• Advances in pediatric surgery
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• 제13권2호
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• pp.179-186
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• 2007

Lipoblastoma is a rare benign soft tissue tumor occurring in infancy and early childhood. It is characterized by fat lobules with varying degrees of maturity, multivaculoated lipoblasts, fibrocapillary networks and myxoid stroma. Lipoblastoma has a good prognosis with no metastases despite its potential for local invasion. From Jan, 1990 through April, 2007, 12 children underwent the operation for lipoblastoma, 7 boys and 5 girls, diagnosed at median 22 months (5 ~ 43 months). Median follow up was 6 year 7 months. Primary sites included back (n = 5), intraabdominal (n = 2) and one in each of buttock, chest wall, neck, nose and scalp. Tumors presented with a growing mass in 9 patients, abdominal distension in 2, and an incidental finding on chest radiography in one. Complete excisions were done in all patients. There was one recurrence in a patient with a scalp mass. After reoperation, he has been doing well without evidence of recurrence. Lipoblastoma has a favorable prognosis, but recurrence can occur even with complete excision. Regular follow up is necessary to detect recurrences.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제17권3호
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• pp.283-288
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• 1995

저자 등은 성인에서는 드물게 발생하는 것으로 알려진 낭포성활액종을 가진 22세 남자 환자에서 임상소견 및 방사선 소견상 우측 전경부삼각에 발생한 낭포성활액종의 가진하에 악하부 절개를 통해 낭종성의 종물을 악하선을 포함하여 완전 절제후 재발 등의 특별한 문제 없이 양호한 결과를 얻었기에 문헌 고찰과 함께 보고 드리는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.240-244
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• 1999

저자들은 만성설사와 범발성 혈관내 응고 증후군을 동반한 2개월된 남아에서 장병성 선단 피부염을 의심하여 아연을 경구 투여한 후, 증상호전을 보인 장병성 선단 피부염 환아 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.227-232
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• 1999

저자들은 발열, 복통, 관절통, 구강내 궤양의 증상으로 내원한 6세 남아에서 발생한 Crohn 병 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.42-44
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• 2018

Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

• East Asian Economic Review
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• 제24권4호
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• pp.313-336
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• 2020

In this paper, I review evidence on changing global specialization in labor-intensive exporting. Production of apparel, footwear, furniture, and related products are how many low-income countries first enter export manufacturing. Just as China's rise as a powerhouse in these goods supplanted a role previously occupied by the East Asian Tigers, the world may again be on the cusp of significant change in where labor-intensive goods are produced. China's prowess in these sectors peaked in the early 2010s; its share in their global exports, while still substantial, is now in decline. Mechanisms through which the global economy may adjust to China's graduation into more technologically sophisticated activities include expanded labor-intensive export production in other emerging economies and labor-saving technological change in products currently heavily reliant on less-educated labor. Available evidence suggests that the first mechanism is operating slowly and the second hardly at all. As a third mechanism, China may in part replace itself by moving labor-heavy factories out of densely populated and expensive coastal cities and into the country's interior. Such a transition, though still in its infancy, would mirror the decentralization of manufacturing production in the U.S. and Europe, which occurred after World War II.

• International Journal of Computer Science & Network Security
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• 제22권7호
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• pp.123-130
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• 2022

Researchers had theorized that the earliest years' experiences were extremely critical to a child's future development (Jung, 2008; Liu et al., 2020), and parental involvement was a key character (Lamb et al., 2002; Liu, 2020). Though the critical role of parental involvement in education has been confirmed, less attention has been paid to its influence on infants' development. For the sake of infants' appropriate development, infant teachers always met barriers to finding, designing, and implementing age-appropriate activities. This case study aims at exploring the role of parental involvement in the development in infancy by surveying one director, four infant teachers, and six early childhood pre-service teachers' perspectives and practices regarding parental involvement in infant rooms within a Reggio Emilia-inspired child development center. Findings revealed that all participants: (1) thought parental involvement was essential to their decision-making process; (2) were aware of the benefits of implementing parental involvement in infants' rooms; (3) gained an opportunity to reflect on their practices regarding parental in-volvement and reprocess their previous perspectives. This study recommends that, in order to facilitate infants' development appropriately, child-care centers need to take into account parental involvement and work with parents collaboratively.

