• Bulletin of the Korean Chemical Society
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• 제29권2호
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• pp.381-388
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• 2008

An X-linked skeletal disorder, SEDT (spondyloepiphyseal dysplasia tarda) is a genetic disease characterized by a disproportionately short trunk and short stature caused by mutations in the SEDL gene. This gene is evolutionarily conserved from yeast to human. The yeast SEDL protein ortholog, Trs20p, has been isolated as a member of a large multi-protein complex called the transport protein particle (TRAPP), which is involved in endoplasmic reticulum (ER)-to-Golgi transport. The interaction between SEDL and partner proteins is important in order to understand the molecular mechanism of SEDL functions. We isolated several SEDL-binding proteins derived from rat cells by an immobilized GST-fusion method. Furthermore, the SEDL-homologue proteins were identified using motif based methods. Common motifs between SEDL-binding proteins and SEDL-homologue proteins were classified into seven types and 78 common motifs were revealed. Sequence similarities were contracted to seven types using phylogenetic trees. In general, types I-III and VI were classified as having the function of acetyl-CoA carboxylase, glycogen phosphorylase, isocitrate dehydrogenase, and enolase, respectively, and type IV was found to be functionally related to the GST protein. Types V and VII were found to contribute to TRAPP vesicle trafficking.

• Clinical and Experimental Pediatrics
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• 제53권9호
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• pp.845-851
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• 2010

Purpose: Recombinant human growth hormone (rhGH) has been widely used to treat short stature. However, there are some concerns that growth hormone treatment may induce skeletal maturation and early onset of puberty. In this study, we investigated whether rhGH can directly affect the neuronal activities of of gonadotropin-releasing hormone (GnRH). Methods: We performed brain slice gramicidin-perforated current clamp recording to examine the direct membrane effects of rhGH on GnRH neurons, and a whole-cell voltage-clamp recording to examine the effects of rhGH on spontaneous postsynaptic events and holding currents in immature (postnatal days 13-21) and adult (postnatal days 42-73) mice. Results: In immature mice, all 5 GnRH neurons recorded in gramicidin-perforated current clamp mode showed no membrane potential changes on application of rhGH (0.4, $1{\mu}g/mL$). In adult GnRH neurons, 7 (78%) of 9 neurons tested showed no response to rhGH ($0.2-1{\mu}g/mL$) and 2 neurons showed slight depolarization. In 9 (90%) of 10 immature neurons tested, rhGH did not induce any membrane holding current changes or spontaneous postsynaptic currents (sPSCs). There was no change in sPSCs and holding current in 4 of 5 adult GnRH neurons. Conclusion: These findings demonstrate that rhGH does not directly affect the GnRH neuronal activities in our experimental model.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.137-140
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• 2011

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were $10{\times}6$ cm and $7.5{\times}4.5$ cm, respectively. His height was 155.1 cm (standard deviation score (SDS), -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and $9{\alpha}$-fludrocortisone; thereafter, the reduced testis size has been maintained.

• Clinical and Experimental Pediatrics
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• 제60권10호
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• pp.320-326
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• 2017

Purpose: This study aimed to evaluate a pilot project of the Nursing Feeding Center "Posyandu Plus" (NFCPP) through local food-based complementary feeding (LFCF) program designed to improve the nutritional status of children aged 6-36 months at community health centers in Indonesia. Methods: A quasi-experimental design was used to obtain data regarding the nutritional status of 109 children who participated in the project from 6 rural areas. The NFCPP was conducted for 9 weeks, comprising 2 weeks of preintervention, 6 weeks of intervention, and one week of postintervention. The LFCF intervention consisted of 12 sets of recipes to be made by mothers and given to their children 4 times daily over 6 weeks. The weight-for-age z score (WAZ), height-for-age z score (HAZ), weight-for-height z score (WHZ), and body mass index-for-age z score (BAZ) were calculated using World Health Organization Anthro Plus version 1.0.3. Results: LFCF intervention significantly increased WHZ, WAZ, and BAZ scores but decreased HAZ scores (P<0.001). Average scores of WHZ ($0.96{\pm}0.97$) and WAZ ($0.45{\pm}0.72$) increased; BAZ increased ($1.12{\pm}0.93$) after 6 weeks of LFCF. WAZ scores postintervention were 50.5% of normal, and WHZ scores were 77.1% of normal. However, the HAZ score decreased by $0.53{\pm}0.52$, which indicated 57.8% had short stature. Conclusion: The NFCPP program with LFCF intervention can improve the nutritional status of children in rural areas. It should be implemented as a sustained program for better provision of complementary feeding during the period of lactation using local food made available at community health centers.

• 대한장애인치과학회지
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• 제7권2호
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• pp.123-126
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• 2011

본 증례는 누난 증후군으로 진단 받은 환아에 대한 내용으로, 성장 지연, 작은 키, 좁고 높은 악궁 등의 특징적인 소견을 보였다. 체중은 8.8kg이었으며 신장은 100cm으로 2세 아이들의 성장 양상과 비슷한 수준을 나타내고 있었다. 이소맹출 되고 있는 매복 상악 측절치에 의해 상악 중절치의 맹출이 방해 받고 있었으며, 다수의 치아우식증이 관찰되었다. 이에 상악궁의 심한 총생이 예상되며, 측절치 치배로 인해 영구 상악 중절치 발육에 영향을 줄 것을 고려하여 전신마취 하에 측절치의 발거 및 치아우식증의 치료를 시행하였다. 주기적인 관찰기간 동안 영구 중절치의 발육에 큰 문제가 나타나지 않았으며, 정상적인 성장이 이루어지는 것을 관찰할 수 있었다.

