9, Generalized Anxiety Disorder-7, and Post Traumatic Stress Disorder Checklist for Diagnostic and Statistical Manual of Mental Disorders 5th edition (r = 0.75-0.76). Multivariate analysis revealed longer ICU stays associated with lower Cognitive and Functional (p = 0.03 for each) domains and longer mechanical ventilation days with a lower Behavioural/Psychological domain (p < 0.01). CONCLUSION: The translated Japanese HABC-M SR showed high validity for assessment of the Cognitive, Functional, and Behavioural/Psychological domains of PICS. Therefore, we recommend that the Japanese version of the HABC-M SR be routinely used in the assessment of PICS.

Initial manifestations in Patients with Inborn Errors of Immunity Based on Onset Age: a Study from a Nationwide Survey in Japan.

PURPOSE: Patients with inborn errors of immunity (IEI) manifest various initial symptoms; however, those that are critical for the early diagnosis of IEI have not been identified. Also, the significance of the ten warning signs of primary immunodeficiency (PID) among infants has not been established. This study aimed to conduct a nationwide survey of IEI in Japan and investigated the initial manifestations based on onset age. METHODS: Among 1298 patients, data regarding the initial manifestation were available from 505 patients. Patients with autoinflammatory diseases, complement deficiency, and phenocopies of IEI were excluded. RESULTS: The ten warning signs were positive in 67.3% of the cases. The positivity rate was low (20.5%) in patients with immune dysregulation. Although the positivity rate was low (36.6%) in patients aged less than 3 months, they were highly positive for family history of IEI (26.8%). Infectious symptoms were the most commonly observed in all age groups and in all disease categories. Symptoms of "immune dysregulation" were present in approximately 15% of the patients. Regarding the anatomical category, almost all initial symptoms were "systemic" infections in patients with X-linked severe combined immunodeficiency. Moreover, "respiratory" symptoms were the most common in patients with IEI aged ≥ 1 year and accounted for more than 50% in all age groups in patients with common variable immunodeficiency. CONCLUSION: These results highlight the significance of the 10 warning signs and may serve as clinical indicators for early diagnosis, considering the initial presentation of IEI.

Validation of the Japan Coma Scale for the prediction of mortality in children: analysis of a nationwide trauma database.

Objective: The Japan Coma Scale (JCS) is widely used in clinical practice to evaluate levels of consciousness in Japan. There have been several studies on the usefulness of JCS in adults. However, its usefulness in evaluating children has not been reported. Therefore, this study aimed to assess the usefulness of the JCS for the prediction of mortality in children. Methods: This is a multicenter cohort study which used data from a national trauma registry (Japan Trauma Data Bank). This study included patients under 16 years of age who were treated between 2004 and 2015.The primary outcome measure was in-hospital mortality. Two models were used to examine each item of the Glasgow Coma Scale (GCS) and the JCS. Model A included the discrete levels of each index. In model B, data regarding age, sex, vital signs on arrival to hospital, the Injury Severity Score, and blunt trauma were added to each index. The effectivity of the JCS score was then evaluated using the area under the curve (AUC) for discrimination, a calibration plot, and the Hosmer-Lemeshow test for calibration. Results: A total of 9045 patients were identified. The AUCs of the GCS and JCS were 0.929 (95% confidence interval (CI) 0.904 to 0.954) and 0.930 (95% CI 0.906 to 0.954) in model A and 0.975 (95% CI 0.963 to 0.987) and 0.974 (95% CI 0.963 to 0.985) in model B, respectively. The results of the Hosmer-Lemeshow test were 0.00 (p=1.00) and 0.00 (p=1.00) in model A and 4.14 (p=0.84) and 8.55 (p=0.38) in model B for the GCS and JCS, respectively. Conclusions: We demonstrated that the JCS is as valid as the GCS for predicting mortality. The findings of this study indicate that the JCS is a useful and relevant tool for pediatric trauma care and future research.

Vaccination for Patients with Inborn Errors of Immunity: a Nationwide Survey in Japan.