• 대한유전성대사질환학회지
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• 제22권1호
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• pp.1-8
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• 2022

Long-chain fatty acid oxidation disorders (LC-FAOD) are an autosomal recessive inherited rare disease group that result in an acute metabolic crisis and chronic energy deficiency owing to the deficiency in an enzyme that converts long-chain fatty acids into energy. LC-FAOD includes carnitine palmitoyltransferase type 1 (CPT1), carnitine-acylcarnitine translocase (CACT), carnitine palmitoyltransferase type 2 (CPT2), very long-chain acyl-CoA dehydrogenase (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and trifunctional protein (TFP) deficiencies. Common symptoms of LC-FAOD are hypoketotic hypoglycemia, cardiomyopathy, and myopathy. Depending on symptom onset, the disease can be divided as neonatal period, late infancy and early childhood, adolescence, or adult onset, but symptoms can appear at any time. The neonatal screening test (NBS) can be used to identify the characteristic plasma acylcarnitine profiles for each disease and confirmed by deficient enzyme analysis or molecular testing. Before introduction of NBS, the mortality rate of LC-FAOD was very high. With NBS implementation as routine neonatal care, the mortality rate was dramatically decreased, but severe symptoms such as rhabdomyolysis recur frequently and affect the quality of life. Triheptanoin (Dojolvi^®), the first drug for pediatric and adult patients with molecularly confirmed LC-FAOD, has recently been approved by the US Food and Drug Administration in 2020. In this review, the diagnosis of LC-FAOD and treatment including triheptanoin are summarized.

• 한국아동복지학
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• 제54호
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• pp.79-111
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• 2016

본 연구는 영유아 시기에 경험하는 생애 초기 가족의 누적위험요인이 가정학습환경을 통해 유아의 언어 및 사회성 발달에 영향을 미치는 구조적 모델을 검증하는 데 그 목적이 있다. 만 3세 유아의 언어발달은 표현어휘력으로, 사회성 발달은 또래유능성으로 나타내었다. 한국아동패널(PSKC)의 2차년도와 4차년도 자료를 이용하여, 1,725가족을 대상으로 분석을 실시하였다. 상관관계 분석 및 구조방정식모델링 기법을 통하여 분석한 결과는 다음과 같다. 우선, 만 1세 누적위험요인과 만 3세 누적위험요인 간 상관관계는 높아, 누적위험요인의 종단적 지속성을 시사하였다. 만 1세 누적위험요인은 만 3세 가정학습환경의 질적 수준에 부정적인 영향을 미치고 가정학습환경은 유아의 언어 및 사회성 발달 모두를 유의하게 예측하였다. 하지만 만 1세 누적위험요인은 유아발달을 직접적으로 예측하지 않았다. 마지막으로 만3세 누적위험요인은 유아의 언어발달을 직접 예측하였으나 사회성 발달 및 가정학습환경에 유의한 영향을 미치지 않았다. 만 1세 누적위험요인${\rightarrow}$가정학습환경${\rightarrow}$유아발달의 구조적 관계에서 가정학습환경의 매개적 역할은 통계적으로 유의하였다. 종합하면, 영아기 가족 내 누적위험요인은 유아기 자녀의 발달을 돕는 가정학습환경 구성에 부정적인 영향을 미쳐 유아 발달에 간접적인 효과를 가지는 것으로 밝혀졌다. 영아기 자녀를 둔 위기가족을 대상으로 하는 조기 중재 및 지원에 대한 정책적 시사점이 논의되었다.

• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.580-584
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• 2007

다발성 경화증은 중추신경계 백질의 여러 부위를 시간 간격을 두고 침범하는 만성재발성 질환이다. 주로 20세에서 40세 사이에 발병하며 15세 이하 소아에서도 3-5% 정도 발병하는 소아기에는 매우 드문 질환이다. 원인은 아직 정확히 밝혀지지 않았으나 유전적, 환경적 및 감염과 연관된 자가면역반응 등 여러요인이 복합적으로 작용하는 것으로 생각하고 있다. 임상증상은 침범된 백질 부위에 따라 다양한데 사지근력 약화나 저림, 시력장애, 감각장애, 운동실조 등 다양한 증세로 호전과 재발을 반복한다. 본 저자들은 경련, 왼쪽 편마비 등의 증상으로 6세에 첫 발병 후 메틸프레드니솔론(methylprednisolon) 치료 후 증상 완전 회복 있었으나 6개월 후 경련, 두통, 왼쪽 안와주위 통증 등으로 다시 입원하는 등 4년간 추적 관찰 중 4차례 메틸프레드니솔론 치료 실시하였으나 다른 양상의 신경학적 증상으로 재발하여 Interferon-${\beta}$-1b($Betaferon^{(R)}$, SheringAG, Germany) 예방 치료를 실시한 다발성 경화증 환자 1례를 경험 하였기에 문헌 고찰과 함께 보고하는 바이다.