Pediatrics international : official journal of the Japan Pediatric Society
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One of the most important functions of the emergency department (ED) is to assess patient status. Only one, the pediatric early warning score (PEWS), has been designed for ED with acceptable validity, but it has never been validated in Thailand. The objective of this study was to validate PEWS in predicting hospitalization in children visiting the ED. ⋯ PEWS can be helpful in assessing patient status in pediatric ED with acceptable validity and can serve as a potentially excellent screening tool for prediction of ICU admission.
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Case Reports
Differing phenotypes of Moyamoya disease in a familial case involving heterozygous c.14429G > A variant in RNF213.
Moyamoya disease (MMD) is a chronic steno-occlusive arteriopathy involving the development of abnormal collateral vessels. Ring finger protein (RNF213) on the 17q25.3 locus was identified as an MMD-susceptibility gene in East Asian populations. We report a 5-year-old Japanese boy diagnosed with cerebral infarction and unilateral MMD. ⋯ The boy's mother had no neurological symptoms, but sequencing of RNF213 showed the same variant, and MRA indicated stenosis of the terminal bilateral ICA. This is the first report, to our knowledge, of different MMD phenotypes in a familial case involving the same heterozygous c.14429G > A variant in RNF213. Genetic testing for RNF213 is suggested for family member screening.
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The Consensus on Science and Treatment Recommendations 2010 supported simulation-based training for education in resuscitation. This approach has been introduced into neonatal cardiopulmonary resuscitation (NCPR) courses in Japan, but no method for teaching instructors has been established. We developed a course for training instructors of NCPR, with inclusion of an instruction practice program. The goal of the study was to evaluate the performance of instructors who completed the course. ⋯ This is the first trial of a resuscitation training course using scenarios that participants developed themselves. A new course including instruction practice for training NCPR instructors was effective for improving instructor performance.
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Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. ⋯ In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.