• Journal of Korean Medical Science
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• v.33 no.51
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• pp.324.1-324.6
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• 2018

Oxysterol $7{\alpha}$-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of $3{\beta}$-monohydroxy-${\Delta}^5$-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol $7{\alpha}$-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.

• Korean Journal of Childcare and Education
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• v.17 no.5
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• pp.83-103
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• 2021

Objective: This study aimed to develop a longitudinal predictive model that identifies first-grade children who are at risk for ADHD and to investigate the factors that predict the probability of belonging to the at-risk group for ADHD by using machine learning. Methods: The data of 1,445 first-grade children from the 1st, 3rd, 6th, 7th, and 8th waves of the Korean Children's Panel were analyzed. The output factors were the at-risk and non-risk group for ADHD divided by the CBCL DSM-ADHD scale. Prenatal as well as developmental factors during infancy and early childhood were used as input factors. Results: The model that best classifies the at-risk and the non-risk group for ADHD was the LASSO model. The input factors which increased the probability of being in the at-risk group for ADHD were temperament of negative emotionality, communication abilities, gross motor skills, social competences, and academic readiness. Conclusion/Implications: The outcomes indicate that children who showed specific risk indicators during infancy and early childhood are likely to be classified as being at risk for ADHD when entering elementary schools. The results may enable parents and clinicians to identify children with ADHD early by observing early signs and thus provide interventions as early as possible.

• Human Ecology Research
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• v.57 no.2
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• pp.159-170
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• 2019

The growth accelerator period from a child's body into an adult body is a huge transition characterized by rapid growth in the near term. Body shape changes at this time of growth should be continually studied because they can result in different outcomes due to various variables. This study is basic study for the production of a junior brassiere patternmaking was conducted to separate the upper torso and breasts of adolescents by growth level. Analysis was conducted by age classification according to sales trends. In this study. 3D body shape data of Korean girls, Based on the 6th Size Korea data, analyzed statistically the upper body and breast according to the rate of growth. The results of this study represent the basis for the development of a junior brassiere to help lead a better life in regards to clothing. The study used 3D-data from girls aged 13 to 18. The analysis indicates that the upper body is in a different shape at age 15 with an increasing circumference, width and shoulder length of the chest; in addition, the sides are analyzed differently, suggesting that the brassiere configuration should be made differently at age 15. The breast form also showed different growth patterns at age 13 and the result was that the shape of the cup in brassiere should be configured differently depending on type.

• Archives of Plastic Surgery
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• v.50 no.6
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• pp.578-585
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• 2023

An artificial sphincter implanted in the bulbous urethra to treat severe postprostatectomy urinary incontinence is effective, but embedding-associated complications can occur. We assessed the feasibility, efficacy, and safety of urethral graciloplasty cross-innervated by the pudendal nerve. A simulation surgery on three male fresh cadavers was performed. Both ends of the gracilis muscle were isolated only on its vascular pedicle with proximal end of the obturator nerve severed and transferred to the perineum. We examined whether the gracilis muscle could be wrapped around the bulbous urethra and whether the obturator nerve was long enough to suture with the pudendal nerve. In addition, surgery was performed on a 71-year-old male patient with severe urinary incontinence. The postoperative 12-month outcomes were assessed using a 24-hour pad test and urodynamic study. In all cadaveric simulations, the gracilis muscles could be wrapped around the bulbous urethra in a γ-loop configuration. The length of the obturator nerve was sufficient for neurorrhaphy with the pudendal nerve. In the clinical case, the postoperative course was uneventful. The mean maximum urethral closure pressure and functional profile length increased from 40.7 to 70 cm H₂O and from 40.1 to 45.3 mm, respectively. Although urinary incontinence was not completely cured, the patient was able to maintain urinary continence at night. Urethral graciloplasty cross-innervated by the pudendal nerve is effective in raising the urethral pressure and reducing urinary incontinence.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• The Journal of Korean Academy of Sensory Integration
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• v.15 no.1
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• pp.21-32
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• 2017

