• Journal of Genetic Medicine
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• v.13 no.2
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• pp.72-77
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• 2016

Purpose: Gaucher disease (GD) is the most common lysosomal storage disease caused by beta-glucocerebrosidase (GBA) deficiency. Oral substrate reduction therapy with miglustat ($Zavesca^{(R)}$) was approved for the treatment of adults with GD type 1, for whom enzyme replacement therapy (ERT) is unsuitable or not a therapeutic option. In this study, we report the effect of miglustat ($Zavesca^{(R)}$) in three Korean GD patients. Materials and Methods: Clinical findings comprising age at diagnosis, presenting signs, laboratory findings at diagnosis, GBA activity and mutations, and clinical courses of the three patients were reviewed. Results: Miglustat was administered to three patients who reported allergic reactions during intravenous imiglucerase infusions. One patient withdrew after 15 months of miglustat administration owing to continuous elevation of disease biomarker levels (chitotriosidase, acid phosphatase, and angiotensin-converting enzyme). Poor adherence to medication was suspected but was denied by the patient. In the other two patients, platelet count and levels of hemoglobin and other biomarkers remained stable during miglustat administration. However, they suffered from severe diarrhea and weight loss, which led to miglustat discontinuation after 1 and 12 months of administration. Conclusion: Our study shows that although miglustat is suggested to GD patients as an alternative treatment to ERT, significant adverse reactions may lead to discontinuation of miglustat. In addition, it is difficult to monitor the drug adherence.

• The Journal of the Acoustical Society of Korea
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• v.41 no.1
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• pp.99-107
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• 2022

This study attempted to analyze the consonant perception ability and errors and to verify compatibility items for hearing impaired children wearing hearing aids and cochlear implants using the Phoneme Perception Test for School-Aged children (PPT-S). As a result of the study, it was found that children with hearing impairments have more difficulty in perceiving final consonants than initial consonants. The hard type of PPT-S, in which the articulation method and articulation place of the target and foil words are similar, felt more difficult than the easy type. Among the initial consonants, the incorrect response rate for aspiration sound was higher. In the case of final consonants, the incorrect answer rate for 'ㄷ' and 'ㅁ' was relatively higher. There was no significant difference in the percentage of correct response rate according to the gender of the speaker. The above results can be usefully used as basic data for standardizing of PPT-S and evaluating the intervention effects before and after hearing rehabilitation with hearing impaired children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.6
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• pp.346-354
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• 2023

Purpose: Approximately 30% of children with chronic liver disease (CLD) are malnourished. However, proper assessment of their nutritional status is difficult. The subjective global nutritional assessment (SGNA) is a comprehensive approach that uses nutrition-focused history and examination, followed by grading of malnourishment. We aimed to study the prevalence of malnutrition in children with CLD using the SGNA tool. Methods: This cross-sectional observational study included patients aged <18 years with CLD. Nutritional assessments were recorded using SGNA tool. Conventional anthropometric measurements were performed and corroborated with nutritional status using SGNA tool. Results: A total of 85 children with CLD and mean age of 62 months were enrolled in this study. The prevalence of malnourished children according to SGNA was 34%; 22% were moderately malnourished and 12% were severely malnourished. We found statistically significant differences in anthropometric parameters among the three groups. A moderate degree of agreement was found between SGNA and weight-for-age (W/A) (p=0.020), mid-upper arm circumference (MUAC) (p<0.001), and triceps skin-fold thickness (TSF)-for-age (p=0.029). Furthermore, a fair degree of agreement was found between height-for-age (H/A) (p=0.001) and weight-for-height (W/H) (p<0.001). The sensitivity of W/A for detecting malnutrition was 93%, H/A was 90%, MUAC was 86%, and TSF was 88%. The sensitivity was much lower for W/H and body mass index for age (55% for both). Conclusion: In our study, more than one-third of children with CLD were malnourished. Nutritional assessment using SGNA is a reliable method for evaluating nutritional status and is significantly correlated with common anthropometric measurements.

