• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.1-10
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• 2017

Purpose: This study aimed to investigate the perception and emotional experiences in rare and intractable diseases for caregivers of pediatric patients with mitochondrial diseases in order to provide therapeutic interventions for patients, caregivers, and families. Methods: A total of 83 caregivers of pediatric patients with mitochondrial diseases were recruited from the pediatric mitochondrial disease clinics of the Gangnam Severance Hospital in South Korea. Participants completed the survey about their perception of mitochondrial disease and emotional experiences after the diagnosis, and these clinical data were analyzed accordingly. Results: Surveys from a total of 83 caregivers of patients were analyzed, and the patients' age ranged from 6 to 12 years (33%), followed by ages 1 to 6 years (30%). Children with mitochondrial diseases were between 0 and 0.5 years of age at the time of first symptom onset (43%), and the duration of illness lasted more than 10 years in most cases (42%). Prior to diagnosis of mitochondrial diseases, the amount of awareness the caregivers had was 'Not at all' for both rare and intractable diseases and mitochondrial diseases in 44 cases and 68 cases, respectively. For the caregivers' emotional experiences, the most common initial responses were 'Discouraged/despair', 'Helpless/lethargic', and 'Disconcerted'. 'Anxious', 'Committed to treatment', and 'Responsibility as family members' were the most common emotional responses from the caregivers, followed by 'Disconcerted' and 'Helpless/lethargic'. Conclusion: It is important to consider the level of perception and emotional experiences of caregivers and patients with rare and intractable mitochondrial diseases for planning treatment programs.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.86-92
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• 2016

Purpose: The $BH_4$ loading test is an important test that distinguishes PKU from $BH_4$ deficiency and identifies the $BH_4$ reactivity of PKU patients. Phenylalanine and $BH_4$ loading tests are useful methods that can shorten the length of hospital stay while improving patients' convenience. However, sufficient research on the dose of phenylalanine loading and $BH_4$ administration time after the loading has not been carried out. The present study investigates the effectiveness of the existing phenylalanine loading method by analyzing the medical records of six patients who underwent the $BH_4$ loading test after taking 100 mg/kg of phenylalanine patients. Methods: The medical records of six patients who underwent the $BH_4$ load test after taking 100 mg/kg of phenylalanine were examined out of 207 patients who were followed up in the Genetic Metabolic Clinic in Soonchunhyang University Hospital. All of the six patients had a low phenylalanine diet. First, they were taking 100 mg/kg of phenylalanine. 3 hours later, 20 mg/kg of $BH_4$ were loaded. The phenylalanine levels in the blood were continuously measured at 1, 2, 4, 6, 8, 12, and 24 hours by setting the time the $BH_4$ was loaded as the basal. Results: The average of the highest phenylalanine concentrations of six patients was $20.0{\pm}11.70mg/dL$. One reached the highest concentration seven hours after taking phenylalanine; another reached it five hours after that, and the remaining three reached it four hours after that. Only one patient reached the highest concentration within three hours. The phenylalanine levels of four out of six patients (66%) rose above $400{\mu}mol/L$ after being loaded with phenylalanine. The phenylalanine levels of the remaining two were 6.1 mg/dL ($366{\mu}mol/L$) and 5 mg/dL ($300{\mu}mol/L$), respectively. Conclusion: One of six patients (16%) reached the highest concentration three hours after taking 100 mg/kg of phenylalanine and four patients (66%) reached $400{\mu}mol/L$ or higher phenylalanine levels. There were patients whose phenylalanine levels did not rise above $400{\mu}mol/L$ using a commonly known test method; moreover, this method had the disadvantage of reaching the highest concentration after more than three hours. Therefore, it is considered that taking 200 mg/kg or more of phenylalanine and performing $BH_4$ loading four to six hours after taking phenylalanine are helpful in proper diagnosis.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.154-161
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• 2003

Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, $12{\pm}9$ years; mean follow-up duration, $9{\pm}6.4$ years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. Results : Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P=0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. Conclusion : TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.14-21
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• 2014

Purpose: To analyze the clinical features and treatment outcome of Langerhans' cell histocytosis. Materials and Methods: From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively. Results: A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow-up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3-6 months observation, intralesional steroid injection was performed. Conclusion: Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3-6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.