Objective : The purpose of this study is to explore the effectiveness of a perceptual motor program in promoting visual motor and motor control outcomes in a child with Attention-Deficit/Hyperactivity Disorder (ADHD) who is enrolled in an elementary school. Methods : The subject is 8-year-old boy who was diagnosed with ADHD but did not receive medication. Research design is a single-subject A-B-A(follow-up). Baseline and follow-up phase were 3 sessions and intervention phase were 8 sessions. After performing 40 minutes of free play on the baseline A and follow-up A, we used the copying subtest of the Korean Development Test of Visual Perception 2 (K-DTVP-2) for evaluating visual motor integration skill and the rope jumping subtest for motor skill. After providing the perceptual motor activity in intervention phase B, copying and ropejumping were performed. Results : The standard scores of the Copying for measuring visual motor integration skill were poor at an average of 4.7 points at baseline phase A, but maintained an average of 9.6 points at intervention phase B and an below average of 7.7 points at the follow-up phase A. In the Rope jumping for the motor skill, it was increased by 4.3 times in the baseline phase A and 5.9 times in the intervention phase B, but slightly decreased by 5 times in the follow-up phase A. Conclusion : This study suggests that perceptual motor program has a positive effect on visual motor and motor function of ADHD children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.4
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• pp.258-265
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• 2024

Purpose: To date, there is no region-specific guideline for pediatric endoscopy training. This study aimed to illustrate the current status of pediatric endoscopy training in Asia-Pacific region and identify opportunities for improvement. Methods: A cross-sectional survey, using a standardized electronic questionnaire, was conducted among medical schools in the Asia-Pacific region in January 2024. Results: A total of 57 medical centers in 12 countries offering formal Pediatric Gastroenterology training programs participated in this regional survey. More than 75% of the centers had an average case load of <10 cases per week for both diagnostic and therapeutic endoscopies. Only 36% of the study programs employed competency-based outcomes for program development, whereas nearly half (48%) used volume-based curricula. Foreign body retrieval, polypectomy, percutaneous endoscopic gastrostomy, and esophageal variceal hemostasis, that is, sclerotherapy or band ligation (endoscopic variceal sclerotherapy and endoscopic variceal ligation), comprised the top four priorities that the trainees should acquire in the autonomous stage (unconscious) of competence. Regarding the learning environment, only 31.5% provided formal hands-on workshops/simulation training. The direct observation of procedural skills was the most commonly used assessment method. The application of a quality assurance (QA) system in both educational and patient care (Pediatric Endoscopy Quality Improvement Network) aspects was present in only 28% and 17% of the centers, respectively. Conclusion: Compared with Western academic societies, the limited availability of cases remains a major concern. To close this gap, simulation and adult endoscopy training are essential. The implementation of reliable and valid assessment tools and QA systems can lead to significant development in future programs.

• Journal of Korean Academy of Nursing
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• v.30 no.5
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• pp.1195-1206
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• 2000

The purpose of this study was to analyze womens' health problems using Green & Kreuter's 1991 PRECEDE model and to develop a model for a womens' health care center located in the community. The subjects were recruited from Wonju City. 1. The results showed that 23% of the sample population felt there was a need for a womens' health care center. The mean number of health problems was 3.1. The prevalence rate, was 44.4%, and the rate for an artificial abortion, was 36.4%. Also 30.5% did not have a health examination in the past year. Women using the hospital for medical care accounted for 45% of the sample, while 40% used the drugstore. The average score on the HPLP was 2.41, and this was influenced by self-efficacy, family support, sexual role, and health locus of control. There are a few educational programs in the city provided by the Wonju Health Center and by community health nurse practitioners. 2. The nursing center, as defined in North America, is a nurse-anchored system of primary health care delivery or neighborhood health center. Centers offer various services ranging from primary care to the more traditional such as education, health promotion, wellness screening, and coordination of services by advanced practice nurses. For examples in Sweden MCH centers provide total services for childbearing women and their families, sexual counseling and education for adolescents, and screening by midwives for cervical cancer. 3. The developed model combines purpose, target population, organization, and services, and is related to health resources. The purpose is primary health care and promotion of the quality of life. The target population can be grouped according to the life cycle, (premarriaged age group, the childbearing/child rearing age group, and middle aged and elderly women) and focuses on self-help. The organization of the center includes an advisory committee to plan and evaluate, and a health services team that will be multidisciplinary to provide health care, counseling education, and research. The model development suggested that a variety of women's health care centers are needed to insure adequate management of women's health. Follow-up research using PROCEED is needed to analyze health outcomes. Also a health nursing specialist system is required to develop health promotion, and improve the quality of life of women.

