• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Journal of Oral Medicine and Pain
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• v.32 no.2
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• pp.157-175
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• 2007

This study was conducted through an interview process in which questionnaires were administered to 293 people. The questionnaires related to the behaviors of oral hygiene care, and disease history related to halitosis, and status of halitosis, halitosis measurement, oral examination, and caries activity tests such as the snyder test, Salivary flow rate test, and Salivary buffering capacity test. Our sample was taken from 293 rural residents within the period from 4th to 21st of January 2006. This was done in order to provide basic data to prepare both policies of halitosis prevention and a device to efficiently measure halitosis status and investigate the factors related therein. The major findings of this study results are as follows: 1. As for frequency of tooth brushing, twice a day occupied the greatest portion at 46.1 % Women exceeded men in frequency of tooth brushing. Tongue brushing everyday produced a 25.6 % result among subjects and The use of auxiliary oral hygiene devices occupied 9.2 %. 2. As for degree of usual self-awareness of halitosis: 62.5 %. This result also demonstrate that the severest time of self-awareness in regards to halitosis is wake up time in the morning. The time period produced the highest portion of 72.7 % in times of self-awareness. In terms of the area in which halitosis was observed, gum resulted in 23.0 %. As for types of halitosis, fetid smell was the most frequent at 37.2 %. 3. As for the result of halitosis measurement, values of OG less than 50 ppm occupied 54.3 % and $50{\sim}100ppm$ occupied 41.6 %. As for $NH_3$ values, $20{\sim}60ppm$ showed the highest value range of 52.6 %. 4. As for OG per disease history related to halitosis, values of OG were significantly high in the ranges of $50{\sim}100ppm$ within family history groups of food impaction by dental caries, diabetes mellitus and halitosis. As for values of $NH_3$, there showed a significant difference in respiratory system disease groups. 5 Value range of OG per ordinary halitosis self-awareness degree: values ranging less than 50 ppm were recorded at 55.9 % from the group realizing not aware of smell. 57.5 % from groups only realizing sometimes, while values range of $50{\sim}100ppm$ were recorded at 52.0 % from groups always aware of smell. 63.6 % from groups always strongly aware of smell. Meanwhile as for the values ranges of $NH_3$, $20{\sim}60ppm$. they occupied high portions for all groups of exams. 6. Values of OG per oral examination: the more pulp-exposed teeth and food impaction and the higher the tongue plaque index, values of OG increased within the range of $50{\sim}100ppm$. As for values of $NH_3$, the more prosthetic teeth and the higher the tongue plaque index, this value increased significantly, and the values increased up to no less than 60 ppm for groups of mandibular partial denture. 7. Within the realm of caries activity test: as for the Snyder test, high activity was highest by 43.0 % wherewith the higher the activity of acidogenic bacteria the higher the OG values. As for the salivary flow rate test, the number of cases below 8.0 ml showed the highest tendency by 62.5 %. The larger the salivary flow rate the more decreased OG values distribution. As for the salivary buffering capacity test, $6{\sim}10$ drops of 0.1N lactic acid showed the overwhelming trend by 58.7 % whereby the higher the salivary buffering capacity the greater distribution occupancy ratio of OG values below 50 ppm which is scentless to on ordinary person. 8. As for the correlation between oral environment and halitosis, OG showed the positive correlation with pulp exposed teeth, filled teeth, present teeth, tongue plaque index, and food impaction, while the negative correlation with salivary flow rate and prosthetic teeth. $NH_3$ showed a positive correlation with prosthetic teeth and frequency of tooth brushing, while decayed teeth was negative correlation. 9. As for the multiple regression analysis result, there have been selected female, pulp exposed teeth, prosthetic teeth, food impaction, salivary flow rate, tongue plaque index and severe activities in the Snyder test as factors affecting OG wherein explanatory power on it was 45.1 %. There have been selected females, pulp exposed teeth, tongue plaque index, and prosthetic teeth as factors affecting on $NH_3$ wherein explanatory power on it was 6.6 %. With the aforementioned results in mind, the status of halitosis among rural residents is considered to bare a close relation with oral environments and other factors related to halitosis such as the Snyder test from caries activity test, and salivary flow rate test. For the prevention of halitosis of residents in rural areas, we have to focus on correct tooth brushing methods and tongue brushing, with using auxiliary oral hygiene devices to remove fur of tongue plaque and food impaction. Also, when the cause and ingredients of halitosis are diverse and complex, in order to analyze exactly the factors of individual halitosis development, we need continuous and systematic study in order to provide rural residents with programs of oral hygiene education and encourage the use of dental hygienists in public health centers.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.73-77
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• 2008