• Korean Journal of Child Studies
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• v.28 no.4
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• pp.1-17
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• 2007

Participants in this study on links between toddlers' compliance and variables were 97 toddlers and their mothers. Instruments were the Toddler Behavior Assessment Questionnaire (Goldsmith, 1988) and the Child Rearing Practice Report-Q Sort (Block, 1981). Toddlers' compliance was observed on clean-up and behavior-delay tasks. Data analysis was by logistic and regression analyses. Results on the clean-up task indicated lower compliance by difficult children and higher compliance by girls. On the behavior-delay task, boys with less difficult temperament showed higher compliance by mother's higher punishment attitudes, whereas highly difficult boys were more compliant by mother's lower punishment attitudes. Girls' compliance was higher than boys in the low inhibition group whereas boys' compliance was higher than girls in the high inhibition group.

• Korean Parent-Child Health Journal
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• v.8 no.1
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• pp.3-16
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• 2005

Purpose: This study was to investigate the stress of parents while they were rearing their children. Method: This study reviewed the preceding research related to parents' stress and breeding stress with consideration of modem society, family system and parents' role. Results: In the parent-child relationship, parents feel stressed when they found it difficult to come up with an expectation to the parents' role of rearing child. Therefore, considering the health of parents and children, it is important to mediate parents who are under stress. Moreover, since parents cannot handle this problem all alone, it should be discussed and solved not only by individual but also by family, and further by social context. The parent-child relationship in early infancy is a core element to the development of an individual. Parents have influence on a child's development the most and play a big role. In particular, many parents these days believe that parent-child relationship is changing in a democratic way and a couple breeds their child together. However, in reality, the relationship has not been changed and is even regarded as more difficult situation than it was in the traditional society. Parents are oppressed with not being adapted to social changes and advance, and child also feels the same. Conclusion: Parents need education and support for child rearing without having any stresses. Health care provider consider this issues and to build a healthy parent-child relationship by helping parents.

• Journal of Distribution Science
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• v.12 no.8
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• pp.91-102
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• 2014

Purpose - Today, the importance of the service industry has increased. Social welfare businesses that follow the non-profit principle have neglected the distribution of the service. However, an intangible service, when produced and consumed, has a similar service delivery system to common service distribution and social welfare. Therefore, the author has assumed the efficiency of the service delivery system of social welfare to be equivalent to the service distribution industry. Hence, various kinds of social welfare services for the prevention of elderly suicide, from the perspective of distribution, are discussed. Studies on generational relations have thus far investigated depression from parent-children conflict, and satisfaction with their lives. Studies on the relationship between family solidarity and elderly suicide by analyzing existing social problems are rare. This study investigated the effects of family solidarity between old parents and adult children upon elderly suicide by analyzing serious psychological and social problems. This study revealed basic measures for elderly suicide prevention through services that could elevate family solidarity from the perspective of social welfare service distribution. Research design, data, and methodology - The subjects were 468 elderly, 65 years or older, at 18 senior welfare centers and halls for the elderly at Seoul and Gyeonggi-do. Questionnaire surveys, excluding invalid answers, were analyzed. Convenience sampling was used from February 5, 2014 to March 5, 2014. The findings were: First, women and those having spouses with advanced educational backgrounds and social activities had primary solidarity such as contact solidarity, affectionate solidarity, functional solidarity (giving help), and functional solidarity (receiving help). In addition, those who had good health, from a subjective point of view, had more solidarity. Second, the elderly who were men and single, and who had poorer educational backgrounds thought of suicide, lacked social activities, and had poor health, from a subjective point of view. Third, family solidarity between old parents and adult children had significantly negative influence on the suicidal ideation of the elderly. Results - The study revealed the following implications. First, the preparation for old age should consider not only economic welfare but also the psychological welfare due to the relationship with children. Second, the phenomenon of elderly suicide because of a lack of family solidarity could increase national loss and have great influence upon welfare in old age. Therefore, planning of welfare services for the elderly should consider the value of relationships with children to improve the psychological welfare of the elderly. Third, the social expenses of government-oriented support for parents should be discussed from the people's point of view. Conclusions - The subjects of the study comprised the elderly at Seoul and Gyenggi-do; therefore, it would be difficult to extrapolate the findings to all the elderly in the nation. The subject visited senior welfare centers as well as halls for the elderly; therefore, it would be difficult to assume that the trends were representative of the elderly in the nation.