We conducted a nationwide survey of inborn errors of immunity (IEI) in Japan for the second time in 10 years, focusing on protective measures for IEI patients against infectious diseases. Questionnaires were sent to various medical departments nationwide, and a total of 1307 patients were reported. The prevalence of IEI was 2.2 patients per 100,000 population, which was comparable with the previous nationwide study. The most common disease category was autoinflammatory disorders (25%), followed by antibody deficiencies (24%) and congenital defects of phagocyte number or function (16%). We found that a significant number of patients received contraindicated vaccines, principally because the patients were not diagnosed with IEI by the time of the vaccination. Regarding diseases for which BCG vaccination is contraindicated, 43% of patients had actually received BCG, of which 14% developed BCG-related infections. BCG-related infections were mainly observed among patients with CGD and MSMD. In order to prevent IEI patients from receiving inadequate vaccines, continuous education to parents and physicians is needed, along with the expansion of newborn screening, but efforts to screen IEI at the site of vaccination also remain important.

Association between off-hour admission of critically ill children to intensive care units and mortality in a Japanese registry.

Limited information exists regarding the effect of off-hour admission among critically ill children. To evaluate whether children admitted to intensive care units (ICUs) in off-hour have worse outcomes, we conducted a cohort study in 2013-2018 in a multicenter registry in Japan. Pediatric (age < 16 years) unplanned ICU admissions were divided into regular-hour (daytime on business days) or off-hour (others). Mortality and changes in the functional score at discharge from the unit were compared between the two groups. We established multivariate logistic regression models to examine the independent association between off-hour admission and outcomes. Due to the small number of outcomes, two different models were used. There were 2512 admissions, including 757 for regular-hour and 1745 for off-hour. Mortality rates were 2.4% (18/757) and 1.9% (34/1745) in regular-hour and off-hour admissions, respectively. There was no significant association between off-hour admission and mortality both in model 1 adjusting for age, sex, and Pediatric Index of Mortality 2 (adjusted odds ratio [aOR] 0.89, 95% confidence interval [CI] 0.46-1.72) and in model 2 adjusting for propensity score predicting off-hour admission (aOR 1.05, 95% CI 0.57-1.91). In addition, off-hour admission did not show an independent association with deterioration of functional score.

Development of the Japanese version of the Intensive Care Unit Trigger Tool to detect adverse events in critically ill patients.

AIM: The Intensive Care Unit Trigger Tool (ICUTT) was developed to detect adverse events (AEs) in intensive care unit (ICU) patients. The purpose of this study was to determine the validity and reliability of the Japanese version of the ICUTT (ICUTT-J). METHODS: The translation of ICUTT was carried out based on the guideline for translation of instruments. Subsequently, two review teams independently reviewed 50 patients' medical records using the ICUTT-J, and agreement regarding the presence and number of AEs was evaluated to ensure reliability. RESULTS: The ICUTT-J was submitted to the authors of the original ICUTT, who confirmed it as being equivalent to the original version. The item-content validity index and scale-content validity index were 1.00 and 1.00, respectively. Interrater reliability showed moderate agreement of κ = 0.52 in terms of the presence of AEs and linear weighting of κ = 0.49 (95% confidence interval, 0.28, 0.71) in terms of the number of AEs. CONCLUSION: This study's findings suggest that the ICUTT-J is valid and moderately reliable for use in ICUs.

Dexmedetomidine use and mortality in mechanically ventilated patients with severe burns: A cohort study using a national inpatient database in Japan.

BACKGROUND: Dexmedetomidine is an alpha 2-adrenergic receptor agonist. Apart from its sedative effects, dexmedetomidine can potentially reduce mortality through its anti-inflammatory effect. However, the impact of dexmedetomidine on in-hospital outcomes of patients with severe burns remains unclear. Therefore, we aimed to elucidate the association between dexmedetomidine use and mortality in mechanically ventilated patients with severe burns, using a Japanese nationwide database of in-hospital patients. METHODS: We included adults with severe burns (burn index ≥ 10) who were registered in the Japanese Diagnosis Procedure Combination national inpatient database from 2010 to 2018, started mechanical ventilation within 3 days of admission, and received any sedative drug (dexmedetomidine, midazolam, or propofol). One-to-one propensity score matching was performed between patients who received dexmedetomidine on the day of mechanical ventilation initiation (dexmedetomidine group) and those who did not receive dexmedetomidine (control group). The primary outcome was all-cause 30-day in-hospital mortality. Secondary outcomes were length of hospital stay and duration of mechanical ventilation in patients and survivors. RESULTS: Eligible patients (n = 1888) were classified into the dexmedetomidine group (n = 371) or the control group (n = 1517). After one-to-one propensity score matching, we compared 329 patients from both groups. No significant difference was observed in the 30-day mortality between patients in the dexmedetomidine and control groups (22.8% vs. 22.5%, respectively; odds ratio, 1.02; 95% confidence interval, 0.71-1.46). Moreover, there were no significant differences between patients in the dexmedetomidine and control groups in terms of the length of hospital stay or the duration of mechanical ventilation. CONCLUSIONS: We found no significant association between dexmedetomidine use and in-hospital outcomes (mortality, length of hospital stay, and length of mechanical ventilation) in mechanically ventilated patients with severe burns. Dexmedetomidine use may not improve the aforementioned outcomes; therefore, its selection should be based on the patient's general condition and the target level of sedation.

