Pediatrics International : Official Journal of the Japan Pediatric Society

BACKGROUND: Hyaline fibromatosis syndrome (HFS) is a congenital disorder characterized by subcutaneous skin nodules, congenital multiple arthrogryposis, gingival hyperplasia, and chronic pain. The intellectual ability of patients with HFS is generally normal. This syndrome arises from variants of ANTXR2. Thus far, about 100 cases have been reported but few of these were reported from Japan. METHODS: This study reports five additional Japanese patients with genetically confirmed HFS, from unrelatd families, and discusses the clinical course and quality of life of these patients...

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BACKGROUND: Pediatric inflammatory bowel disease (PIBD) affects different age groups and its incidence is increasing worldwide. However, there is a lack of research focusing on age subgroups in Asian countries. In this nationwide population-based study, we investigated the epidemiology of PIBD among different age subgroups in Korea. METHODS: We analyzed Korean health administration data from 2005 to 2016. Data were divided by age at diagnosis as follows: group 1, 0-1 years; group 2, 2-5 years; group 3, 6-9 years; group 4, 10-16 years...

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BACKGROUND: The increasing worldwide prevalence of multidrug-resistant (MDR) bacteria underscores the pressing demand for innovative therapeutic solutions. Ceftazidime-avibactam (CAZ-AVI) represents a promising new drug combination that has received approval for specific infection types. However, there is limited information regarding its application in pediatric patients. METHODS: This study investigates the effectiveness and adverse reactions associated with CAZ-AVI treatment in pediatric patients with life-threatening infections caused by MDR pathogens...

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BACKGROUND: The aim of our study was to investigate the effect of an exercise program on health-related quality of life (HRQoL) and sleep quality in children with inflammatory bowel disease (IBD) in remission. METHODS: A total of 42 pediatric IBD patients in remission were recruited to participate in a 6-month-long home-based exercise program. Their mean age was 15.3 years (with a range of ± 2.08 years) and there were 25 boys...

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BACKGROUND: The incidence of dysnatremia in children with acute gastritis/gastroenteritis varies, and factors associated with either dysnatremia or hyponatremia at presentation have not been identified clearly. METHODS: This retrospective study included patients aged 1 month to 18 years hospitalized for community-acquired acute gastritis/gastroenteritis from January to October 2016. Factors associated with dysnatremia at presentation were identified using multivariable analysis...

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BACKGROUND: The objective of this study was to develop the Parenting Difficulties in Infectious Disease Pandemic Inventory (PDIDPI) for the assessment of parenting difficulties during the coronavirus disease 2019 (COVID-19) pandemic and to examine its psychometric properties. METHODS: The 31-item PDIDPI was developed on the basis of the results of focus group interviews. An exploratory factor analysis using principal axis factoring was conducted to examine the PDIDPI factor structure...

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