Holter Electrocardiographic Approach to Predicting Outcomes of Pediatric Patients With Long QT Syndrome.

Background: This study was performed to clarify the clinical findings of pediatric patients diagnosed with long QT syndrome (LQTS) through electrocardiographic screening programs and to predict their outcome using Holter electrocardiographic approaches.

Methods and results: This retrospective study included pediatric patients with a Schwartz score of ≥3.5 who visited the National Hospital Organization Kagoshima Medical Center between April 2005 and March 2019.

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.

Tentative Screening Criteria for Short QT Interval in Children and Adolescents.

Background: While the prevalence of short QT syndrome (SQTS) in children and adolescents is low, early detection is important because SQTS can cause life-threatening arrhythmia. The aim of this study was to determine the tentative screening criteria for short QT interval in children and adolescents.

Methods and results: A total of 75,040 digitally stored electrocardiograms (ECG) of participants in a school-based ECG screening program were obtained between 2009 and 2013 in Kagoshima, Japan.

A surviving 24-month-old patient with neonatal-onset carnitine palmitoyltransferase II deficiency.

The early-onset form of carnitine palmitoyltransferase (CPT) II deficiency has severe outcomes; patients typically die during the newborn period. We report a case of neonatal-onset CPT II deficiency with prolonged survival, exceeding 24 months. The patient was successfully treated by continuous hemodialysis (CHD), which enabled her to overcome repeated crises.

Disruption of Endothelial Cell Homeostasis Plays a Key Role in the Early Pathogenesis of Coronary Artery Abnormalities in Kawasaki Disease.

Disruption of endothelial cell homeostasis may be associated with the pathogenesis of coronary artery abnormalities (CAA) in Kawasaki disease (KD). We sought to clarify the poorly understood pathogenic role of endothelial cell survival and death in KD vasculitis. Human umbilical vein endothelial cells (HUVECs) stimulated with sera from KD patients, compared with sera from patients with bacterial infections, exhibited significant increases in cytotoxicity, high mobility group box protein 1 (HMGB-1), and caspase-3/7 and a decrease in phosphorylated Akt/Akt (pAkt/Akt) ratios.

Dexmedetomidine is Associated with an Increased Incidence of Bradycardia in Patients with Trisomy 21 After Surgery for Congenital Heart Disease.

This study aimed to evaluate adverse cardiac events using dexmedetomidine in infants with trisomy 21 and those without (controls) and examined potential risk factors in infants after cardiovascular surgery. We conducted a single-center retrospective cohort study. The medical records of 124 consecutive infants who had undergone cardiovascular surgery between April 1, 2013, and October 31, 2015, were enrolled.

Potential risk factors for dexmedetomidine withdrawal seizures in infants after surgery for congenital heart disease.

Purpose: Few studies are available on withdrawal seizures about dexmedetomidine (DEX). Thus, we retrospectively evaluated the incidence of withdrawal seizures after discontinuation of DEX and examined potential risk factors in infants after cardiovascular surgery.

Methods: The medical records of 142 infants who had undergone cardiovascular surgery between April 2010 and November 2013 were examined.

Combination of flecainide and propranolol for congenital junctional ectopic tachycardia.

Congenital junctional ectopic tachycardia is a rare tachyarrhythmia with high mortality. A pharmacological approach in early infancy is regarded as the first-line therapeutic option. Pharmacologically, amiodarone alone or in combination with other drugs is the most commonly reported effective agent for congenital junctional ectopic tachycardia, but it has many adverse effects.

Current human T-cell lymphotropic virus type 1 mother-to-child transmission prevention status in Kagoshima.

The aim of this study was to assess the current human T-cell lymphotropic virus type 1 (HTLV-I) mother-to-child transmission (MTCT) prevention system in Kagoshima Prefecture. We investigated the rate of carrier pregnant women from obstetrics facilities in Kagoshima by mail in 2012 and compared our results with previous study results. We interviewed carrier pregnant women about their choices for infant nutrition, and we interviewed midwives about the follow-up system.

Genetic characteristics of children and adolescents with long-QT syndrome diagnosed by school-based electrocardiographic screening programs.

Background: A school-based electrocardiographic screening program has been developed in Japan. However, few data are available on the genetic characteristics of pediatric patients with long-QT syndrome who were diagnosed by this program.

Methods And Results: A total of 117 unrelated probands aged ≤18 years were the subjects who were referred to our centers for genetic testing.

Risk factors for symptoms in long QT syndrome patients in a single pediatric center.

Background: Long QT syndrome (LQTS) is a leading cause of sudden cardiac death due to arrhythmia in the pediatric population. This study aimed to determine risk factors for the presence of LQTS-related symptoms in a single pediatric center.

Methods: Subjects were 146 consecutive LQTS patients (M:F = 72:74) who visited our hospital between April 2005 and August 2012 and during the preceding 24 months.

K-Ras and H-Ras activation promote distinct consequences on endometrial cell survival.

A considerable amount of evidence indicates that Ras signaling contributes to the development of endometrial cancer. We previously demonstrated that endometrial cancer cells carrying oncogenic [(12)Val]K-ras were susceptible to apoptosis. The present study examined the role of K-and H-Ras in the induction of apoptosis using rat endometrial cells (RENT4 cells).