• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.312-316
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• 2006

Purpose : Growth hormone(GH) has been recognized as an effective treatment for short children born small for their gestational ages(SGA), and nowadays it has been widely used for the treatment of short children born SGA. The aim of this study is to assess the efficacy of GH treatment for the children born SGA. Methods : The study population was made of 40 short children born SGA with GH-treated(n=26) and untreated control group(n=14). In order to evaluate the effect of GH treatment, the changes in standard deviation scores(SDS) of the GH-treated group were compared to the changes in SDS before and after treatment from the control group in the same period. Results : There were no differences between the GH-treated group and the control group in gestational age, birth weight, chronological age, target height and the period of follow-up observation; however, the GH-treated group had lower height SDS($-3.3{\pm}0.9$) than the control group($-2.4{\pm}0.4$) before treatment(P<0.05). The GH-treated group had gained $1.2{\pm}1.0$ height SDS during GH treatment while the control group had gained $0.5{\pm}0.6$ height SDS. In the GH treatment group, HDL-cholesterol increased from $48.5{\pm}9.9mg/dL$ to $56.1{\pm}8.7mg/dL$(P<0.05) and LDL-cholesterol decreased from $88.1{\pm}23.3mg/dL$ to $76.4{\pm}19.4mg/dL$(P<0.05) after treatment. There were no changes in total cholesterol, triglyceride, free fatty acid and fasting blood sugar. IGF-I increased from $224.9{\pm}191.3{\mu}g/L$ to $443.2{\pm}152.5{\mu}g/L$(P<0.05) and IGFBP-3 also increased from $3.7{\pm}1.3mg/L$ to $5.6{\pm}1.2mg/L$(P<0.05). Conclusion : We conclude that growth hormone treatment is effective in the treatment of short children born SGA.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Radiation Oncology Journal
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• v.15 no.3
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• pp.207-213
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• 1997

Purpose : Intracranial germinoma is the most radiocurable tumor of theprimary intracranial neoplasm. But, the optimum radiation dose and target volume remain controversial In this retrospective study, we analysed the spreading pattern at presentation and the pattern of the failure and survival of intracranial germinoma, Materials and Methods : From 1989 to 1996, 23 Patients were treated for intracranial germinoma at Department of Radiation Oncology, Twenty-one Patients were treated at their initial Presentation and 2 Patients were treated for recurrent disease. Six patients had multiple tumor masses on MRI and 7patients had ventricular seeding on MRI. The examination of cerebrospinal fluid cytology was done in 15 patients and 3 out of 15 patients had positive cerebrospinal cytology. In tumor marker study of $\alpha-FP\;and\;\beta-hCG$, 6 patients had mildly elevated $\beta-hCG$ in serum or cerebrospinal fluid. Twentyone Patients were treated with whole craniospinal axis irradiation and 2 Patients were given whole ventricular radiation therapy. The total dose was ranged between 4500cGy and 5600cGy to primary tumor site (median 5580cGy) Dose to the entire ventricular system ranged from 1980cGy to 3960 cGy (median 2700cGy) and dose to the spinal axis ranged from 2160cGy to 3900cGy (median 2700cGy) Results : Of 23 patients, 21 Patients are alive without evidence of diseasefor median 4 years follow-up. One Patient who had markedly elevated $\alpha-FP\;and\;\beta-hCG$, suffered from Persistent disease after radiation therapy and received 2 cycles of chemotherapy. She died 9 months after chemotherapy One patient who developed ventricular seeding after gamma-knife was treated with whole craniospinal irradiation, he died after 1 year due to probably brain necrosis. The hematologic toxicity of 3 or 4 grade were seen in 7 patients and patient's endocrinologic dysfunction was not deteriorated after radiation therapy. One patient had been treated with growth hormone replacement due to short stature. Conclusions : This retrospective study has confirmed the excellent result of radiation therapy in intracranial germinoma. The complication rate during or after radiation therapy is considered within acceptable range. ft is necessary to further investigate the optimal dose and treatment volume of radiation therapy. The role of chemotherapy in the treatment of intracranial germinoma should be further investigated.

