Usefulness of deep sedation with intravenous dexmedetomidine and midazolam in cardiac catheterization procedures for pediatric patients.

Background: Dexmedetomidine (DEX) is a highly selective alpha 2 receptor agonist that has the advantage of causing less respiratory depression than other sedative agents. We evaluated the add-on effects of DEX on sedation among pediatric patients who received midazolam and pentazocine during cardiac catheterization.

Methods: 120 cardiac catheterization procedures in 110 patients under deep sedation at Department of Pediatrics, Kanazawa University Hospital from January 2013 to August 2018: 63 procedures without DEX (i.

Feasibility and reliability of the pandemic-adapted online-onsite hybrid graduation OSCE in Japan.

Objective structured clinical examination (OSCE) is widely used to assess medical students' clinical skills. Virtual OSCEs were used in place of in-person OSCEs during the COVID-19 pandemic; however, their reliability is yet to be robustly analyzed. By applying generalizability (G) theory, this study aimed to evaluate the reliability of a hybrid OSCE, which admixed in-person and online methods, and gain insights into improving OSCEs' reliability.

The utility of zebrafish cardiac arrhythmia model to predict the pathogenicity of KCNQ1 variants.

Genetic testing for inherited arrhythmias and discriminating pathogenic or benign variants from variants of unknown significance (VUS) is essential for gene-based medicine. KCNQ1 is a causative gene of type 1 long QT syndrome (LQTS), and approximately 30% of the variants found in type 1 LQTS are classified as VUS. We studied the role of zebrafish cardiac arrhythmia model in determining the clinical significance of KCNQ1 variants.

Electrocardiographic Diagnosis of Hypertrophic Cardiomyopathy in the Pre- and Post-Diagnostic Phases in Children and Adolescents.

Background: The usefulness of electrocardiographic (ECG) voltage criteria for diagnosing hypertrophic cardiomyopathy (HCM) in pediatric patients is poorly defined.

Methods and results: ECGs at the 1st grade (mean [±SD] age 6.6±0.

A significance of school screening electrocardiogram in the patients with ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy and is associated with high morbidity and mortality. However, the role and significance of school screening for LVNC have not been fully elucidated. In this multicenter, retrospective cohort study, a total of 105 children with LVNC were included from 2000 to 2017.

Characteristics of induced pluripotent stem cells from clinically divergent female monozygotic twins with Danon disease.

Rationale: Induced pluripotent stem cells (iPSCs) have been generated from patients with various forms of disease, including Danon disease (DD); however, few reports exist regarding disease-specific iPSCs derived from clinically divergent monozygotic twins.

Objective: We examined the characteristics of iPSCs and iPSC-derived cardiomyocytes (iPSC-CMs) generated from clinically divergent monozygotic female twins with DD.

Methods And Results: We generated iPSCs derived from T-cells isolated from clinically divergent, 18-year-old female twins with DD harboring a mutation in LAMP2 at the intron 6 splice site (IVS6+1_4delGTGA).

Tumor necrosis factor-α modifies the effects of Shiga toxin on glial cells.

Shiga toxin (STX) is one of the main factors inducing hemorrhagic colitis and hemolytic-uremic syndrome (HUS) in infections with STX-producing Escherichia coli (STEC). Approximately 62% of patients with HUS showed symptoms of encephalopathy in the 2011 Japanese outbreak of STEC infections. At that time, we reported elevated serum concentrations of tumor necrosis factor (TNF)-α in patients with acute encephalopathy during the HUS phase.

The Effectiveness of Remote Facilitation in Simulation-Based Pediatric Resuscitation Training for Medical Students.

Objectives: To assess the effectiveness of pediatric simulation by remote facilitation. We hypothesized that simulation by remote facilitation is more effective compared to simulation by an on-site facilitator. We defined remote facilitation as a facilitator remotely (1) introduces simulation-based learning and simulation environment, (2) runs scenarios, and (3) performs debriefing with an on-site facilitator.

Impact of Updated Diagnostic Criteria for Long QT Syndrome on Clinical Detection of Diseased Patients: Results From a Study of Patients Carrying Gene Mutations.

Objectives: In this study, we scored patients with long QT syndrome (LQTS) according to the different Schwartz diagnostic criteria from 1993, 2006, and 2011, and to examine the validation of the criteria in relevance to the frequency of LQTS-related gene mutation.

Background: Although updated diagnostic criteria have been used in clinical settings, few data exist regarding their impact on the diagnosis of LQTS.

Methods: We used a cohort of 132 patients who presented with prolonged QTc intervals and/or abnormal clinical history in cardiac screening and who underwent exercise stress testing.

Early diagnosis of Danon disease: Flow cytometric detection of lysosome-associated membrane protein-2-negative leukocytes.

