• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.302-310
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.865-870
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• 2003

Purpose : We measured the umbilical cord arterial concentrations of isoprostane($8-iso-PGF_{2{\alpha}}$) and intended to decide whether the umbilical cord arterial concentrations of isoprostane could be used as a useful parameter for lipid peroxidation in newborn infants. Methods : The isoprostane and malondialdehyde(MDA) concentrations of the umbilical cord were measured by enzyme immunoassay and TBARS(thiobarbituric acid reactive substance) assay in 33 preterm and 28 term infants, respectively. The concentrations of isoprostane and MDA were compared between preterm infants and term infants, and were analysed for association with perinatal risk factors and neonatal complications. Results : Umbilical cord arterial concentrations of isoprostane were $704.7{\pm}635.6pg/mL$ and $421.9{\pm}306.5pg/mL$ in preterm and term infants, respectively. Umbilical cord arterial concentrations of MDA were $44.0{\pm}22.9{\mu}M/L$ and $26.2{\pm}10.7{\mu}M/L$ in preterm and term infants, respectively. Umbilical cord arterial concentrations of isoprostane and MDA in preterm infants were significantly higher than those in term infants(P<0.05). The umbilical cord arterial concentrations of isoprostane were significantly associated with perinatal risk factors such as fetal distress, oligohydramnios, and breech delivery in preterm infants and pregnancy-induced hypertension in term infants(P<0.05). Conclusion : Umbilical cord arterial concentrations of isoprostane in preterm infants were higher than those in term infants, and those are significantly associated with some perinatal risk factors.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.351-357
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• 2003

Purpose : To raise awareness of the clinical importance of, and the need for proper management of acute focal bacterial nephritis(AFBN), we analyzed 22 AFBN patients and 22 other upper urinary tract infection patients by use of comparative studies. Methods : From January 2000 to May 2002, 22 AFBN patients aged from 1 month to 12 months were selected. As a control group, 22 UTI patients with no radiologic abnormalities were selected and matched by age and sex. Results : The incidence of AFBN was more common in boys than in girls. Since both groups had similar symptoms, it was difficult to diagnose AFBN by clinical presentations alone. ESR and CRP were significantly higher in AFBN patients. The most common causative organism was E. coli in both groups. On the sonographic findings, the most lesions were seen on the upper lobe of the kidney; more frequently, on left kidney. The lesions showed globular or wedge-shaped increased echogenecity. $^{99m}Tc-DMSA$ scan showed the complete coincidence of the location, size and shape in all cases compared to the findings of renal sonography. Conclusion : The roles of renal sonography and DMSA scan were very important, and ultrasonography was an excellent initial tool in diagnosing AFBN. Since the degree of infection in AFBN is more severe than other urinary tract infections and evollution into a renal abscess is possible, early diagnosis and appropriate antibiotics therapy is essential.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1212-1216
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• 2003

Purpose : Rotavirus is a leading cause of severe gastroenteritis in infants and young children around the world. The aim of this study is to investigate the fat content in stools of patients with rotaviral enteritis compared to the stools of children who had no gastroenteritis. Methods : Seventy two patients who were admitted to Konkuk University Hospital, College of Medicine from Jun 2001 to May 2002 due to rotaviral enteritis and seventy five patients who were admitted at the same time with other diseases with no gastrointestinal problems as control, were enrolled in this study. The age of patients was from one month to five years. The average age of children with rotaviral enteritis was $17{\pm}11months$ and the average age of control patients was $14{\pm}15months$. Fat content of stools was investigated by acid steatocrit tests in both patients with rotaviral enteritis and control. Results : Acid steatocrit value of patients with rotaviral enteritis was higher than that of control patients. There was no difference in acid steatocrit value of children with rotaviral enteritis among the age groups. In one month- to six month-old infants, there was no difference in acid steatocrit values between the children with rotaviral enteritis and control patients. But, over the age of seven months, the acid steatocrit value of children with rotaviral enteritis was higher than that of control patients. Conclusion : We are of the opinion that fat malabsorption in patients with rotaviral enteritis and steatorrhea in rotaviral enteritis may result from decreased fat absorption in the small intestine.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.242-249
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• 2003

