• Neonatal Medicine
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• v.25 no.1
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• pp.23-28
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• 2018

Purpose: Abdominoperineal solid tumors presenting in neonates often require surgical intervention during the neonatal period. Although we report our single-center experience, this study would be meaningful to understand the clinical implications of these neoplasms. Methods: We retrospectively reviewed and analyzed the clinical data and characteristics of 22 patients (${\leq}28$ days old) diagnosed with histopathologically confirmed abdominoperineal solid neoplasms (benign or malignant) after surgical resection. Results: The mean gestational age and postnatal age at the time of operation were $38.3{\pm}1.8weeks$ and $13.5{\pm}8.3days$, respectively. Most patients (18/22, 81.8%) were diagnosed during antenatal care visits; however, 4 (18.2%) were identified after birth. The mean tumor size was $6.4{\times}5.3cm$ (3.5-17.0 cm), and tumors occurred most frequently within the sacrococcygeal region (8/22, 36.4%). Histopathologically, 14 patients (63.6%) demonstrated benign tumors and 8 (36.4%) demonstrated malignant tumors. Germ cell tumors and hepatoblastomas were the most commonly observed tumors. Fortunately, all patients showed a localized pattern of tumor involvement without distant metastasis. No recurrence or mortality was observed during the follow-up period (mean $66.4{\pm}44.2months$). Conclusion: Abdominoperineal solid tumors occurring in neonates show variable clinical patterns during the antenatal and postnatal monitoring/screening periods. We conclude that aggressive and multidisciplinary approaches could achieve good clinical results in these patients.

• Clinical and Experimental Pediatrics
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• v.61 no.8
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• pp.253-257
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• 2018

Purpose: Child safety seats (CSS) are critical for the protection of children, in case of motor vehicle accidents. Although the national legislation mandates that all newborns must be placed in an appropriately installed CSS during transportation, people often do not perceive the importance of CSS and do not use it as recommended. The purpose of this survey was to understand the use of CSS for the safe transport of newborns from hospital to home. Methods: We interviewed parents of newborn infants, using a structured questionnaire, at the time of their discharge from Cheil General Hospital & Women's Health Care Center, between May 2014 and July 2014. Results: A total of 403 participants were interviewed. The rate of CSS use was only 14.9%. Overall, 76.4% of the families interviewed were not aware about the recommendations on CSS use for newborns when travelling in a car. The provision of education on using CSS significantly influenced their rate of use. Parents who were educated about mounting the CSS in a car used it more as compared with others (25.7% vs. 12.2%) (P=0.002). Furthermore, if parents had heard about the importance or necessity of CSS, they used it more than others did (19.5% vs. 10.6%, P=0.032). Conclusion: Despite the legal regulation, most parents transport their newborn infants without a CSS while traveling from hospital to their home. The rate of CSS use was influenced by parental education and their knowledge about its necessity. Education programs for parents must be reinforced to increase the CSS use.

• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.14-20
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• 2018

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders caused by specific lysosomal enzyme deficiencies leading to the sequential degradation of glycosaminoglycans, causing substrate accumulation in various cells and tissues and progressive multiple organ dysfunction. The rare disease medical care team at Mackay Memorial Hospital in Taiwan has been dedicated to the study of MPSs for more than 20 years. Since 1999, more than 50 academic papers focusing on MPSs have been published in international medical journals. Topics of research include the following items regarding MPSs: incidence, natural history, clinical manifestations, gene mutation characteristics, cardiac function, bone mineral density, sleep studies, pulmonary function tests, hearing assessments, percutaneous endoscopic gastrostomy, anesthetic experience, imaging analysis, special biochemical tests, laboratory diagnostics, global expert consensus conferences, prenatal diagnosis, new drug clinical trials, newborn screening, and treatment outcomes. Of these published academic research papers, more than half were cross-domain, cross-industry, and international studies with results in cooperation with experts from European, American and other Asian countries. A cross-specialty collaboration platform was established based on high-risk population screening criteria with the acronym "BECARE" (Bone and joints, Eyes, Cardiac and central nervous system, Abdomen and appearance, Respiratory system, and Ear, nose, and throat involvement). Through this platform, orthopedic surgeons, rheumatologists, ophthalmologists, cardiologists, rehabilitation physicians, gastroenterologists, otorhinolaryngologists, and medical geneticists have been educated with regards to awareness of suspected cases of MPSs patients to allow for a further confirmative diagnosis of MPSs. Because of the progressive nature of the disease, an early diagnosis and early multidisciplinary therapeutic interventions including surgery, rehabilitation programs, symptom-based treatments, hematopoietic stem cell transplantation, and enzyme replacement therapy, are very important.

