• Bulletin of the Korean Chemical Society
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• v.29 no.2
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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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• v.53 no.9
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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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• v.54 no.3
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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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• v.60 no.10
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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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.7 no.2
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• pp.123-126
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• 2011

Noonan syndrome (NS) is a developmental disorder characterized by dysmorphic facial features in association with short stature, mental retardation and congenital heart disease. NS may be sporadic or inherited as an autosomal dominant or recessive trait. The children with NS usually have ocular hypertelorism, downslanting palpebral fissures, low-set ears and a webbed neck, chest deformity. In addition, oral features include micrognathia, high arched palate, dental malocclusion, dental anomalies and rarely, cleft palate. The phenotype of NS bears similarities to that of Turner syndrome. However, NS occurs in both males and females with a normal sex chromosome 46, XX and 46, XY constitution. This case presents the intermittent treatment of an 8-year-old girl who was referred from a local clinic for the extraction of supernumerary teeth and treatment of dental caries. The focus of this case report is the oral aspects on NS and particularities of the dental treatment in subjects affected by this genetic disease.

• Clinical and Experimental Reproductive Medicine
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• v.26 no.2
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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.

• Journal of Korean Home Economics Education Association
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• v.10 no.1
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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)

• Journal of National Security and Military Science
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• s.4
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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.

• The Research Journal of the Costume Culture
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• v.20 no.3
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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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• v.8 no.2
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• pp.125-129
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• 2011

Stickler syndrome is a very rare connective tissue disorder. The authors of the present study describe an 11-month-old girl with high myopia, retinal abnormalities, flat nose, cleft palate, retrognathia, micrognathia, short stature and arthrogryposis. Radiological evaluation also showed irregularity of the epiphysis of the femur and tibia and spondyloepiphyseal dysplasia. Genetic analysis using a peripheral blood sample revealed a novel variant c.787G>A (p.Gly246Asp) mutation of the COL2A1 gene. This is the first Korean case with Stickler syndrome confirmed by genetic testing.