• Journal of Science of Art and Design
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• v.7
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• pp.5-63
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• 2005

Generally, the meanings of destruction are related in the meaning of demolition, breakdown, into fragments ... and so on, and the similar meanings are twist, crush, demolish, split, cut, into pieces , break up ... etc. Further, it has related in the cruelty and destructive heart which are linked with orgy, Sadism, Necrophilia and so on. The meanings are also expressed by the initial , which are deprivation, deface, defame, deform, degrade, delegitimize, denounce , deride, destroy, devalue, as well as debase, debunk, declaim, declassify, decry, delete, denigrate, deprecate, despise or detract ...and so on. Dario Gamboni has discussed the meaning in his book as two categories Iconoclasm and Vandalism. And the similar meanings could be found in the words which has the initial of , like abase, abate, abhor, abjure, abolish, abridge, abuse ...and so on. Even though the distinct meanings of Iconoclasm and Vandalism, it is not easy to distinguish clearly between the differences when the results are accomplished in contemporary paintings because of the similarity of the results. In korean vocabulary there are no similar words to distinguish between the meanings of destruction and deconstruction, and the deconstruction is not recorded in the general dictionaries. However the meaning of is diminishing, separation, contrast and so on. So the unification of the word as do-construction is not construct, minus construction, reverse construction. And Vincent Ditch explained that there are the meaning of destroy the text. From Jacques Derrida, the deconstruction strategy is to criticise the world of traditional metaphysics and logocentrism, and not to reconstruire the philosophical meaning of texts but $d\'{e}construire$ them. And Saussure emphasized that the signifers could have more meaning that there can be more signified in traditional texts in the art. as a result, deconstruction is explained that there are many signified meanings in a signifer. In this thesis , from using the meanings of destruction and deconstruction, to distinguish the expressive skills in contemporary art works are arising. Therefore, special methods which are linked in the destruction styles are selected. As a result, the two different purposes of destruction is arising, one is enmity destruction and the other is self destruction another word, auto destruction or destruction to create The enmity destruction can be distinguished by the two category Iconoclasm and Vandalism. They come from the moment of different historical aspect is arising and want to attack the Icon or masterpiece this concept is from the study of John Philips and especially iconoclasm is linked with religious and artistic heart, but Vandalism is come from the political attack. Sometime, this distinguish is not clearly arising, because the two aspects are co-related in the attack. As a result, firstly, the Iconoclastic controversy had arisen in the methods of Dadaism which has developed by Man Ray, Francis Picabia and Marcel Duchamp. They want to attack the pre-established master-pieces and painting spaces, and they had 'non-artistic attitude' not to be art. Since 1980, the German artist Anselm Kiefer adapted the methods and made them his special skills so he had tried to paint tough brush strokes and draw with hugh pallette image line and fire and water images , they can be the image attack as the Iconoclasm. secondly, the model of vandalism is to be done by hammer, drill, canon and so on. the method is to attack the content of painting. Further, the object of destruction is bound by cords and iron lings to demolish or to declare the authority of pre-statues; it symbolize the pre-authority is gone already. Self-destruction based paintings are clearly different in the purpose of approaching the art work. First of all, they can be auto-destruction, creative destruction and metamorphosis destruction, which is linked with the skill the material aspect and basic stature, and sign destruction or signifier destruction, which is link with the inner meaning destruction that is considered as the Semiotical destruction in post-modern paintings. Since 1960, the auto destruction is based on the method of firing, melting, grinding and similar skills, which is linked with Neo-Dada and reverse-assemblage. Metamorphosis destruction is strongly linked with the basic inner heart price and quality, so it can be resulted in the changedness of expectation and recognition. Tony Cragg has developed the skills to metamorphose the wood as stone or iron as cloth and stone as sponge and rubber and so on. The researcher has developed the same style in the series of since 2003. The other self-destructive methods are found in the skill of sign destruction. In the methods the meaning of the art is not fixed as one or two, but is developed multi-meaning and differ from original starting situation, so Jacques Derrida called the difference meaning in deconstruction. It is the destruction of textes. These methods are accomplished by David Salle, Francesco Clemente, and recently Tracy Emin, who has developed the attacking heart in the spectators' emotion. Sometime in the method of self-destruction, it is based on horror and shock, the method is explored by Demian Hirst and Jakes and Dinos Chapman. Their destructive styles stimulate ambivalent heart and destroy original sign of girl and animals.

