• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Journal of Gastric Cancer
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• v.8 no.3
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• pp.120-128
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• 2008

Purpose: Mistletoe extract was widely used for cancer treatment as complementary or alternative therapy in European area from early twenty century. It is currently used as alternative anti-cancer remedy by piecemeal in domestic medical group, however, the anti-cancer mechanism of mistletoe extract was not known precisely until now. In this study the effect of mistletoe extract on gastric cancer was studied vis cell line experiments. Materials and Methods: The SNU719 gastric cancer cell line was used, and ABNOBAviscum-Q and ABNOBAviscum-F were treated to cells as mistletoe extract, or 5-FU and cisplatin were used with mistletoe extract. The cell viability and cell death rate were estimated by CCK-8 assay kit and lactate dehydrogenase (LDH) assay kit in each. Caspase 3 assay kit was used to measure caspase 3 activity. The protein expression amounts of Bcl2, p53, and PTEN were estimated through Western blot analysis. Results: The co-treatments of mistletoe extract Q/F and 5-FU/cisplatin decreased lesser cell viability than only mistletoe treat. Caspase 3 activity was increased 4~6 times in co-treatment of mistletoe extracts and 5-FU than control. Bcl2 protein expression was reduced by mistletoe extracts or anti-cancer drugs, further more, the co-treatment of mistletoe extracts and 5-FU/cisplatin diminished more the expression than only mistletoe treatment. Mistletoe extracts did not affect the protein expressions of p53 and PTEN. Conclusion: It was concluded that the anti-cancer mechanism of mistletoe extracts was made by caspase 3 activation and lowered Bcl2 expression, and this apoptosis inducing mechanism was independent to p53.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.247-254
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• 2007

Purpose : Continuous renal replacement therapy(CRRT) has been the first choice for the treatment of acute renal failure in critically ill children not only in western countries but also in Korea. However, there are very few studies that have analyzed the outcome and prognosis of this modality in Korean children. We performed this study to evaluate the factors associated with the outcome and prognosis of patients treated with CRRT. Methods : We retrospectively reviewed the medical records of 32 children who had received CRRT at Severance hospital from 2003 to 2006. The mean age was 7.5 years(range 4 days-16 years) and the mean body weight was 25.8 kg (range 3.2-63 kg). Results : Eleven(34.4%) of the 32 patients survived. Bone marrow transplantation and malignancy were the most common causes of death and underlying disease leading to the need for CRRT Mean patient weight, age, duration of CRRT, number of organ failures, urine output, estimated glomerular filtration rate(eGFR), C-reactive protein, and blood urea level did not differ significantly between survivors and nonsurvivors. (1) Pediatric risk of mortality(PRISM) III score at CRRT initiation($9.8{\pm}5.3$ vs. $26.7{\pm}7.6$, P<0.0001), (2) maximum pressor number ($2.1{\pm}1.2$ vs. $3.0{\pm}1.0$, P=0.038), and (3) the degree of fluid overload($5.2{\pm}6.0$ vs. $15.0{\pm}8.9$, P=0.002) were significantly lower in survivers than in nonsurvivors. Multivariate analysis revealed that fluid overload was the only independent factor reducing survival rate. Conclusion : CRRT was successfully applied to the treatment of acute renal failure in a wide range of critically ill children. To improve survival, we suggest the early initiation of CRRT to prevent the systemic worsening and progression of fluid overload in critically ill children with acute renal failure. (J Korean Soc Pediatr Nephrol 2007;11:247-254)

• Journal of Hospice and Palliative Care
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• v.19 no.2
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• pp.163-169
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• 2016

Purpose: The unmet medical service needs of caregivers critically influence their caring for terminal cancer patients, but not much research has been done in this regard. Thus, the purpose of this study is to investigate the association between caregivers' characteristics and their unmet medical service needs. Methods: The survey was conducted with 109 family caregivers of terminal cancer patients admitted to four hospice units. The data were collected from March 2014 through December 2014 using a structured questionnaire. The unmet medical service needs were measured using 14 items which were adopted and modified by authors. Results: Seven areas of unmet medical service needs were shown to be significant. A well-educated group showed stronger needs for counsel about cancer screening and complementary-alternative medicine and health supplement food. A never-smoked group was identified with less need for sexual dysfunction counsel. Counsel about family and personal relations was more necessary for current drinkers and current workers, and less necessary for the married. Insurance counsel was more needed for a no-religion group. Occupation counsel was less necessary for healthy patients. Financial support was less necessary for the married group. Conclusion: Based on the results, it is highly recommended to further investigate the unmet medical service needs of family caregivers for terminal cancer patients and causes of the unmet needs.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.565-569
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• 2007