Abdominal aortic thrombus formation in a neonate with an interrupted aortic arch.

We note the risk of paradoxical embolism in patients with congenital heart defects with a right-to-left shunt. These patients should be managed to ensure that abdominal aortic thrombi are not overlooked when their clinical conditions change.

A Follow-Up Report of an Infant Born to a Mother Receiving Tamoxifen.

Tamoxifen, an estrogen receptor antagonist, is contraindicated in pregnant women due to its teratogenic activity. In the present study, we report the case of an infant whose mother received tamoxifen for breast cancer while unaware of the pregnancy. The infant, born at 29 weeks and 6 days of gestational age with a birth weight of 1664 g, had no congenital anomalies. This case presents detailed information on the development of an infant with placental transfer of tamoxifen. The infant has grown and developed normally throughout a 5-year follow-up period, but long-term vigilance continues.

The Validity and Reliability of the Japanese Version of the Cornell Assessment of Pediatric Delirium.

OBJECTIVES: To examine validity and reliability of the Japanese version of the Cornell Assessment of Pediatric Delirium. DESIGN: Prospective double-blinded observational cohort study. SETTING: Eight-bed mixed PICU (post-surgical and internal medicine) from May 2017 to June 2018. PATIENTS: All children between the ages of 0-13 years who were admitted to the PICU for at least 24 hours were eligible for inclusion, as long as the child was arousable to verbal stimulation. INTERVENTIONS: Two nurses simultaneously and independently assessed each patient for pediatric delirium with the Japanese version of the Cornell Assessment of Pediatric Delirium; this was compared to the gold standard of psychiatric diagnosis based on Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition standards. MEASUREMENTS AND MAIN RESULTS: Forty-one children were enrolled and 92 assessments (ranging from one to four per subject) were completed. Congenital heart disease patients accounted for 73 enrollees (79%). Forty-three percent of observations were performed in children on invasive mechanical ventilation. Pediatric delirium prevalence (as determined by psychiatric diagnosis) was 53%. The Japanese version of the Cornell Assessment of Pediatric Delirium demonstrated an optimal scoring cutpoint of 9. Overall, area under the curve was 92%, sensitivity 90% (95% CI, 79-97%), specificity 88% (95% CI, 75-96%), positive predictive value of 90% (95% CI, 79-97%), negative predictive value of 91% (95% CI, 80-97%), and a Cohen's κ of 0.89 (95% CI, 0.8-0.98). In children on invasive mechanical ventilation, the Japanese version of the Cornell Assessment of Pediatric Delirium maintained an area under the curve 87%, sensitivity 97%, and specificity 64%. CONCLUSIONS: The Japanese version of the Cornell Assessment of Pediatric Delirium is a valid and reliable tool for use in Japanese PICUs. This will allow for detection of delirium in real-time and may lead to better identification of the population and risk factors for appropriate management and therapeutic and preventative interventions.

Outcomes of paediatric out-of-hospital cardiac arrest according to hospital characteristic defined by the annual number of paediatric patients with invasive mechanical ventilation: A nationwide study in Japan.