Purpose : p16 gene, mapped to the 9p21 chromosomal region, has emerged as a candidate tumor suppressor gene in human neoplasm. It is an inhibitor of cyclin-dependent kinase and inhibits Rb phosphorylation. In a variety of tumors including childhood acute lymphoblastic leukemia (ALL), deletion and/or mutation of the p16 gene has been found. Despite their high frequency, the prognostic importance of p16 alterations is still controversial in ALL and has been reported to be either unfavorable or similar to that of other patients. We studied the correlation between loss of p16 protein confirmed by immunohistochemical staining and clinical outcomes of patients diagnosed as ALL. Methods : We performed an immunohistochemical staining for p16 protein in 74 cases of bone marrow biopsy slide initially diagnosed as ALL between January 1998 and December 2006. We reviewed the clinical manifestations, laboratory findings, treatment outcomes retrospectively. Results : Of 74 slides, 12 were negative for p16 protein. Seven were males and 5 were females with a median age at diagnosis was 5.8 (1.3-18.8) years. Initial WBC were 17,225 $(500-403,300)/{\mu}L$. By immunologic surface marker analysis, 7 patients were early pre-B CALLA (+) and 5 patients were T-cell ALL. Two patients of intermediate risk group had relapsed and died. Three patients had family history of breast cancer. Four patients died and overall survival rates were $53.5{\pm}18.7%$. Conclusion : Loss of p16 protein is supposed to be an independent risk factor of childhood ALL associated with poor outcomes. In clinical setting, the clinician must take into account p16 status, not only at the genomic but also at the protein level. Further clinical experience on thoroughly investigated cases will help a better understanding between p16 status and clinical outcomes.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.20-27
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• 2007

Purpose : Child sexual abuse is a significant and serious problem that affects public health and society. Pediatricians are responsible for preventing and detecting sexual abuse of children. The aim of this study was to examine clinical findings of child sexual abuse. Methods : The authors retrospectively studied 292 patients between the ages of 0 and 18 who were referred for evaluation of sexual abuse to the Emergency Department of the National Police Hospital from Oct 1, 2001 to May 5, 2005. Results : Sixty three (21.6 percent) of sexual abuse victims were younger than 6 years of age; 89 (30.5 percent) were 6-12 years of age; and 104 (47.9 percent) were 12-18 years of age. Attack time was the most common between noon and 6 pm. Victims were attacked in their home or nearby (51.4 percent). One hundred fifty six (53.6 percent) offenders were accquaitances, thirty nine (13.4 percent) of whom were family members. The disclosure of attack by parents with abnormal physical symptoms was more common in younger children than in adolescence. Eighty nine (30.5 percent) victims had no specific physical findings, 51 percent had injury to the hymen, 37.3% had injury to external genitalia, and 4 percent had anal injury. One case of gonorrhea and thirteen cases of chlamydia were found. Sperm was found in 19 cases and acid phosphatase was positive in 28 cases. The absence of physical findings and laboratory findings was more common in younger children than in those in adolescence. The time interval from attacks to hospital visits had a significant negative correlation with age. Conclusion : For younger victims, the time interval from attack to hospital visitis was more prolonged. Victims may have no physical evidence of sexual abuse. Therefore, a careful history should be obtained and a through physical examination should be performed. Pediatricians must play a leading role in evaluation and treatment of sexually abused children, considering children,s growth and development. Coordination with other professionals is necessary to provide consultation, medical treatment, and legal assistance for the sexually abused children and families.

