• Clinical and Experimental Pediatrics
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• v.45 no.6
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• pp.783-789
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• 2002

Purpose : We evaluated the epidemiologic characteristics and incidence of coronary artery sequele of children with KD according to treatment. Methods : We retrospectively analyzed 506 medical records of children with KD, who were admitted at Daejeon St. Mary's Hospital from Jan. 1987 to Dec. 2000. Results : The mean annual incidence was $36.1{\pm}11.1$ cases per year. There was a slightly higher occurrence in summer with no significant difference in monthly incidence. The mean age was $2.4{\pm}1.7$ years and 450 children(88.9%) were below four years of age. The male to female ratio was 1.7 : 1. When the 345 cases between 1987 and 1994 were divided into three groups according to treatment, incidences of the coronary abnormality(above grade II) of aspirin-treated(54 cases; 15.6%), divided-intravenous immunoglobulin(IVIG) treated($400-500mg/day{\times}4-5days$, 224 cases; 64.9%), and one-dose IVIG treated(2.0 g/day, 67 cases; 19.5%) groups were 8.3%, 6.0%, and 7.5%, respectively. Between 1995 and 2000, 143 cases were treated with only one-dose IVIG and 21 cases(14.7%) showed coronary artery abnormalities(grade I, 15 cases; grade II, two cases; and grade III, four cases). Among the 143 cases, 22 cases(15.1%) were retreated with IVIG and/or steroid pulse therapy. The incidence of coronary artery abnormality in this group was 50.0%. Incidences of cases in recurrence and among siblings were 0.6% and 0.4% respectively. There was no fatal case. Conclusion : In Daejeon, Korea, the epidemiologic feature of KD showed slight annual variations without monthly differences. The incidence of coronary abnormality with one-dose IVIG therapy was 14.7%. The nonresponse of this therapy was 15.1% with a coronary abnormality of 50.0%.

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.984-990
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• 2009

Purpose : Being small for gestational age (SGA) is a risk factor of short stature in children. Genetic background such as mid-parental height (MPH) is known to influence growth of children born SGA. We studied the relationship between growth of children born SGA and MPH and studied the effects of insulin-like growth factor (IGF-I) and insulin-like growth factor binding protein 3 (IGFBP-3) on postnatal growth in children born SGA according to MPH. Methods : Forty-nine neonates born SGA were included in this study. We defined corrected height standard deviation score (cHtSDS) by modified height SDS (HtSDS) based on their MPH. We categorized subjects into group 1 consisting of children with cHtSDS ${\geq}0$ (n=35) and group 2 consisting of children with cHtSDS <0 (n=14), and compared IGF-I and IGFBP-3 between the two groups. Results : The HtSDSs and cHtSDSs in groups 1 and 2 were $0.06{\pm}1.05$ vs. $-0.95{\pm}0.85$ (P=0.000) and $0.78{\pm}0.93$ vs. $-0.46{\pm}0.67$ (P=0.000), respectively. IGF-I SDS was higher in group 1 than in group 2 ($2.82{\pm}3.69$ vs. $0.23{\pm}2.42$, P=0.012). Total cHtSDS ($0.42{\pm}1.03$) was significantly higher than HtSDS ($-0.22{\pm}1.10$) (P=0.000). Conclusion : Our results show that cHtSDS differs significantly from HtSDS. Growth assessment by standardized growth curve does not uniformly show effects of genetic factors. A more accurate assessment of growth uses a personalized corrected growth curve that considers the genetic factor measured by MPH.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1068-1075
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• 2005

Purpose : The role of ghrelin, which promotes the secretion of growth hormone, was not well known until now. Recently it was found that the mutation of ghrelin gene is related to obesity and diabetes. This study is to find the screening method that can easily and effectively detect the polymorphism of Leu72Met in ghrelin gene of obesity patients and apply it to clinical usage. Methods : We compared PCR-RFLP, PCR-SSCP and ARMS methodologies for analyzing of the polymorphism of Leu72Met in ghrelin gene of obesity children, and also studied the merits and demerits of these methodologies. Results : In this study, we were able to find out the band of peculiar allele of Leu72Met in ghrelin gene using PCR-RFLP, PCR-SSCP and ARMS analyses. The polymorphism of Leu72Met in ghrelin gene determined by all above methodologies was in complete agreement. Compared to the PCR-RFLP and PCR-SSCP, ARMS analysis is simple, inexpensive and also consume less time. It is very sensitive to analyze the polymorphism and easy to understand the results of test. Conclusion : Though PCR-RFLP, PCR-SSCP and ARMS analyses were sensitive to analyze the polymorphism of Leu72Met in ghrelin gene, ARMS analysis appears to be more efficient than PCR-RFLP and PCR-SSCP. Therefore, we conclude that ARMS analysis is suitable to analyze the polymorphism of Leu72Met in ghrelin gene for large quantity of specimens.