• Journal of Christian Education in Korea
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• v.61
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• pp.165-201
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• 2020

The purpose of this study is to explore the development of language education of Christian early childhood education by exploring the change of pre-Christian preschool teachers through the development of biblical language education activities. Interviews, surveys, action plans, and reflections of 19 Christian education students who participated in the development of bible-based language education activities for children based on the language of early childhood language, were conducted from September 3 to December 28, 2018. The data were collected through a portfolio. By analyzing the collected data, the key categories were derived and categorized. For the objectification of data analysis and interpretation, two thematic and early childhood education specialists were identified. As a result, the preparatory Christian teacher experience for the development of bible-based langage education activities for young children was categorized into cognitive change, personality change and practical change. First, through the development of bible-based early childhood language education activities, Christian pre-service early childhood teachers have brought cognitive changes as 'processes not outcomes', 'integration not separation', 'living non-curriculum' and 'meaning not effect'. In developing bible-based language education activities for young children, Christian pre-service early childhood teachers experienced a cognitive change in the 'process of language education activities' rather than the developmental achievements and results of early childhood language education. Christian pre-service early childhood teachers recognized the necessity of 'integration of listening-speaking-reading-writing', not the separation of early childhood language education. They recognized the importance of 'informal language education in kindergarten life', as well as teacher-centered formal language education. In addition, they have made a cognitive change that 'child-centered meaningful language education experience' is more important than the effectiveness of early childhood language education. Second, in developing bible-based language education activities for young children, Christian pre-service early childhood teachers showed personality changes as 'confident teachers', 'professional teachers', and 'teachers with reflective thoughts and attitudes'. Finally, in developing bible-based language education activities for young children, Christian pre-service early childhood teachers recognized the power of positive language and practiced it to form habits of using the right language and to link Christian education with early childhood education. Through the development of bible-based language education activities for young children, Christian pre-service early childhood teachers are equipped with the heart attitude and enthusiasm required to become true early childhood teachers for young children in unpredictable educational conditions and rapidly changing educational realities. Teacher efficacy has improved. In the future, it is expected that various teacher education programs linking Christian education and early childhood education will be continuously and systematically implemented.

• Journal of Korean Academy of Nursing
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• v.25 no.3
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• pp.472-484
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• 1995

The purpose of this study was to develop a home health care model in the public health system and to test the effectiveness of the model. Seven com-munity health practitioners in Yon- Cheon county. Kyunggi province, carried out home health care service for this research. The subjects of the home health care were a total of 111 community residents with chronic health problems and risk-prone infants and children; 29 persons with hypertension, 18 persons with diabetes, 12 persons with neurologic problems, 12 elderly, and 40 infants and children. During the period of study, from December, 1993 to March, 1995, a demonstrative home health care model was developed in the Yon-Cheon County community health centers with the cooperation of the Yon-Cheon Medical Center and Yon-Cheon Public Health Center for the first six months. A home care practice manual and recording system for home visits were also co-developed by the researchers and community health practitioners. Four workshops and monthly conferences were held for this purpose. Actual home care practice took place for two months, and on-going evaluation and replanning accompanied this process. The result of the evaluation of home care service were as follows. 1) For persons with hypertension, diabetes, neurologic problems, there was significant improvement in knowledge of disease and care, but no significant difference was seen in health behavior or symptoms after home care service. 2) No significant difference was seen in level of self esteem or depression after reminiscence therapy among 12 elderly subjects. 3) There were significant differences in satis-faction toward child rearing and parental sup-port, but no significant difference In education needs for parental role after home care service among parents of infants and children. 4) There was significant improvement in the quality of life among the subjects after the home care service. 5) Subjects responded that they were highly satisfied with the home care service given by the community health practitioners. Although, the actual implementation period was very short, and not all of the evaluation outcomes showed significant improvement, the home health care model of community health practitioners was, in general, positively evaluated. Through this re-search, the possibility of community health practitioners working as active home care personnel in the public health care system is supported. Further research with an expanded area and subjects for a longer period is recommended. Cost effectiveness research is also needed.