• Clinical and Experimental Reproductive Medicine
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• 제26권2호
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• pp.275-280
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• 1999

Male sexual differentiation involves a cascade of events initiated by the presence on the Y chromosome of the of the SRY (sex determining region of Y chromosome) gene, which causes the indifferent gonad to develop into a testis. Hormonal products of the testis, predominantly testosterone and Mullerian inhibiting subtance (MIS), then control the sexual differentiation of the developing fetus. SRY is a transcription factor; however, target genes for its action have yet to be identified, because the DNA recognition sequence for SRY is found in many genes. Therefore the study of intersex disorders is being used to identify other genes active in the pathway of sexual differentiation. Patients with 46,XY gonadal dysgenesis, or Swyer's syndrome, have streak gonads, normal stature, and a sexually infantile phenotype with Mullerian structures present. The inheritance is usually sporadic but can be autosomal dominant or X-linked recessive. Unlike 45,X patients, stigmata of Turner syndrome are rare. As many as 20 to 30% of patients are at risk for malignant gonadal tumor formation and should undergo gonadectomy soon after the diagnosis is made. We have experienced a case of Swyer syndrome which showed a positive SRY gene in peripheral blood and gonad. So we report this case with a brief review of literatures.

• 한국가정과교육학회지
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• 제10권1호
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• pp.17-27
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• 1998

The purpose of this study was to investigate the recognition of body shape, the difference between self-evaluated obesity and obesity index of RBW, and attitude toward weight control of middle students. The questionnaires were completed by 195 male and 189 female students of liveing in Yongwol county. The average stature of the boys is 166.97$\pm$6.21cm, the girls is 158.13$\pm$5.83cm, and the average weight of the boys is 57.97$\pm$12.39kg, the girls is 52.88$\pm$8.35kg. 2. In the present body structure of them, the underweight structure is 6.0%, the normal weight structure is 87.2%, and the overweight structure is 6.7%. In the recognition of their own body structures, there are meaningful differences between boys and girls. 3. In the contentment degree of the present body structure of their own, the underweiht structure is much more satisfied with it.(p〈0.001) It shows a meaningful difference between boys and girls. 25.6% boys are satisfied with their own body structures, and 7.4% girls are satisfied with it.(p〈0.001) 4. In the interest degree about the weight's control, 87.1% is interested in it, it is lower in the underweight structure. 88.0% girls and 77.4% boys are interested in the weight's control. There is a meaningful difference between boys and girls, too.(p〈0.001) Each of the weight groups has differences in a experienced or experiencing for the weight's control. 5. The recognition degree of the present body structure showed the meaningful positive relation to the contentment degree of the present body structure and the weight's control.(p〈0.001)

• 안보군사학연구
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• 통권4호
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• pp.241-287
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• 2006

16th CPC National Party Congress established multi-polarization strategy as an external strategy, and 'The Great Reinvigoration of Chinese Nation' as the aim of The Great Strategy of China. It is required to maintain stabilized and peaceful surrounding circumstance in order to make it possible. The ideal surrounding circumstance that China is aiming for is to achieve multi-polarization of international society while maintaining strategic deterrence. Multi-polarization now in 'One super Multi powers' is that 'Multi powers' deter 'One super' to prevent predominance of USA and to decentralize the power. China wishes to realign international order as it accomplishes multi-polarization. China who wants 'Peace and Stabilization' emphasizes autonomy, peace and unification of Korean peninsula. Autonomy means the restraint intervention of foreign power, that is, American influence in Korean peninsula. Multi-polarization strategy of China will be used in Korean peninsula to solve nuclear issue of North Korea, to support neutral unification and to keep balance of power with USA, Japan and Russia as it renders economic advantages to South Korea and security engagement to North Korea, based on special geographical, cultural and historical relationship with two Koreas. The nuclear issue of North Korea will be a stage to test multi-polarization of China. When it deduces positive solution of nuclear issue due to successful six-party talks, and advances to security talks of Northeast Asia, it will contribute a lot to power and elevation of national stature of China in international society. Thus, the Chinese strategy will be an accommodative condition for the security of South Korea, and it requires wise decision of South Korea to make hay while the sun shine.

• 복식문화연구
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• 제20권3호
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• pp.309-318
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• 2012

This study aimed to find the components of visual image by researching main image-related adjectives applied to high-waist skirts. In addition, it compared and analyzed the differences and mutual influences of visual images according to variations in the waistline and skirt length of high-waist skirts. The researchers made nine high-waist skirts-combinations of three different levels of waistlines and skirt lengths. The test involved 121 people who hold degrees in the field of Clothing and Textiles. The panels tested actual images of all models wearing the high-waist skirts shown on a computer screen. The results of the study are as follows.: Four factors affected the visual image as waist-height and skirt-length changed: attractiveness, upper-body compensation, length compensation, and hips compensation. Among these factors, attractiveness was estimated as the most important factor. The torso and the hip were highlighted as the waistline increased from 3cm to 11cm, due to the optical illusion of a tighter skirt wrapping the bust, waist, and hips. As the waistline increased, the stature and leg length increased, possibly revising the overall appearance. For the skirt length, a length measuring 20cm above the knees, proved to be most attractive. This resulted in elongated height and leg length, and a slimmer overall look.

• Journal of Genetic Medicine
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• 제8권2호
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• pp.125-129
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• 2011

저자들은 고도근시, 망막이상, 납작한콧등, 구개열, 하악후퇴, 소하악증, 저신장, 양쪽 손가락의 관절구축증(arthrogryposis)이 있고, 방사선 검사상 불규칙한 대퇴골과 경골의 골단면, 척추골단이형성증이 보이는 Stickler 증후군 환자 1례에서 COL2A1 유전자의 새로운 돌연변이를 국내 최초로 증명하였기에 문헌고찰과 함께 보고하는 바이다.