Purpose : With a duration of action of approximately 24 hours and peakless levels, Lantus is a more physiologic basal insulin analogue compared with NPH. The aim of this study was to compare the glycemic control of Lantus plus Humalog with that of premixed insulin in children and adolescents with type 1 diabetes mellitus. Methods : The subjects consisted of 25 patients with type 1 diabetes mellitus, aged 12-19 years, who changed their insulin regimen from premixed insulin to Lantus plus Humalog. Daily insulin doses, frequency of hypoglycemia, fasting blood glucose, C-peptide concentration and HbA1c before and 6 months after Lantus treatment were compared. 24 hour blood glucose of 11 patients among Lantus treatment group (n=25) and premixed insulin treatment group (n=10) were self-monitored and compared. Results : 6 months after Lantus treatment, the episodes of hypoglycemia were reduced by 50%(15.1 vs. 7.6 events/month), especially nocturnal hypoglycemia by 67%(6.7 vs. 2.5 events/month). HbA1c was reduced from 9.3% to 8.7% after Lantus treatment. Self-monitored blood glucose of Lantus treatment group at postbreakfast 30, 60, 90 and 120 minutes were 171.1, 169.5, 171.0 and 154.1 mg/dL respectively and lower than those of premixed insulin treatment group (259.7, 282.7, 280.0 and 250.9 mg/dL respectively). Conclusion : Compared with premixed insulin, Lantus plus Humalog is more effective in glycemic control and reduction in nocturnal hypoglycemia in children and adolescents with type 1 diabetes mellitus.

• Korean Journal of Environmental Agriculture
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• v.27 no.2
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• pp.156-162
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• 2008

Salmonellosis is a major bacterial zoonosis that causes a variety of disease syndromes, self-limited enteritis to fatal infection in animals and food-borne infection and typhoid fever in humans. Recently, the emergence of multidrug resistant strains of Salmonella spp. causes more serious problems in environment and public health. The present study was investigated the antibacterial effect of Houttuynia cordata ethanol extract(HCEE) for murine salmonellosis. In the cytotoxic effect of HCEE on RAW 264.7 cells, there was no detectable effect with any concentrations between 25 and 100 ${\mu}g/ml$ after 8 h incubation. The bacteriocidal effect of HCEE was not showed on a Salmonella enterica serovar Typhimurium(S. typhimurium). HCEE makes morphological change of the RAW 264.7 cells, and there was significant decreased bacterial uptake and intracellular replication within Salmonella infected cells. And further nitric oxide(NO) production of Salmonella infected RAW 264.7 cells with HCEE was decreased comparing to RAW 264.7 cells without HCEE until 8 h post infection. Oral administration of HCEE showed a therapeutic effect for S. typhimurium infected BALB/c mice. The mortality of HCEE treated mouse was 80% until 12 days, while that of HCEE untreated mouse was 100 % until 8 days after lethal dose of S. typhimurium infection. These data suggested that HCEE has a potency treatment for intracellular replicative pathogen including salmonellosis, brucellosis, tuberculosis, listeriosis etc., and the application of HCEE makes new strategies for safety medicine development without antibiotic resistance bacterial appearance and residue problem in food and solves the public health problem from antibiotic mis- and over use.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.33-39
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• 2000

Purpose: Our study was designed to investigate the association of MHC Class II (DR, DQ) allele with IgA nephropathy and its significance as a prognostic factor for progression to ESRD Material and Methods: 69 children with IgA nephropathy with normal renal function(serum creatinine $\leq$ 1.5mg/dL) was classified as group A and 70 patients who received renal transplantation due to IgA nephropathy were selected as group B. The HLA-DQB1 and HLA-DRB1 alleles were studied by polymerase chain reaction using sequence specific primers. We have compared the difference in alleles between these two groups and with normal control and also examined any possible effect of the MHC class II genes on the histopathological severity and prognosis of IgAN. Results: Mean age was $8.8{\pm}2.9$ years in group A and $35.0{\pm}15.5$ years in group B. Male to female ratio was 2.8:1 in group A and 2.5:1 in group B. There was a significantly higher frequency of HLA-$DQB1^*03\;and\;DQB1^*05$ in Group B. The frequency of HLA-$DQB1^*0302\;and\;^*05031$ allele had increasing tendency in Group B(P<0.05). HLA-$DRB1^*03\;and\;^*05$ were more common in Group B(P<0.05). HLA-$DRB1^*04$ allele was the most common DR alleles in both group, but there was no statistical significance. There were no significant correlation with MHC class 13 genes on the hjstopathological severity in Group A. Conclusion: In conclusion, $HLA-DQB1^*0302\;and\;HLA-DQB1^*05031 $ allele seemed to be more common in transplanted patients compared to group with normal renal function suggesting that this allele is associated with poor prognosis in IgAN. However larger studies and follow up are required to confirm this due to uncharacterized heterogeneity in etiopathogenesis of IgA nephropathy and possibly one or more than one gene may exert influence in determining susceptibility to the diseases.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.68-74
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• 2005

