• Clinical and Experimental Pediatrics
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• 제54권2호
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• pp.55-63
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• 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2-q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy in patients with PWS.

• 아동학회지
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• 제25권5호
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• pp.147-161
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• 2004

This study was designed to explore developmental evaluation in healthy full-term, at risk preterm and full-term infants. Specifically the purposes of the study were to investigate Psychomotor Developmental Index(PDI) and Mental Developmental Index(MDI) based on Bayley Scales of Infant Development(1993). The subjects were 72 infants, 24 each for healthy full-term infants, 24 each for at risk preterm infants and 24 each for at risk full-term infants such as having neonatal asphyxia, hypoxic ischemic brain damage, respiratory distress syndrome. The data were analyzed through Kruskal-Wallis test and correlations to examine healthy full-term, at risk pre-term and at risk full-term infants. Results showed that there were significant differences among healthy full-term, at risk pre-term and at risk full-term infants in PDI and MDI. On the correlation with PDI and MDI, infants showed significant correlations. Early interventions for developmental improvement are required for functional outcome in these infants.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.224-231
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• 2012

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion, maternal disomy, and deficient imprinting) are recognized. Maternal disomy can arise because of 4 possible mechanisms: trisomy rescue (TR), gamete complementation (GC), monosomy rescue (MR), and postfertilization mitotic nondisjunction (Mit). Recently, TR/GC caused by nondisjunction at maternal meiosis 1 has been identified increasingly, as a result of advanced maternal childbearing age in Korea. We verified that the d3 allele increases the responsiveness of the growth hormone (GH) receptor to endogenous GH. This paper also provides an overview of endocrine dysfunctions in children with PWS, including GH deficiency, obesity, sexual development, hypothyroidism, and adrenal insufficiency, as well as the effects of GH treatment. GH treatment coupled with a strictly controlled diet during early childhood may help to reduce obesity, improve neurodevelopment, and increase muscle mass. A more active approach to correct these hormone deficiencies would benefit patients with PWS.

• Journal of Korean Neurosurgical Society
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• 제56권4호
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• pp.334-337
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• 2014

Objective : The purpose of this study is to identify the risk factors associated with the development of germinal matrix-intraventricular hemorrhage (GM-IVH) and the relationship of the severity of disease and prematurity. Methods : A total of 168 premature neonates whose birth weight ${\leq}1500g$ or gestational age ${\leq}34$ weeks were examined by cranial ultrasound (CUS) for detection of GM-IVH among the babies admitted between January 2011 and December 2012 in our medical center neonatal intensive care unit. The babies were divided into two groups : GM-IVH and non-IVH. Clinical presentations, precipitating factors of the patients and maternal factors were analyzed. Results : In univariate analysis, gestational age, birth weight, delivery method, presence of premature rupture of membrane (PROM) and level of sodium and glucose were statistically meaningful factors (p<0.05). But only two factors, gestational age and presence of patent ductus arteriosus (PDA) were statistically meaningful in multivariate logistic regression (p<0.05). Delivery method [normal vaginal delivery (NVD) to Caeserean section] was borderline significant (p<0.10). Conclusion : Presence of PDA and gestational age were the important risk factors associated with development of GM-IVH.

• Journal of Animal Science and Technology
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• 제55권6호
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• pp.521-530
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• 2013

Direct cell-cell communication via the transfer of small molecules between neighboring cells in tissue is accomplished by gap junctions composed of various connexins (Cxs). Proper postnatal development of the epididymis is important for acquisition of male reproduction. The epididymal epithelium is composed of several cell types, and some of these cells are connected by gap junctions. The present study was conducted to determine the presence of Cx transcripts in the corpus and cauda epididymis. In addition, transcriptional changes of Cxs expressed during different postnatal stages were examined by real-time PCR analysis. In both epididymal regions, the same nine Cx transcripts of thirteen Cxs tested were detected. In the corpus epididymis, the highest levels of Cxs31.1 and 37 transcripts were observed at 45 days of age, and amounts of Cxs26, 30.3, and 32 transcripts increased with age and subsequently decreased in the elderly. Expression of Cx31 was greatly increased in the adult and elder stages, while Cxs40, 43, and 45 were abundant in the early postnatal stages. In the cauda epididymis, expression of Cxs26, 30.3, 31.1, 37, and 40 reached the highest levels at 5 months of age. The levels of Cxs31 and 32 mRNAs fluctuated throughout the postnatal period. The amounts of Cxs43 and 45 transcripts were more abundant during the late neonatal and prepubertal ages than later ages. These findings suggest that regional specification of the epididymis is partly regulated by differential expression of Cx genes during the postnatal developmental period.

• Child Health Nursing Research
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• 제18권2호
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• pp.85-94
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• 2012

Purpose: Instead of a chaotic non circadian environmental approach, continuing regular day-night rhythm in neonatal nurseries may benefit the development of preterm infants. The purpose of this study was to define the effects of cycled lighting on circadian rhythms of premature infants. Methods: The experimental group included 15 preterm infants, and the control group, 15 premature infants in the NICU of a general hospital in Seoul. All infants were between 32 and 37 weeks' postconceptional age. The experimental group infants were provided with cycled lighting before discharge. The incubator or bassinet cover of the experimental group was off between 7 AM and 7 PM, and was covered between 7 PM and 7 AM. Results: There were significant differences in the NNNS score ($p$=.039), and some significant differences in the sleep-activity pattern between the experimental group and the control group, but distinct differences in sleep-activity patterns between the two groups could not be defined. Conclusion: The results of this study suggest that cycled lighting can be helpful in the neurobehavioral development of preterm infants. By modifying the NICU environment to provide a more developmentally supportive milieu, nursing professionals can better meet the infants' physiologic and neurobehavioral needs.