• Journal of the Korean Society of Food Science and Nutrition
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• v.5 no.1
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• pp.87-92
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• 1976

Comparing the amount of Hb, the nutriture of the deaf-mutes are better than that of the amentias and the nursery school children. The former, however, shows less condition than that of the boys and girls in the junior physical education school (Hb : 12.7) and the normal family children (Hb : 10.8). In the group of six to eleven years old that is a class a elementary school, the physical condition such as stature, chest circumference, and weight of the deaf -mute is respectively almost the same degree, which is above standard in our country. On the contrary the degree of the amentias and the nursery school children has an inferiority approaching to the standard. In the group of twelve to fifteen years old that is a class of junior high school, the nursery school children have the lowest physical condition and the boys and girls of the physical training school the highest. The order of the growth of physical condition is as follows : Nursery school children

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.191-196
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• 2005

Purpose : Short stature is one of the characteristic features of Turner syndrome. We investigated the factors affecting final adult height(FAH) in patients with Turner syndrome. Methods : The study group was comprised of 60 patients who were diagnosed with Turner syndrome by chromosomal study and clinical phenotypes and attained FAH. Data were obtained from retrospective review of the medical records. We analyzed the factors influencing FAH in growth hormone(GH) treated and GH untreated groups. Results : Sixty patients were enrolled; 48 patients received GH treatment, and 12 patients did not. Mean duration of GH treatment was 35.8 months(range 4 to 120 months), and mean dosage of GH was $0.8{\pm}0.2IU/kg/wk$ in GH treated group. Mean growth velocity was $5.6{\pm}2.0cm/yr$, which was significantly higher than that during pretreatment period. In the GH treated group, mean chronological age, bone age, mean height, and height standard deviation(SD) score at GH treatment were $12.2{\pm}2.7yr$ $10.3{\pm}2.5yr$ $127.5{\pm}10.1cm$ and $-3.1{\pm}1.1$, respectively. In the GH treated group, the mean FAH and SD score of FAH were $146.9{\pm}5.8cm$ and $-2.7{\pm}1.2$, respectively, which showed significant differences compared with those of the GH untreated group. Analyzing the factors affecting FAH in GH-treated patients, only the SD score of height at the time of treatment was significantly related to FAH. Conclusion : GH treatment leads to an increment in FAH in patients with Turner syndrome. Average FAH gain was as much as 5.8 cm. SD score of height at the time of GH treatment was the only factor influencing FAH.

• Journal of Animal Science and Technology
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• v.51 no.5
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• pp.345-352
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• 2009

This study utilized 332,625 records of linear type scores consisting for 15 primary traits, 22,175 final score and 84,612 pedigree information of 22,175 Holstein cows from 1993 to 2007 in Korea to estimate genetic parameters for 16 type traits. Genetic and error (co)variances between two traits selected from 16 traits were estimated using bi-trait pairwise analyses with DFREML package. The estimated heritabilities for stature (ST), strength (STR), body depth (BD), dairy form (DF), rump angle (RA), thurl width (TW), rear legs side view (RLSV), foot angle (FA), fore udder attachment (FUA), rear udder height (RUH), rear udder width (RUW), udder cleft (UC), udder depth (UD), front teat placement (FTP), front teat length (FTL) and final score (FS) were 0.31, 0.21, 0.25, 0.10, 0.29, 0.19, 0.09, 0.06, 0.12, 0.13, 0.12, 0.08, 0.26, 0.20, 0.28 and 0.15, respectively. ST had the highest positive genetic correlation with BD (0.90), while RLSV had the highest negative genetic correlation with FA (-0.56). RA had negative genetic correlation with most udder traits (-0.17~-0.02). Especially, RUW had the higher positive genetic correlation with STR (0.60), BD (0.62), and TW (0.49), however, UD had the higher negative genetic correlation with STR (-0.40) and BD (-0.40). FTL had negative genetic correlation with FUA, RUH, RUW, UC and UD. FS had positive genetic correlation with UC, UD and FTP (0.12, 0.18 and 0.20). However, additional research is needed on the use of these parameters in the genetic evaluation because estimated genetic and error variance-covariance matrices were not positive definite.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.1
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• pp.51-57
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• 2010