AIM: We examined whether outcomes of paediatric out-of-hospital cardiac arrest (OHCA) are associated with a hospital characteristic defined by the annual number of invasive mechanical ventilation cases, suggesting hospitals' experience in caring for severely ill paediatric patients. METHOD: We analysed the Japanese Diagnosis Procedure Combination database from 2010 to 2017. We identified children (<18 years) with OHCA and post-resuscitation intensive care (defined as invasive mechanical ventilation and/or catecholamine infusion). Hospitals were divided into four groups by mean annual number of paediatric cases involving invasive mechanical ventilation. The primary outcome was in-hospital mortality, and the secondary outcome was unfavourable outcomes (death or medical care dependency at discharge). Multivariable logistic regression analyses were conducted to examine the relationship between hospitals' experience and outcomes. RESULTS: We included 2540 paediatric OHCA patients from 385 institutions. Overall in-hospital mortality was 62.4%, with rates of 69.6%, 61.3%, 61.8%, and 57.0% in hospitals with low (≤48 cases/year), low-intermediate (48-110), high-intermediate (110-164), and high (>164) experience levels (P < .001), respectively. Compared to hospitals with low experience, adjusted odds ratios (95% confidence interval) for hospitals with low-intermediate, high-intermediate, and high experience were as follows: primary outcome: 0.64 (0.40-1.01), 0.67 (0.42-1.05), and 0.46 (0.31-0.70), respectively; secondary outcome: 0.93 (0.55-1.57), 0.95 (0.63-1.43), and 0.67 (0.46-0.96), respectively. CONCLUSION: Japanese hospitals with higher experience in caring for severely ill paediatric patients showed lower mortality for paediatric OHCA. This fact should be considered by the Emergency Medical Systems when deciding transport strategy.

Verifying the Japanese version of the Preschool Confusion Assessment Method for the ICU (psCAM-ICU).

AIM: Pediatric delirium has been well investigated and its prevalence is reported to be from 20% to 44%. For pediatric intensive care settings, several validated assessment tools for diagnosing delirium, including the Preschool Confusion Assessment Method for the Intensive Care Unit (psCAM-ICU), are available in English. However, validated assessment tools for identifying pediatric delirium are unavailable in Japanese. Therefore, the aim of this study is to verify the Japanese translation of the psCAM-ICU. METHODS: We enrolled patients at the Pediatric ICU at University of Tsukuba Hospital (Tsukuba, Japan) from May 2017 to February 2019. Enrollment criteria included patients aged 6 months to 5 years, and we excluded coma patients scoring under -4 on the Richmond Agitation-Sedation Scale or suffering from stroke. Pediatric patient delirium was simultaneously evaluated by three medical workers (pediatric intensivist and researchers). Psychiatrists then verified these findings against criteria of the Diagnostic and Statistical Manual of Mental Disorders - 5th Edition. We evaluated criterion validity (sensitivity and specificity) and reliability using Cohen's κ coefficient. RESULTS: We made a total of 56 independent assessments of 19 patients (42% female) with an average age of 18 (±15) weeks. Mechanical ventilation was used at least once in 73% of patients and the positive rate of delirium was 54% in total observation. Overall, the psCAM-ICU showed high sensitivity, specificity (sensitivity, 0.90 [95% confidence interval [CI], 0.80-0.94]; specificity, 0.93 [95% CI, 0.83-0.97]), and high reliability within the researcher assessments (κ = 0.92; 95% CI, 0.82-1.0). CONCLUSION: We verified the psCAM-ICU and it shows high validity and reliability.

Development of the Japanese version of the State Behavioral Scale for critically ill children.

AIMS: The State Behavioral Scale (SBS) was developed to assess sedation states, including agitation, in pediatric patients on mechanical ventilation. The purpose of this study was to determine the reliability and validity of a back-translated Japanese version of the SBS. METHODS: Translation was done by the back-translation method followed by a prospective study in a Japanese intensive care unit. For reliability, a nurse/researcher pair evaluated SBS along eight dimensions (respiratory drive, response to ventilation, coughing, best response to stimulation, attentiveness to care provider, tolerance to care, consolability, and movement after consoled). For validity, SBS scores were compared to the Richmond Agitation-Sedation Scale and a visual analog scale (VAS). RESULTS: The original author approved the back-translated SBS. Thirty-one patients aged 0 weeks to 8 years were evaluated from 59 total critical pediatric patient encounters. The researcher and nurse SBS scores demonstrated excellent inter-rater reliability (weighted κ = 0.96, 95% CI 0.92-0.99). In addition, there was a very strong correlation between the researcher and nurse VAS scores (ρ = 0.80, P < 0.001). Weighted kappa coefficients for the eight dimensions ranged from 0.71 (95% confidence interval, 0.55-0.88; consolability) to 0.89 (95% confidence interval, 0.80-0.98; best response to stimulation). In validity testing, nurse SBS and nurse VAS scores were strongly correlated (ρ = 0.80, P < 0.001) with the researcher SBS and researcher Richmond Agitation-Sedation Scale scores (ρ = 0.91, P < 0.001). CONCLUSION: This study suggests that our Japanese version of the SBS is valid and reliable for evaluating sedation for critically ill children.