• Journal of the East Asian Society of Dietary Life
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• v.18 no.4
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• pp.436-445
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• 2008

Stroke is a serious disease despite recent improvements in the medical treatments available. Thirty-six stroke patients were interviewed as case and 36 non-stroke patients were interviewed as controls between February 2005 and August 2005 at Daegu S Medical Center. Information regarding the subjects' food habits was collected using the recall method from 5 years before the onset of stroke. Using interview techniques, we investigated how dietary habits and attitude influence nutrient intake. These results were analyzed by the $X^2$, Student's t-test and Chi-square test using the SPSS 12.0 program. This case-control study was performed to demonstrate the relationships among general quality factors (BMI, WHR, and family history of stroke) and lifestyle factors (smoking, exercise and alcohol drinking). The results were as follows. Analysis of the percentage of nutrient intake in terms of meal pattern showed that WHR was significantly higher in the case group than in the control group. Hypertension and diabetes were the most common preceding diseases associated with the risk of stroke. The frequencies of smoking, alcohol consumption (p<0.001) and exercise (p<0.05) were higher in the cases than in the controls. Thus, the findings of this study are consistent with those of previous studies and suggest that people should be advised to control hypertension, smoking, alcohol drinking and obesity in order to prevent the occurrence of stroke as these factors are major risk factors for stroke.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.149-155
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• 1998

Thirty three members of the Korean Association of Pediatric Surgeons were surveyed to determine the clinical manifestations and current management of infantile hypertrophic pyloric stenosis (IHPS). The members completed a registration form and a questionnaire that indicated their treatment preferences for patients treated during 1996. The response rate was 84.8 %, and 23 institutions participated. Three hundred and sixty-three cases of IHPS were analysed. The male to female ratio was 5.15: 1. The presenting symptoms were nonbilious vomiting in 363 cases(100 %), palpable mass in the upper abdomen in 200 cases (55.1 %) and visible peristaltic waves in 67 cases(18.5 %). Fluid therapy was done in 161 cases(44.4 %) for preoperative preparation. Twenty patients(5.5 %) were premature. There were 27 low birth weight babies(7.7 %). The onset of symptoms ranged from 1 to 14 weeks of age with a peak occurrence at 2 to 4 weeks of age. Two hundred and thirty-six cases(66.6 %) presented within 4 weeks of age. There was one set of male twins and a family history of twins. The modes of feedings were: breast feeding; 69 cases(24.5 %), formula feeding; 176 cases(62.4 %) and mixed feeding; 37 cases(13.1 %). The preferred diagnostic modalities were ultrasonography (US) 229 cases(63.1 %), UGI series 27 cases(7.4%) and US and UGI series combined 86 cases(23.7 %). Only 21 cases(5.8 %) were operated on without imaging study, ie. with positive physical finding. Perforation of the mucosa during pyloromytomy occurred in 5 cases. All were treated successfully with primary closure. Persistent vomiting over 2 weeks after operation were reported in 5 cases. Three wound infections, one aspiration pneumonia and one postoperative bleeding were reported. There was no mortality in 363 cases.

• Journal of the Korean Institute of Landscape Architecture
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• v.41 no.3
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• pp.56-71
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• 2013

This study was carried out to investigate the construction background of multi-functional vacant grounds in the rear gardens of Kyeongbok Palace and Kyeongun Palace, by understanding the building history of multi-functional vacant ground during the Joseon Dynasty with a special reference to the places formed in the period of King Kojong. The multi-functional vacant grounds in the Palace were the important element from the political aspect of Joseon Dynasty, and became an infrastructure of a state - run organization. The vacant space between Kwangyeonlu(廣延樓) and Haeonjeong, the whole region of Yeolmujeong(閱武亭), Seochongdae(瑞蔥臺), and Chundangdae(春塘臺) were investigated as the multi-functional places, where the events were held for the royal family in the Changdeok Palace after the establishment of the Joseon Dynasty. Various events such as the archery competition, official examination and military training were held at the places. After the inauguration of King Kojong, Kyeongmudae(京武臺) was made in the process of the reconstruction of the Kyeongbok Palace. The Kyeonghee Palace was used as the off-palace rear garden after the year that King Kojong returned to the Kyeongun Palace. The characteristics of two spaces were to apply the space organization of the Changdeok Palace into the rear garden outside of Sinmumun(神武門), Kyeongmudae at Kyeongbok Palace and the pedestrian overpass was established to connect the spaces efficiently in Kyeonghee Palace. Especially, the multi-functional vacant ground in the rear garden during the period of King Kojong functioned as the place to show off the internal and external independence of a nation and to show the authority and dignity of King at the point of time that accelerated the plundering Joseon Dynasty by Japanese imperialism powers, beyond the meaning of national event space that had existed before, such as official examination or archery test. In addition, the fact that the multi-functional vacant ground used as training place for military exercise where many people were mobilized was considered to reflect the contemporary situation.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Journal of The Korean Association For Science Education
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• v.34 no.5
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• pp.499-510
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• 2014