• Journal of The Korean Association For Science Education
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• v.6 no.2
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• pp.15-33
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• 1986

The main purpose of this study is to analyze the fourth and sixth grade in the elemental)' science contents using piagetian thinking patterns. An analysis of the elementary Science contents used the systems of Han Jong-Ha(1977), Lee Won-Shic and Lee Sang-On(1979). They analyzed the science contents based on Piagets' cognitive developmental stages. According to the results of this study, science contents consist of more difficult concepts those of children's thinking level. In other words, 6.4-19.3 percent to 23.5-25.0 percent in the fourth and sixth grade of the science contents are consisted with abstract level concepts that formal operational children can perform. But each grade, 99.2-95.3 percent of children have a difficulty in understanding such a abstract level concepts. They are in the concrete operation thinking level. Therefore, we say that children have difficulty in understanding of science contents and may lose interest in the contents.

• Phonetics and Speech Sciences
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• v.4 no.3
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• pp.129-136
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• 2012

Korean alveolar fricatives are late-developing speech sounds. Most previous research on phonemes used individual words or pseudo words to produce sounds, but word-level phonological analysis does not always reflect a child's practical articulation ability. Also, there has been limited research on articulation development looking at speech production by grammatical morphemes despite its importance in Korean language. Therefore, this research examines the articulation development and phonological patterns of the /s/ phoneme in terms of morphological types produced in children's spontaneous conversational speech. The subjects were twenty-two typically developing 3- and 4-year-old Koreans. All children showed normal levels in three screening tests: hearing, vocabulary, and articulation. Spontaneous conversational samples were recorded at the children's homes. The results are as follows. The error rates decreased with increasing age in all morphological contexts. Also, error percentages within an age group were significantly lower in lexical morphemes than in grammatical morphemes. The stopping of fricative sounds was the main error pattern in all morphological contexts and reduced as age increased. This research shows that articulation performance can differ significantly by morphological contexts. The present study provides data that can be used to identify the difficult context for articulatory evaluation and therapy of alveolar fricative sounds.

• Journal of Korean Academy of Nursing
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• v.35 no.1
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• pp.5-15
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• 2005

Purpose: This study was conducted to develop a behavioral checklist to predict an autistic disorder and to identify the earliest detecting time. Method: One hundred and fifty eight children including normal, autistic, institutionalized normal, and retarded were assessed using critical interaction behavioral markers from literature review. Data was collected by semi-structured mother-child interaction by videotape recording and analyzed byfactor analysis, Cronbach a, Kappa, $x^2$, and Duncan. Result: Ten behavioral markers were sorted into 2 factors; joint-attention and synchronized behavior. Autistic children were impaired in pretend play, odeclarative pointing, proimperative pointing, gaze-monitoring, referential looking, showing, joint-attention, rhythmical vocal exchange, and synchronized laughing. The sychronized behavior was also a critical marker to predict the autistic disorder. However, it was difficult to differentiate autistic disorder from mental retardation. In addition, the appropriate detecting time was around 18 months after birth. Conclusion: This checklist should be behavior markers to predict autistic disorder and could be useful as educational material at children's clinics, parents class, and for caregivers in the health center. In addition, early detection should lead to treatment being started as soon after 18 months of age as possible.

• Journal of the East Asian Society of Dietary Life
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• v.21 no.1
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• pp.146-153
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• 2011

For the purpose of carrying out nutrition counseling effectively and efficiently, this study assessed the nutrition counseling practice, perception and nutrition knowledge of elementary school children in Gyeongbuk province. Survey questionnaires were distributed between November 2009 and December 2009 to 100 nutrition counseling experienced children (participants) and 110 nutrition counseling non-experienced children (non-participants) and were completed with nutrition teachers' assistance. According to the survey results, girls and overweight/obese children showed higher tendency of participation in nutrition counseling (p<0.001). Participants who took nutrition counseling according to their own intentions showed satisfaction after counseling on diverse subjects such as obesity, unbalanced diet, and weight control. Particularly, 'lack of counseling session time', 'unfavorable counseling condition' or 'difficult explanation' caused participants unsatisfaction after counseling. Participants usually perceived the meaning of nutrition counseling more correctly than non-participants and showed positive intentions for taking further counseling in the future(p<0.001). Diet good for weight control, good food to fix unbalanced diet and diet good for growing stature were the top three subjects of nutrition counseling chosen by subjects (p<0.001). Participants also showed higher nutrition knowledge scores than non-participants. Therefore, it is critically important to apply nutrition counseling in the proper environment (counseling room, time, teaching materials, etc) to elementary school students to fix healthy food habits. Therefore, nutrition teachers need to be provide professional nutrition counseling skills and knowledge.