• Child Health Nursing Research
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• 제27권1호
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• pp.34-42
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• 2021

Purpose: The purpose of this study was to identify the supportive care needs of parents of preterm children in South Korea using text data from a portal site. Methods: In total, 628 online newspaper articles and 1,966 social network service posts published between January 1 and December 31, 2019 were analyzed. The procedures in this study were conducted in the following order: keyword selection, data collection, morpheme analysis, keyword analysis, and topic modeling. Results: The term "yirundung-yi", which is a native Korean word referring to premature infants, was confirmed to be a useful term for parents. The following four topics were identified as the supportive care needs of parents of preterm children: 1) a vague fear of caring for a baby upon imminent neonatal intensive care unit discharge, 2) real-world difficulties encountered while caring for preterm children, 3) concerns about growth and development problems, and 4) anxiety about possible complications. Conclusion: Supportive care interventions for parents of preterm children should include general parenting methods for babies. A team composed of multidisciplinary experts must support the individual growth and development of preterm children and manage the complications of prematurity using highly accessible media.

• Journal of Biomedical and Translational Research
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• 제19권4호
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• pp.125-129
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• 2018

Dopaminergic neurons are one of the major neuronal components in the brain. Mesencephalon dopamine (DA) neurogenesis takes place in the ventricular zone of the floor plate, when DA progenitors divide to generate postmitotic cells. These cells migrate through the intermediate zone while they differentiate and become DA neurons on reaching the mantle zone. However, neurogenesis and neuronal migration on dopaminergic neurons remain largely unexplored in the mesencephalon development. This study presents neurogenesis and neuronal migration patterns of dopaminergic neurons during mesencephalic development of the mouse. Neurons from embryonic day (E) 10-14 were labelled by a single injection of 5-bromodeoxyuridine and immunohistochemistry was performed. The neurogenesis occurred mainly at the E10 and E11, which was uniformly distributed in the mesencephalic region, but neurons after E13 were observed only in the dorsal mesencephalon. At the postnatal day 0 (P0), E10 generated neurons were spread out uniformly in the whole mesencephalon whereas E11-originated neurons were clearly depleted in the red nucleus region. DA neurons mainly originated in the ventromedial mesencephalon at the early embryonic stage especially E10 to E11. DA neurons after E12 were only observed in the ventral mesencephalon. At E17, E10 labelled neurons were only observed in the substantia nigra (SN) region. Our study demonstrated that major neurogenesis occurred at E10 and E11. However, neuronal migration continued until neonatal period during mesencephalic development.

• Child Health Nursing Research
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• 제22권4호
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• pp.336-345
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• 2016

Purpose: This study was conducted to describe health in optimal fitness (HOF) in young children born prematurely and to analyze factors affecting HOF in health status, investment resources, and anthropological values, based on HOF theory. Methods: A case-control study of 76 children with preterm births (PTB) was conducted at 24 to 42 months of corrected age. Their HOF status was evaluated based on height, weight, head circumference, and the Korean-Bayley Scale of Infant Development-II and classified as either HOF-achieved or HOF-uncertain in the domain of growth, development, and all together. Results: For growth, development, and all, 26.3%, 27.6%, and 47.4% of children, respectively, belonged to the HOF-uncertain group. Logistic regression analysis showed that longer length of hospital stay (${\geq}21days$; OR=7.8; 95% CI [1.5, 40.5]), worse scores on the Home Observation for Measurement of the Environment (HOME) (${\geq}38$; OR=0.1; 95% CI [0.0, 0.4]), having a working mother, (OR=5.7; 95% CI [1.2, 27.6]), and an older mother (${\geq}35years$; OR=8.8; 95% CI [2.1, 37.3]) were statistically significant contributors of HOF-uncertain in the domain of all. Conclusion: Findings show that young children born prematurely with prolonged stays in a neonatal intensive care unit and insufficient socioeconomic resources at home are more likely to exhibit delayed growth and development.

• Journal of Animal Science and Technology
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• 제51권6호
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• pp.485-492
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• 2009

Estrogen has dramatic effects on the development and function of the reproductive tract in mammals. Although diethylstilbestrol (DES) triggers the development of reproductive organs in immature animals, continued exposure to DES induces dysfunction of the female reproductive tract in mice. To investigate the effects of neonatal estrogen exposure on the reproductive tract of female chickens, we implanted DES pellets into the abdominal region of immature female chicks and then examined the effects of DES on the oviducts of both immature chicks and sexually mature chickens (30 weeks old). DES induced mass growth and differentiation of the oviduct in immature chicks. The chick oviduct increased by 2.7- and 29-fold in length and weight, respectively, following primary DES stimulation. In secondary DES stimulation, the length and weight of the chick oviduct increased by 4.5- and 74-fold, respectively. Additionally, DES treatments caused abnormal development of the infundibulum and magnum in hen oviducts. Furthermore, infundibulum abnormality gave rise to unusual ovulation of follicles and resulted in infertility and dysfunction of the magnum, such as less production of egg white proteins. Our results indicate that DES exposure during early developmental stages in chickens has detrimental effects on the development and maintenance of the female reproductive tract after sexual maturation.