Purpose : We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. Methods : A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two groups, mild and severe cardiotoxicity, according to the FS. We followed up survivors with cardiotoxicity, and checked their present cardiac function by physical activity, echocardiography, electrocardiography(EKG) and chest X-ray. Results : Of the 154 children treated with anthracyclines, forty(26.0%) were diagnosed as cardiotoxicity. The incidence of cardiotoxicity increased in exponential fashion with increases in the cumulative dose of anthracyclines. There was minimal increase of incidence until a dose of $300mg/m^2$ after which the incidence increased rapidly. After mean $3.8{\pm}1.8year$ follow-up of 23 survivors with cardiotoxicity, FS increased significantly. EKG and chest X-rays were not helpful for the diagnosis of cardiotoxicity because of their low sensitivity and specificity. Conclusion : Although convenient, non-invasive and inexpensive, EKG and chest X-rays were not helpful for the follow-up of anthracycline cardiotoxicity. Almost all survivors with anthracycline cardiotoxicity have improved in both physical activity and echocardiographic findings after discontinuation of anthracyclines.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.181-186
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• 2009

Purpose : Inflammation plays a potential role in the pathogenesis of bronchopulmonary dysplasia (BPD). Strategies for preventing BPD include respiratory management, antioxidants, nutritional treatment, and others such as anti-inflammatory agents. We aimed to assess the safety, tolerability, and efficacy of montelukast (MK), a cysteinyl leukotriene 1 receptor antagonist, as an add-on therapy in BPD. Methods : In addition to currently available standard measures such as oxygen supplementation, bronchodilators, nutritional support, and/or diuretics, montelukast was administered to 15 preterm infants with BPD. MK was given orally (1 mg/kg/d) for a mean period of 12 weeks. We compared safety and efficacy parameters with historical controls. Results : All 15 patients survived, and no differences were found in the incidence of adverse reactions between the 2 groups. The ventilation index was significantly improved after 2 weeks in MK group compared with historical controls. There were no significant differences in other respiratory parameters (MAP, oxygen dependency, and ventilator dependency) between the groups, but the MK group showed trends of greater improvement. Conclusion : Administration of MK 1 mg/kg/d was well tolerated in preterm BPD patients as an add-on therapy. We demonstrated that after 2 weeks of MK administration of 1 mg/kg/d, MK had beneficial therapeutic effects on BPD patients as an add-on to the standard therapy. Further multicenter randomized controlled clinical trials are needed to confirm the efficacy and safety of MK as a useful supplement to standard therapy for BPD patients.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.409-414
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• 2008

Purpose : To assess the prevalence of hyponatremia in epileptic children receiving carbamazepine or oxcarbazpine, we investigate serum sodium changes according to age, serum carbamazepine level, and daily oxcarbazepine dosage, and the prevalence of symptoms of hyponatremia. Methods : We reviewed the clinical data of the 197 children receiving carbamazepine and/or oxcarbazepine with or without antiepileptic therapy. And these were classified into the carbamazepine treated patients (group I), oxcarbazepine treated patients (group II), and carbamzepine or oxcarbazepine with other antiepileptics treated patients (group III). Potentially predictive values for development of hyponatremia were examined in each group: age, plasma level of carbamazepine and daily dosage of oxcarbazepine. We assessed the symptoms of hyponatremia. Results : The overall prevalence of hyponatremia was 20.8% (group I, II and III : 17.9%, 22.6%, and 21.8%, respectively), and the prevalence in groups II and III compared with controls (P<0.03) was significantly lower. The changes of serum sodium levels relation to age were not significantly different. The changes of serum sodium levels by increasing of serum levels of carbamazepine and dosage of oxcarbazepine were statistically significant (P<0.01). Among the 41 patients who had biochemical hyponatremia, the prevalence of hyponatremic symptoms was 17.1%. Conclusion : Hyponatremia may occur relatively more frequently with oxcarbazepine or combined other antiepileptics than carbamzepine therapy only. Age of children receiving carbamazepine or oxcarbazepine was no predictive value for occurrence of hyponatremia. The patients whose serum level were less than 125 mEq/L showed more severe clinical symptoms than any other study groups.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1109-1118
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• 2009