Purpose : There is growing use of continuous renal replacement therapy(CRRT) for pediatric patients, but reports about the use and outcome of CRRT in children is rare in Korea. We report our experiences of CRRT in critically ill pediatric patients. Methods : We reviewed the medical records of 23 pediatric patients who underwent CRRT at Asan Medical Center between May 2001 and May 2004. We evaluated underlying diseases, clinical features, treatment courses, CRRT modalities and outcomes. Results : Ages ranged from three days to 16 years with a median of five years. Patients weighed 2.4 to 63.9 kg(median 23.0 kg; 10 patients ${\leq}20kg$). The underlying diseases were malignancy(nine cases), multiple organ dysfunction syndrome(five cases), hyperammonemia(four cases), acute renal failure associated with liver failure(three cases), dilated cardiomyopathy(one case) and congenital nephrotic syndrome(one case). Pediatric Risk of Mortality(PRISM) III score was $17.6{\pm}7.6$ and the mean number of failing organs was $3.0{\pm}1.7$. Duration of CRRT was one to 27 days(median : nine days). Eleven patients(47.8%) survived. Chronic renal failure developed in two cases, intracranial hemorrhage in one case, and chylothorax in one case among the survivors. PRISM III score and the number of vasopressor before the start of CRRT was significantly lower in the survivors($12.7{\pm}4.2$ and $0.9{\pm}1.1$) compared with nonsurvivors($22.1{\pm}7.8$ and $2.4{\pm}1.4$)(P<0.05). Conclusion : CRRT driven in venovenous mode is an effective and safe method of renal support for critically-ill infants and children to control fluid balance and metabolic derangement. Survival is affected by PRISM III score and the number of vasopressors at the initiation of CRRT.

• Journal of Life Science
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• v.19 no.9
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• pp.1226-1231
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• 2009

Enzyme substitution with recombinant phenylalanine ammonia-lyase (PAL, EC 4.3.1.5) is currently being explored for treatment of phenylketonuria (PKU), an autosomal recessive genetic disorder with mutations of the gene encoding phenylalanine-4-hydroxylase (EC 1.14.16.1). However, oral administration of PAL is limited because of proteolytic digestion in the gastrointestinal tract. The aim of this study was to determine the biochemical properties of PAL and delinate the susceptibility of wild-type PAL to pancreatic proteolysis by exploring several mutants, and to develop therapeutic drugs with PAL for PKU. The specific activity of PAL was assayed and its optimal pH, temperature stability, and intestinal protease susceptibility were investigated. Its $V_{max}$ values for phenylalanine and tyrosine were 1.77 and $0.47{\mu}mol$/ min/mg protein, respectively, and its $K_m$ values were $4.77{\times}10^{-4}$ and $4.37{\times}10^{-4}\;M$, respectively. PAL showed an optimal pH at 8.5, corresponding to the average pH range of the small intestine. It showed no loss of activity at $-80^{\circ}C$ for 5 months and possessed 93.4% of its activity under $4^{\circ}C$ for 4 wks. PAL was susceptible to chymotrypsin digestion and, to a lesser extent, to trypsin, elastase, carboxypeptidase A, and B. The trypsin and chymotrypsin cleaving sites were mutated to investigate protection from pancreatic digestion and the specific activities of these mutants were evaluated. The six mutants displayed low specific activities compared to the wild-type, suggesting that the primary trypsin and chymotrypsin cleaving sites may be essential for catalytic reaction. The PAL mutants could therefore be applied as a pretreatment modality without susceptibility to proteolytic attack, however, additional modification for enhancing enzymatic activity is needed to reduce the Phe levels effectively.

• Journal of Nutrition and Health
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• v.40 no.4
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• pp.334-346
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• 2007

The present study examined the reproductive factors and food intake pattern which influence on the breast cancer risk in Daegu${\cdot}$Gyungbuk area. The case subjects were 103 patients newly diagnosed as breast cancer at Kyungpook National University Hospital. The control subjects were 159 healthy women selected by frequency matching of age and menopausal status in the same community. The survey was conducted by individual interviews using questionnaires which include general and reproductive characteristics, dietary habits, and food frequency. The odds ratios were calculated by using unconditional logistic regression after adjusting for confounding variables. The mean age of the subjects was 50 yrs, and mean body mass index was significantly high in the patient group than in the control. The patient group had a significantly higher familial history of breast cancer and a significantly shorter breast-feeding period compared to the control group. It has been found no significant relationship between extrinsic hormone use such as oral contraceptives or estrogen replacement therapy and breast cancer risk. In regard of food habits, a high preference for the cooking method of steaming rather than frying, panbroiling or roasting was significantly associated with lower relative risk of breast cancer. The higher intake frequencies of fruits for all subjects, and seaweeds only for postmenopausal subjects were related with a significantly lower relative risk of breast cancer. The results of the study suggest that the possible risk factors for the breast cancer occurrence include high BMI, family history, less breast feeding experience, preference for the cooking method of frying, and less consumption of fruits and seaweeds. This study provides an useful data for nutrition education to prevent breast cancer for the residents in Daegu${\cdot}$Gyungbuk area.