• Journal of Internet Computing and Services
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• v.17 no.4
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• pp.145-159
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• 2016

This study points out the problems of absence of the legislation for standard regulation on Video on Demand(VoD) advertisement which grows so fast lately, for this it recommends making legal references, which have the definition of non-linear broadcasting & VoD advertisement and VoD advertisement standard regulation in the merged Broadcasting Act, and adopting co-regulation system. Pay TV operators providing VoD service have the opportunities to make money as subscribers uses it increasingly. In case of linear service, the Broadcasting Act regulates the advertisement strictly, but not the VoD ads. The reason why is that Korean legislation including the Broadcasting Act does not have legal reference to regulate it, instead of that, it rely on the self-regulation system which is operated by pay-tv players who provide the VoD ads. So, there is the limitation to protect the minors such as children and youth from the harmful VoD ads, to be invulnerable for advertisers to influence to advertising agents, and to ensure the regulatory effectiveness under player-centric self-regulatory regime. In this context, this study analyses the how to regulate VoD ads standard with a three-pronged approach. First, it analyses the VoD ads regulation system in overseas countries, UK, Canada, EU and Ireland. Each country has the legal reference to regulate it in the Broadcasting Act or lower statures and adopts the co-regulatory regime the NRA and the 3rd entity operate together. Second, it reviews the objectives and scope of VoD ads standard. This study recommends that the objective of it is users protection and the scope of it is standard regulation not commercial practice. Third, this study researches how to legislate for regulation of VoD ads standard. Considering VoD service's characteristics(non-linear service) and legal position of Ads agency(i.e. pay tv operators), it suggest that legal reference will be in the integrated Broadcasting bill, which is the general law, not individual. If it is available to regulate VoD ads standard with co-regulatory regime, it expects the enhancement of user protection from the harmful VoD ads and make up sustainability of the pay-tv players' self-regulation.

• Journal of Life Science
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• v.24 no.10
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• pp.1102-1109
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• 2014

Chungkookjang has several functional properties, such as fibrinolytic activity, anticancer effects, and antioxidant effects. However, children do not like Chungkookjang because of its foul odor. A mixed culture of Bacillus subtilis MC31 and Lactobacillus sakei 383 was used to improve the production of GABA in Chungkookjang and its flavor. Most of the foul odor of Chungkookjang was removed. The slime content and viscosity of Chungkookjang fermented in the mixed culture were similar to those of commercial Chungkookjang when B. subtilis MC31 and Lactobacillus sakei 383 were inoculated in a 1:1 ratio. The maximum GABA content was obtained when Chungkookjang was fermented with B. subtilis MC31 and L. sakei 383, which was fermented at $37^{\circ}C$ for 72 hr. During the period of fermentation, the viable cell number of B. subtilis MC31 reached a peak (log 9.13 CFU/g) at six days, and L. sakei 383 reached a peak (log 6.78 CFU/g) at two days. The moisture, crude ash, crude protein, crude fat, and crude fiber contents were 61.71%, 2.05%, 17.54%, 8.36%, and 1.95%, respectively. The amino-type nitrogen content of Chungkookjang fermented by B. subtilis MC31 and L. sakei 383 was less than Chungkookjang fermented by B. subtilis MC31 alone. The ammonia-type nitrogen and reducing sugar content of the Chungkookjang fermented by B. subtilis MC31 and L. sakei 383 were higher than that of steamed soybean. The glutamic acid and GABA content detected with an amino acid analyzer were 1.40 mg/g and 0.47 mg/g, respectively. These results suggest that fermentation with B. subtilis MC31 and L. sakei 383 in a 1:1 ratio removes more of the foul odor and increases the GABA content compared with single fermentation.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.63-74
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• 2009

Purpose : The current nationally representative data on inpatient care are important to make the of the national public health policy because distributions and the prevalence of diseases among children and adolescents represent the socioeconomic status of the society. The prevalence of chronic disease is increasing now in Korea as the socioeconomic condition is improving. We analyzed a part of genitourinary tract disease of the cross-sectional hospital discharge survey data in Korea collected recently to delineate the trend of genitourinary tract diseases. Methods : Korean nationwide hospital discharge survey for pediatric inpatients in the period from 2004 to 2006 was analyzed. Diagnoses in the data were coded using ICD-10 classification. Totally 826,896 cases were collected from the 85 training hospitals. Selected data of genitourinary tract diseases (belonging to N00-N99 by ICD-10) among 826,896 cases of final inpatients data were analyzed for this study. Results : Among total patients of 826,896, diseases of the genitourinary system accounted for 4.1%. and four diagnostic categories accounted for 92.8%. These were other diseases of the urinary system (N30-39), 45.8%, disease of male genital organs (N40-51),19.1%, glomerular diseases (N00-08), 17.3%, renal tubulo-interstitial diseases (N10-16), 10.6%, respectively. Conclusion : Genitourinary tract disease in pediatric inpatient shows decreasing tendency but the prevalence of chronic diseases is increasing in Korea as the socioeconomic condition is improving. For further comprehensive analysis, regular and organized nationwide survey should be performed. Development of a new data collecting system will improve the performance of such nationwide survey.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.1
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• pp.24-30
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• 2003