Purpose : Metabolic syndrome (MS), characterized by obesity and insulin resistance, elicits risk factors such as hyperlipidemia, hypertension, and glucose intolerance with additive effects on atherosclerosis, leading to cardiovascular diseases. The purposes of this study were to evaluate the prevalence of MS among overweight and obese adolescents and to investigate the impact of obesity on the cardiovascular system. Methods : In all, 684 adolescents were included in the study. Blood pressure, body mass index (BMI), fasting blood glucose, total cholesterol, triglyceride, low-density-lipoprotein (LDL)-cholesterol, high-density-lipoprotein (HDL)-cholesterol, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and high-sensitive C-reactive protein (hs-CRP) were measured in the patients with a BMI of >85 percentile. Brachial-ankle pulse wave velocity (BaPWV) and ankle brachial index were measured using Vascular Profiler (VP)-1000. Results : MS was confirmed in 19.5% of the overweight and obese adolescents and 50.8% of the obese adolescents. The systolic and diastolic blood pressure, height, weight, fat mass, %fat, BMI, obesity index, and waist circumference were higher in the overweight and obese adolescents with MS. Moreover, the triglyceride, AST, ALT, and hs-CRP levels were higher, whereas HDL-cholesterol level was significantly lower in this group. The overweight and obese adolescents with MS showed shorter diastolic and systolic times, higher heart rate and BaPWV, and longer E-wave deceleration time by echocardiography. Conclusion : Overweight and obese adolescents showed characteristic MS features such as hypertension and hyperlipidemia. Thus, obese adolescents predisposed to MS should be provided early treatment for obesity.

• Journal of Nutrition and Health
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• v.40 no.8
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• pp.736-744
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• 2007

Leptin, as an adipocyte-derived hormone, is an important regulator of food intake and energy expenditure. In the cross-sectional study, leptin was shown to be positively related to body adiposity and metabolic disorders in adults. However, there were very few studies which reported the leptin as a predictor of weight gain over time. We examined whether serum leptin can be used as an indicator of the present and 1-year past weight status in very young children. First grade students from elementary schools in Gwacheon City were enrolled in the study since 2005. The study subjects(total 375 students; 195 boys and 180 girls) participated in the investigation of both 2005 and 2006. Physical examinations including height, weight, waist circumference were done. To examine the prevalence of obesity, obesity index was used. Serum leptin was measured, and their nutritional status was also evaluated based on 3-Day dietary records. Serum leptin levels were strongly positively related with the value of the present BMI and with the value of the BMI one year before. We found no association with leptin levels and amount of energy intake and macronutrient intake in this children population. Children were divided into three groups according to leptin tertiles. The highest leptin tertile group showed highest prevalence of obesity in year 2006 as well as in year 2005. Serum leptin levels can reflect the weight status now and as well as 1-year before. Possibly serum leptin levels can predict the weight gain of year later. Without an action against the obesity on children with high leptin level, those children would maintain the excess adiposity growth and progress into the obesity-related metabolic disorders. Further studies are needed to predict the obesity as early as possible and preventive system then after.

• Journal of Nutrition and Health
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• v.42 no.1
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• pp.68-77
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• 2009

This study was researched to provide the accurate nutrition information and the menu. We questionnaired an recognition and necessity of the nutrition labeling to 684 customers in fast-food restaurants. After data cleaning, we used spss package 14.0 and analyzed about the nutrition contents and place that display the nutrition labeling. First, we finded out lower recognition of nutrition labeling in restaurants than processed food. Second, many people hoped that calory and fat in various nutritions were displayed each 100 g or 100 mL. Third, the place displaying the nutrition information was the menu board and the counter to identify easily. Fourth, we analyzed the recognition and necessity of the nutrition labeling in fast-food restaurants by t-test and ANOVA. So, we knew that the recognition and necessity of the nutrition labeling was higher woman than man. And the more they earn much money and learned, the more the nutrition labeling are needed. But house-wife recognized the nutrition labeling lower than others.