• Korean Journal of Child Studies
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• v.25 no.1
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• pp.93-112
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• 2004

The characteristics of traditional childbirth and childcare custom on Jeju were studied by interviews of 134 elder women over 60. This established the nature of traditional custom of prenatal care, delivery, child rearing, and traditional ceremonies for baby as well as popular beliefs. The survey of current childbirth and childcare practices included 168 parents of kindergarteners. Results showed that traditional childbirth and childcare custom of Jeju is based in self-reliance, effectiveness, labor, and popular beliefs and that the heritage consisting of traditional postpartum foods, bathing of the newborn baby, use of a cradle, shamanistic prescriptions, and acupuncture is prevalent today.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.223-229
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• 2007

The fetus is completely dependent on mother for glucose and other nutrient transfer across the placenta. At birth, when the maternal supply is discontinued, the neonate must adjust to an independent existence. The changes in the neonate's glucose homeostasis during this transition to the extrauterine environment are influenced by the mother's metabolism and intrinsic fetal and placental problems. Maturation of carbohydrate homeostasis results from a balance between substrate availability and coordination of developing hormonal, enzymatic, and neural systems. These mechanisms may not be fully developed in neonates, so the neonate is vulnerable to carbohydrate disequilibrium resulting in damage to the central nervous system. Hypoglycemia is a relatively common metabolic problem seen during newborn care. However its definition, management and long term sequalae remain controversial. Hyporglycemia occurs frequently as a transient disorder with excellent prognosis. It also may persist and recur and cause permanent neurological complications. Although the key to effective treatment of hypoglycemia is diagnostic specific, the maintenance of euglycemia is critical to the preservation of central nervous system function. This article discusses physiology of perinatal glucose homeostasis, focusing on evaluation and treatment of hypoglycemia.

• Journal of Genetic Medicine
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• v.14 no.1
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• pp.27-30
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• 2017

Adrenal hypoplasia congenita (AHC) is a rare cause of adrenal insufficiency during neonatal period. Mutations in the gene coding for DAX1 cause X-linked adrenal hypoplasia. Most affected patients are shown to have salt wasting and hyperpigmentation on the skin during the neonatal period and require intensive medical care. In addition, it is usually associated with hypogonadotropic hypogonadism in adolescence. The DAX1 gene is expressed in the adrenal cortex, pituitary gland, hypothalamus, testis, and ovary. We report on a patient with genetically confirmed AHC whose initial clinical presentations were consistent with congenital adrenal hyperplasia. A point mutation in the DAX1 gene identified in this report resulted in a truncated DAX1 protein. Our patient was diagnosed with AHC.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.15-20
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• 2023

Galactosemia is an inborn error disorder of carbohydrate metabolism, caused by metabolic disturbances at various stages of the Leloir pathway. In patients with galactosemia, accurate diagnosis and appropriate care are essential to avoid complications and unnecessary treatments. And a careful differential diagnosis of the type of galactosemia is crucial. Even with an appropriate galactose-restricted diet, long-term complications may occur, especially in patients with classic galactosemia. So new treatment options are being developed. In this review, we will review the new symptoms of each subtype that have been reported recently and GALM (Galactose mutarotase) deficiency, a new form of galactosemia, and treatment policies according to recent guidelines.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1073-1079
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• 2003