The purpose of this research is to understand interactive learning during docent-led tours in a science museum focusing on scaffolding. We developed a scaffolding framework by collating the work of other researchers in related fields. The results show that scaffolding included three dimensions: purpose, interaction, and domain. The purpose dimension, divided into six categories, is related to the intention of the scaffolder and what the scaffolding are for: strategic, social, procedural, conceptual, verbal, and metacognitive. The interaction dimension reflects students' interaction with the scaffolder in two ways: dynamic (situation specific) and static (planned in advance). The domain dimension is related to two contents: domain-general and domain-specific (such as science). The scaffolding framework was applied to dynamic interactions between docents and visitors. The data was collected from elementary school students' family visits with the guidance of two docents at the Seodaemun Museum of Natural History. The data collected consisted of surveys, interviews, video-recordings, and transcripts. The analysis shows that five guiding contexts and scaffolding phases were recognized; 1) strategic scaffolding in a poorly illustrated exhibit; 2) conceptual scaffolding in a thoroughly explanative exhibit; 3) verbal scaffolding in misleading interpretation; 4) procedural scaffolding in a manipulative exhibit; and 5) metacognitive scaffolding with inaccurate content. In addition, the results show that the docents used the dynamic and static scaffolding synthetically so that the docent-led tour was effective. In conclusion, this study presents the usefulness of understanding visitors' science learning through the scaffolding framework, as well as the how docents can scaffold actively.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.69-76
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• 2000

Purpose: The Pathogenesis of primary noctllrnal enuresis(PNE) is still controversial. Genetic factor and maturational delay of micturition reflex including arousal disorder, lack of nocturnal Arginine Vasopressin(AVP) release and functional bladder capacity have been suggested. We analyzed the risk factors of PNE. Methods: Fifty five children with PNE (20 enuretics diagnosed at school physical examination of the first grade students at Mok-Dong Elementary School and 35 enuretics (Age 6-7 year) diagnosed at Ewha Womans University Mok Dong Hospital) and 221 control students without PNE at school physical examination were included. Genetic, stress and developmental factors, arousability, water intake, urine volume, maximun voiding volume and daytime voiding dysfunction were compared. Results: 1) There was no significant difference between PNE and control group in sex ratio, birth order, percentage of working mothers, parental and child personality, age to start walking, school record and duration of sleep. 2) Family history in the PNE group was significantly higher than control group ($20.0\%\;vs\;2.7\%$)(P<0.05). 3) The difficulty in arousal in the PNE group was significantly more common than the control group ($70.9\%\;vs\;54.3\%$)(P<0.05). 4) Nocturnal water intake in the PNE group was significantly greater than the control group ($330{\pm}158.2\;mL\;vs\;235{\pm}129.5\;mL$). Nocturnal urine volume in the PNE group was significantly greater than the control group ( $390{\pm}61.5\;mL\;vs\;140{\pm}43.2\;mL$)(P<0.05). Daily water intake and daily urine volume did not significantly differ between the two groups. 5) Maximum urine volume per void in the PNE group was significantly lower than the control group ($107{\pm}35.9\;mL\;vs\;236{\pm}41.3\;mL$). Daytime voiding dysfunction in the PNE group was significantly more common than the control group ($80.0\%\;vs\;57.9\%$). The voiding frequency in the PNE group was significantly greater than the control group ($7.0{\pm}3.6\;vs\;5.4{\pm}1.6$)(P<0.05). Conclusion: In addition to genetic factors and maturational delay of micturition reflex (difficulty in arousal, nocturnal polyuria and decreased functional bladder capacity) nocturnal polydypsia was found to be the important risk factors fur PNE. So nocturnal fluid restriction should be encouraged as the first-line management of PNE.