• Title/Summary/Keyword: early infancy

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The Clinical Manifestations of Lipoblastoma in Children (소아 Lipoblastoma의 임상 양상)

  • Nam, So-Hyun;Kim, Dae-Yeon;Kim, Seong-Chul;Kim, In-Koo
    • Advances in pediatric surgery
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    • v.13 no.2
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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.

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CYSTIC HYGROMA IN THE ADULT (성인에 발생한 낭포성활액종)

  • Oh, Seong-Seob;Kim, Il-Gyu;Chun, Hye-Kyung
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.17 no.3
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    • pp.283-288
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    • 1995
  • Cystic hygroma is the benign proliferation of lymphatic tissue and has been categorized as a part of a larger spectrum that includes lymphangiomas recently. The majority of lymphangiomas occur in the head and neck as cystic hygromas with the posterior cervical region as the most common site. Cystic hygromas present in infancy or early childhood as compressible masses that may rapidly and intermittently enlarge. Cystic hygromas of the head and neck are especially difficult to manage since enlargement cause serious sequela such as airway obstruction, feeding difficulties, and speech pathology. Surgical exision remains the treatment of choice. But complete extirpation of these lesions is often impossible, and recurrence rates are accordingly different respectively ; these are high in suprahyoid lesions compared with infrahyoid involvement. This is a case report about 22 year old male patient with cystic hygrom. We obtained the successful, functional and esthetic results by surgical excision of the mass. Therefore, we report the case with literatural reviews.

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A Case of Acrodermatitis Enteropathica with Chronic Diarrhea and Disseminated Intravascular Coagulation (만성 설사와 범발성 혈관내 응고 증후군을 초래한 장병성 선단 피부염 1례)

  • Lee, Ji-Eun;Huh, Jun-Ho;Choe, Byung-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.2
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    • pp.240-244
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    • 1999
  • Acrodermatitis enteropathica, an autosomal recessive disease, usually presents with severe acral and circumorificial dermatitis, diarrhea, alopecia, intercurrent bacterial infection during early infancy, and is eventually fatal if left untreated. We report a case of acrodermatitis enteropathica in a 2-month-old male infant who presented with chronic diarrhea not responsive to conventional therapy and developed disseminated intravascular coagulation (DIC). He showed the characteristic eczematoid skin lesions, chronic diarrhea, failure to thrive, and low serum zinc concenturation. $Zn^{2+}$ was administered with dramatic improvement of skin lesions, DIC and diarrhea. He rapidly catched up normal growth and development on continuing zinc supplementation.

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A Case of Crohn Disease (Crohn병 1례)

  • Kim, Ji-Eun;Kim, Jun-Ho;Lee, Dong-Seok;Kim, Doo-Kwun;Choi, Sung-Min;Kim, Woo-Taek
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.2
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    • pp.227-232
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    • 1999
  • Crohn disease is a chronic transmural inflammatory disease that may involve any portion of the gastrointestinal tract. An increased incidence of Crohn disease in the general population has been reported, along with a greater than threefold increase of Crohn disease in children under the age of 16 years noted in a recent study. Crohn disease may be seen as early as infancy, but the most common pediatric age of onset is during the teenage period. We experienced a case of Crohn disease in 6 year old male child complained abdominal pain, oral aphthous ulcers, arthralgia, anorexia, and growth failure. A brief review of related literature is also presented.

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Growth Hormone Therapy in PWS with Mobile Application-Living with PWS

  • Im, Minji
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.4 no.2
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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.

Who Will Fill China's Shoes? The Global Evolution of Labor-Intensive Manufacturing

  • Hanson, Gordon
    • East Asian Economic Review
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    • v.24 no.4
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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.

Implementing Parental Involvement in Infants' Rooms: Explore Infant Teachers' Perspectives and Practices

  • Liu, Yanhui;Sulaimani, Mona F.
    • International Journal of Computer Science & Network Security
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    • v.22 no.7
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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.

Long-chain Fatty Acid Oxidation Disorders and Therapeutic Approach (장쇄 지방산 산화 장애와 치료적 접근법)

  • Lee, Jung Hyun
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.22 no.1
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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.

The Effects of Early Cumulative Risk Factors on Children's Development at Age 3 - The Mediation of Home Learning Environment - (유아기 발달에 대한 생애 초기 가족 누적위험요인의 영향 - 가정학습환경을 매개로 -)

  • Chang, Young Eun
    • Journal of the Korean Society of Child Welfare
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    • no.54
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    • pp.79-111
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    • 2016
  • The purpose of this study was to examine the structural models in which early cumulative risk factors affect children's language(indicated by expressive vocabularies) and social development(indicated by peer competence) at age 3 thorough their effects on the home learning environment. To examine the hypothesized models, the data of 1,725 families from the second and the fourth waves of the Panel Study of Korean Children was used. Correlation analysis and structural equation modeling were conducted to test the models. First, the cumulative risk factors at age 1 and 3 were highly correlated, implying the stability of the risk factors over time. The more cumulative risk factors at age 1 predicted the lower level of the home learning environment at age 3, which, in turn, was significantly related to both language and social development at age 3. However, the early cumulative risk factors did not directly influence later developmental outcomes. Moreover, the cumulative risk factors at age 3 were directly related to the child's language development, but neither social development northe home learning environment. In addition, the mediational role of the home learning environment (i.e., cumulative risk factors at age 1${\rightarrow}$home learning environment${\rightarrow}$language and social development) was statistically supported. In conclusion, the early cumulative risk factors in infancy indirectly predicted children's development at age 3 through the home learning environment. The practical implications for the early intervention and support for the families with infants who are experiencing multiple risk factors were discussed.

A case of childhood relapsing/remitting multiple sclerosis and interferon β-1b treatment in a Korean patient (소아 재발/완화형 다발성 경화증 환자에서 인터페론 베타 1b 치료 1례)

  • Kim, Hyun Seok;Lee, Won Deok;Lee, Jun Hwa;Cho, Kyung Lae
    • Clinical and Experimental Pediatrics
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    • v.50 no.6
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    • pp.580-584
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    • 2007
  • Multiple sclerosis (MS) is a demyelinating disorder that affects discrete areas of the CNS, including the optic nerves, in a quite variable relapsing-remitting fashion over a prolonged period of time. Although MS is usually considered to be a disease that affects peoples in early to middle adulthood, children do develop multiple sclerosis. The frequency of MS onset before the age of 15 years is 2.7-5% of all cases, while MS onset during infancy and early childhood was observed to be 0.2-0.7% of all cases. We report here on a Korean case of a relapsing-remitting MS female child who was treated with four rounds of intravenous methylpredinsolone pulse therapy and preventive Interferon-$\beta$-1b ($Betaferon^{(R)}$).