Purpose : Recently there has been a decrease in ventilator care rate and duration of very low birth weight infants(VLBWI) in Fatima Hospital. The aims of this study were to survey the frequency and duration of ventilation in VLBWI and to develop a non-invasive neonatal intensive care unit (NICU) policy. Methods : We performed a retrospective study of 284 newborn of infants less than 1,500 gm admitted to NICU and discharged from January 1998 to December 2001. Patients were intubated or applied continuous positive airway pressure(CPAP) via nasal prong immediately after presenting signs of respiratory distress. We analyzed epidemiologic data to study the changes in ventilator care rate, duration and outcome of ventilator care groups. Results : Of 284 newborn infants, 146 required invasive management, such as endotracheal intubation and assisted ventilation. The characteristics, the severity of clinical symptoms and laboratory findings in ventilator care groups at birth showed no significant differences. The annual proportion of infants requiring assisted ventilation decreased according to increasing gestational age. The median duration of ventilation decreased markedly from 6.0 days in 1998 to 2.7 days in 2001. Final complications and outcomes in ventilator care groups showed no significant differences. Conclusion : Our study shows a significant reduction in the invasiveness of the treatment of VLBW infants, which was not associated with an increased mortality or morbidity. A non-invasive strategy for the VLBW infant with minimal to moderate respiratory distress after birth in NICU is better than immediate invasive management. Non-invasive nasal CPAP is a simpler and safer method than invasive assisted ventilation.

• Child Health Nursing Research
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• v.25 no.2
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• pp.184-195
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• 2019

Purpose: This study aimed to characterize the relationship between parental stress and nurses' communication as perceived by parents of high-risk newborns in a neonatal intensive care unit (NICU). Methods: The participants were 54 parents of high-risk newborns in a NICU. Data were collected from January to March 2018. Parental stress and parents' perceptions of nurses' communication ability and styles were measured using a questionnaire. Results: The average scores for parental stress and nurses' communication ability were 3.39 and 4.38 respectively, on a 5-point scale. Parents most commonly reported that nurses showed a friendly communication style, followed by informative and authoritative styles. Mothers and fathers reported significantly different levels of parental stress. Parental stress showed a negative correlation with nurses' perceived verbal communication ability. Higher scores for nurses' verbal communication ability and for friendly and informative communication styles were associated with lower parental stress induced by the environment, the baby's appearance and behaviors, and treatments in the NICU. Conclusion: The findings of this study suggest that nurses need to offer proper information for parents and to support parents by encouraging them to express their emotions of stress and by providing parents with therapeutic communication and opportunities to participate in care.

• Child Health Nursing Research
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• v.25 no.2
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• pp.103-111
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• 2019

Purpose: This study aimed to investigate incidence of delirium in the pediatric intensive care unit (PICU) and to analyze associated risk factors. Methods: The participants were 95 patients, newborn to 18 years, who were admitted to the PICU. The instruments used were the Richmond Agitation Sedation Scale (RASS), and the Cornell Assessment of Pediatric Delirium. Data analysis was performed using the descriptive, $x^2$ test, t-test, and logistic regression analyses. Results: The incidence of delirium in children admitted to the PICU was 42.1%. There were significant differences according to age ($x^2=14.10$, p=.007), admission type ($x^2=7.40$, p=.007), use of physical restraints ($x^2=26.11$, p<.001), RASS score ($x^2=14.80$, p=.001), need for oxygen ($x^2=5.31$, p=.021), use of a mechanical device ($x^2=9.97$, p=.041), feeding ($x^2=7.85$, p=.005), and the presence of familiar objects ($x^2=29.21$, p<.001). Factors associated with the diagnosis of delirium were the use of physical restraint (odds ratio [OR]=13.82, 95% confidence interval [CI]=4.16~45.95, p<.001) and the presence of familiar objects (OR=0.09, 95% CI=0.03~0.30, p=.002). Conclusion: Periodic delirium assessments and intervention should be actively performed. The use of restraints should be minimized if possible. The caregiver should surround the child with familiar objects and ensure a friendly hospital environment that is appropriate for the child.