Introduction: Danon disease is an extremely rare X-linked dominant disorder characterized by progressive cardiomyopathy, muscle weakness, and mild mental retardation. Most cases harbor nonsense, frameshift, or splice-site mutations in LAMP2 that result in lysosome-associated membrane protein-2 (LAMP-2) deficiency and lysosomal defects. The identification of LAMP2 mutations makes it possible to detect female carriers with significant cardiomyopathy.

Circumstances and outcomes of out-of-hospital cardiac arrest in elementary and middle school students in the era of public-access defibrillation.

Background:  Circumstances and outcomes of out-of-hospital cardiac arrest (OHCA) in elementary and middle school students while at school in the era of public-access defibrillation are unknown.

Methods And Results:  We conducted a nationwide hospital-based survey of elementary and middle school students who had had OHCA of cardiac origin and received prehospital resuscitation in 2005-2009. Among 58 cases recruited, 90% were witnessed by bystanders; 86% had ventricular fibrillation as the initial rhythm; 74% were resuscitated by bystanders; 24% were defibrillated by bystanders; 55% occurred at school; 66% were exercise-related; 48% were followed up before the event; 67% had structural heart disease.

Public access defibrillation improved the outcome after out-of-hospital cardiac arrest in school-age children: a nationwide, population-based, Utstein registry study in Japan.

Aims: The purpose of this study was to determine whether implementation of public access defibrillation (PAD) improves the outcome after out-of-hospital cardiac arrest (OHCA) in school-age children at national level.

Methods And Results: We conducted a prospective, nationwide, population-based Japanese Utstein registry study of consecutive OHCA cases in elementary and middle school children (7-15 years of age) who had a bystander-witnessed arrest of presumed cardiac origin during 2005-09 and received pre-hospital resuscitation by emergency responders. The primary endpoint was a favourable neurological outcome 1 month after an arrest.

Natural history of medium-sized atrial septal defect in pediatric cases.

Background: The indication for surgical repair of atrial septal defect (ASD) is pulmonary to systemic blood flow ratio (Qp/Qs)>2.0, and therapeutic strategy depends on the facility in cases of Qp/Qs 1.5-2.

Phosphodiesterase inhibitors suppress Lactobacillus casei cell-wall-induced NF-κB and MAPK activations and cell proliferation through protein kinase A--or exchange protein activated by cAMP-dependent signal pathway.

Specific strains of Lactobacillus have been found to be beneficial in treating some types of diarrhea and vaginosis. However, a high mortality rate results from underlying immunosuppressive conditions in patients with Lactobacillus casei bacteremia. Cyclic AMP (cAMP) is a small second messenger molecule that mediates signal transduction.

Lactobacillus casei cell wall extract directly stimulates the expression of COX2 independent of Toll-like receptor 2 in rat glial cells.

Kawasaki disease is an acute illness of early childhood that is characterized by prolonged fever and vasculitis of unknown pathogenesis. Lactobacillus casei cell wall extract (LCWE)-induced vasculitis in mice is a well-validated model of Kawasaki disease. In the nervous system, glial cells play an important role in fever development.

Low-cost and ready-to-go remote-facilitated simulation-based learning.

Introduction: Remote-facilitated simulation-based learning was developed for team training with low-cost, preexisting, and easy-access resources to disseminate training with limited number of the faculty. This study was performed to examine the technical feasibility and to describe its characteristics compared with an on-site simulation system.

Method: We performed 2 pilot remote-facilitated sessions, followed by 3 additional sessions where 16 participants and 2 facilitators assessed the system using posttraining surveys containing items using 5-point Likert scale.

Age-specific differences in outcomes after out-of-hospital cardiac arrests.

Objective: We assessed out-of-hospital cardiac arrests (OHCAs) for various pediatric age groups.

Methods: This prospective, population-based, observational study included all emergency medical service-treated OHCAs in Osaka, Japan, between 1999 and 2006 (excluding 2004). Patients were grouped as adults (>17 years), infants (<1 year), younger children (1-4 years), older children (5-12 years), and adolescents (13-17 years).

Successful treatment with bosentan for pulmonary hypertension and reduced peripheral circulation in juvenile systemic sclerosis.

Pulmonary arterial hypertension (PAH) when associated with systemic sclerosis (SSc) (SSc-PAH) is one of the leading causes of mortality and is found in 10-15% of adult patients with SSc. The ET receptor antagonist bosentan has been shown to be effective in the treatment of adult patients with SSc-PAH. Furthermore, it has been shown that bosentan ameliorates decreased skin perfusion and digital ulceration secondary to SSc.

Effects of antithrombin III treatment in vascular injury model of mice.

Background: Balloon angioplasty has recently been adopted as an acceptable form of treatment for stenotic vessel lesions of congenital heart diseases. However, precise mechanisms of restenosis and thrombosis, which are the most common complications after these procedures, are unknown.

Methods: We examined the effects of antithrombin III (ATIII) on inflammation, thrombus formation, and remodeling of vascular wall after guidewire-induced injury in the femoral artery of mice.