Purpose: To clarify the results of simple bone cyst (SBC) treatment in children by percutaneous autologous bone marrow grafting and xenografting. Materials and Methods: We studied seven cases (4 males, 3 females) of SBC, which were treated by percutaneous autologous marrow and heterograft bone grafting from January 1996 to February 1999. Their mean age at surgery was 10 years (6 to 15), and the mean follow-up period was 35.6 months (20 to 52). Three cases were located in the proximal and middle humerus; three cases were in the proximal femur; and one case occurred in the ilium. Mean volume was 14.7 $cm^2$ (10 to 23). Six cases were active, and one was inactive. Five patients had a history of receiving a mean of 3.2 steroid injections. The mean quantity of bone marrow used in treatment was 14.3 ml (10 to 20), and the mean amount of $Lubboc^{(R)}$ heterograft bone (Transphyto S.A. Clermont Ferrand, France) used was 6.4 blocks (5 to 10). Results were analyzed using the modified Neer classification. Results: Five cases completely healed with obliteration of the cyst cavity (Grade IV). Two cases demonstrated sclerosis around a partially visible cyst (Grade III). All treatment results were satisfactory and without intraoperative or postoperative complications. Conclusione: Percutaneous autologous marrow and heterograft bone grafting is recommended as an effective treatment method for simple bone cyst. It offers ease of operative technique, a high rate of healing, a low recurrence rate, low morbidity, a low incidence of postoperative complications, and free from bone graft donor site problems.

• Pediatric Infection and Vaccine
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• v.18 no.1
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• pp.23-30
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• 2011

Purpose : Acinetobacter baumannii is an aerobic, gram negative coccobacillus. Due to its pathogenicity and ability to accumulate diverse mechanisms of resistance, the importance of this organism is increasing. Many reports have targeted adults, and studies of pediatric patients are limited. This study aims to investigate the current status of A. baumannii infection in children. Methods : From January 2001 to December 2008, 505 patients hospitalized with A. baumannii infection were enrolled. Admission records for underlying disease, duration of hospitalization, previous antibiotic use, location of admission, presence of ventilator care, and resistance to antibiotics were retrospectively reviewed and analyzed. Results : Hemato-oncological disease and neurological disease were 30.6% and 24.3% of all cases; therefore, these were the most common underlying diseases of patients with A. baumannii infection. Prevalence of A. baumannii infection was 78.1% in patients with previous antibiotic use, which was higher than that of the group not using previous antibiotic. And prevalence of multi-drug resistant and pan-drug resistant A. baumannii infection was 76.4% and 38.3% in patients with ICU care, 76.8% and 38.9% with ventilator care, and these were higher than the others. Rate of resistance to all groups of antibiotics showed a gradual increase to over 50% in 2008. Multi-drug resistant A. baumannii was 63.5% and pan-drug resistant A. baumannii was 48.2% of all cases. Conclusion : Prevalence of A. baumannii infection and resistance to antibacterial agents of A. baumannii is increasing. Adequate use of antibiotics and infection control should be emphasized in pediatric patients.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.12 no.2
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• pp.759-764
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• 2011

This study was conducted to provide the basic data for the health management of the primary school, by finding and analyzing the factors affecting the degrees of atopic dermatitis symptom's severeness. The subjects of our study were collected 273 pupils mothers, whose children are undergoing through the atopic symptoms. The investigation process took the entire month of the November in 2006. The result of the older the pupils, the severer their symptoms(p<0.05). The longer the period(p<0.01), the more widely spread over the body, and the severer the symptoms(p<0.01). In the dietary habit, the more meat they eat, the severer the symptom(p<0.05). and in relation to the eating the food outside home, the more frequently they eat the fast-food, the severer the symptom. In the multiple logistic regression analysis, using the degrees of atopic dermatitis symptom's severeness as the dependent variables. it is discovered that the longer infection period they have and the more frequently they have the fast-food, the severer the symptom of the atopic dermatitis. The parent's love for the child, the monthly cost for the management of the infection, and the parent's feeling toward the child, can have a greate influence on the physical health, the feelings and the relation with the friends of the infected child, when the child shows the symptoms of scratching his skin. The conclusions of When all the results above are put together, the dietary habit of eating the meals mostly prepared with the meat should be corrected, and the education to form the pupil's right dietary habit, such as, putting the limit on the fast-food, must be made, in order to prevent the symptom of the atopic dermatitis from getting worse. And by the recognizing the atopic dermatitis as the family health problem, the comprehensive family health management, including the mother should be made.