Purpose: It has been reported that children with hypopituitarism have features of metabolic syndrome, including obesity, impaired glucose tolerance, and dyslipidemia. The aim of this study was to investigate the clinical features and liver histology of pediatric non-alcoholic fatty liver disease (NAFLD) associated with hypopituitarism. Methods: We reviewed the clinical data of 11 children diagnosed with NAFLD among patients with hypopituitarism. Results: The mean age at the time of diagnosis of hypopituitarism was 10.4${\pm}$3.2 years, and the mean age at the time of diagnosis of NAFLD was 13.1${\pm}$2.7 years. A craniopharyngioma was the most common cause of pituitary dysfunction. At the time of diagnosis of NAFLD, 9 patients (82%) had a body mass index greater than the 85th percentile, 5 patients (45%) had elevated fasting blood glucose levels, and 9 patients (82%) had hypertriglyceridemia. The mean height SD score was significantly lower at the time of diagnosis of NAFLD than at the time of diagnosis of hypopituitarism. Of the six patients who were biopsied, one had cirrhosis, two had non-alcoholic steatohepatitis (NASH) with bridging fibrosis, two had NASH with mild portal fibrosis, and one had simple steatosis. Conclusion: Children with hypopituitarism are at risk of short stature, obesity, dyslipidemia, and NAFLD. The early diagnosis of NAFLD is important in children with hypopituitarism because advanced fibrosis is common.

• Korean Journal of Community Nutrition
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• v.7 no.1
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• pp.121-129
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• 2002

Osteoporosis, the typical metabolic bone disease of the elderly, is characterized by a reduction in bone mineral density (BMD) and increased fracture risk. Genetic and environmental factors are known to play a key role in bone metabolism, and diet is also considered to be one of the important factors. The purpose of the present study was to investigate the relationship among the factors affecting BMD, including stature, body weight, age, time period since onset of menopause, and biochemical markers of bone turnover in postmenopausal women. Seventy-eight postmenopausal women who visited health promotion center for health examinations volunteered to participate in this study and they were divided into two groups according to the time period since onset of menopause : women with a time period since onset of menopause of less than 5 years (Group 1) and women with a time period since onset of menopause of 5 years or more (Group 2). The demographic characteristics and dietary intake were surveyed using a questionnaire. BMDs of the lumbar spine and femoral neck of subjects were measured by dual energy X-ray absorptiometry. Serum levels of 25-hydroxy-vitamin D and parathyroid hormone (PTH), known to be indicators of bone related hormone status, were anlyzed. Serum samples were measured for calcium, phosphorus, alkaline phosphatase, and osteocalcin as bone formation indicators, and urine was analysed for deoxypyridinoline, creatinine, calcium, and sodium as bone resorption indicators. The results are as follow : The mean BMDs of the lumbar spin and femoral neck were $1.02 \pm 0.02 g/cm^2 and 0.81 \pm 0.02 g/cm^2 respectively, and the BMD level of Group 2 was significantly lower than tat of Group 1 (p<0.01, p<0.05, respectively). The mean daily intake of energy was 1838 $\pm$ 55 kcal. When nutrient intake was compared with the recommended dietary allowances (RDA) of the subjects, only calcium, vitamin A and riboflavin intake showed means lower than the RDA. The nutrient intake did not show any significant differences between Group 1 and 2 Serum and urine levels of biochemical markers of bone turnover did not show any significant differences between Group 1 and 2, and all were within the normal range. However, the PTH and deoxypyridinoline levels showed a tendency to be higher, and the osteocalcin level to be lower in Group 2 than in Group 1. Although age and years after menopause (YAM) showed negative correlations with lumbar spine bone mineral density (LBMD) (r= -0.38, p<0.001, and r= -0.26, p< 0.05, respectively), no correlation was found with femoral neck bone mineral density (NBMD). While height, body weight and body mass index (BMI) showed a positive correlation with LBMD (r= 0.32, p<0.001, r= 0.38, p<0.001, r= 0.22, p= 0.05, respectively), only body weight and BMI showed a positive correlation with NBMD (r= 0.30, p<0.01, and r= 0.27, p<0.05, respectivley). There was no significant corealtion between BMDs and the nutrient intake of subjects, except in the case of carbohydrates (r= 0.22, p<0.05). Also, serum and urine levels of bone turnover markers showed no significant correlation with nutrient intake. On the other hand, serum osteocalcin had a positive correlation with vitamin C intake (r= 0.22, p= 0.05), and urine deoxypyridinolin showed a negative correlation with niacin intake (r= -0.22, p= 0.05). Urinary na was negatively correlated with protein intake(r= -0.23, p= 0.05). The results suggested that it is difficult to prevent the decrease in bone mass among postmenopausal women eating the usual Korean diet. However, the BMDs of the lumbar spine and femoral neck were positively related to body weight ad BMI in postmenopausal women. Therefore, this study confirmed that one of the most effective ways to minimize bone loss in postmenopausal women would be to maintain an adequate body weight with balanced nutrient intake and activity in the pre-and postmenopausal periods.