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.24-28
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• 2006

Purpose : The purpose of this study was to investigate the health status and nutritional condition of children living in a low-income community through anthropometric, laboratory evaluation. Methods : A community-based survey identified children below 15 years living in a low-income community. Their weight, height, visual acuity, hearing level and dental status were measured. Blood sample were obtained on June and July, 2004. Hemoglobin, serum cholesterol, Hepatitis B antigen/antibody, AST and ALT were measured. Results : A total of 285 students(M : F=141 : 144) aged 6 to 14 years were included in this study. The heights and weights in some grades were smaller than controls. The prevalence of obesity was 10.6 percent in males and 10.7 percent in females. The prevalence of abnormal visual acuity, hearing impairments and dental carries were 20.5 percent, 0.3 percent and 69.4 percent. The prevalence of anemia was 10.1 percent. Serum total cholesterol was over 200 mg/dL in 7 percent. They complained of abdominal pain(22.1 percent) and headache(17.1 percent). Hyperthyroidism, cataract, neurofibromatosis, severe atopic dermatitis, ventricular septal defect, strabismus and inguinal hernia were newly diagnosed. Conclusion : Mean heights and weights of children in the low-income community were smaller than controls. The prevalence of abnormal visual acuity, hearing impairment and dental carries were higher than in the 2003 national health survey. Additional research is needed to evaluate the health status of the low-income community.

• 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.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.431-438
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• 2006

Purpose : Insulin-like growth factor binding protein(IGFBP)-3 has been known as a tumor suppressor gene, and its anti-tumor function was divided into insulin-like growth factor(IGF)-dependent and IGF-independent mechanism. In IGF-independent mechanism, IGFBP-3 directly interacts with a cell without binding of IGFs, becoming an interesting object in oncology. Several studies demonstrate that one of the well-known tumor suppressor genes, p53, induces directly IGFBP-3 transcription, and the increment of IGFBP-3 expression induces apoptosis of many cancer cells. Recently, the anti-tumor mechanisms of IGFBP-3 have been reported, but post-translational modification of IGFBP-3 and its anti-tumor mechanism are not well known. In this study, we examined whether p53 regulated the glycosylation of IGFBP-3, and analysed the meaning of IGFBP-3 glycosylation related to the apoptosis of cancer cell. Methods : The p53-mutated status of MDA-MB-231 human breast cancer cells was used in this experiment. The expression and glycosylation of IGFBP-3 were tested by Western blot analysis after infection of adenovirus mediated Ad/p53 and/or Ad/IGFBP-3. Results : Ad/p53 infected cells resulted in growth retardation and the induced apoptosis. p53 induced direct expression and glycosylation of IGFBP-3. The increase of glcosylated IGFBP-3 was able to promote cellular apoptosis, and the glycosylation of IGFBP-3 was more activated by the double treatment of Ad/p53 and Ad/IGFBP-3. Conclusion : From this study, the anti-tumor activity of IGFBP-3 was shown to improve the stabilization of IGFBP-3 through the increment of glycosylation of IGFBP-3 by p53. This result suggests that the combined gene therapy of p53 and IGFBP-3 may appropriate treatment of cancer.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1253-1259
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• 2003

Backgroud : Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. Methods : A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. Results : The average age at onset of seizures was $6.1{\pm}4.6days$ and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. Conclusion : Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1242-1247
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• 2003

Purpose : Gastroesophageal reflux disease(GERD) is known as one of the most common causes of chronic cough, especially in children. The purpose of this study is to evaluate the efficacy of parameters from proximal esophageal 24-hr pH monitoring through its comparison with those of distal esophageal 24-hr pH monitoring that we generally use for diagnosis of GERD. Methods : We performed chest CT scans to find out the cause of chronic cough in infants with no clinical manifestation suggesting GERD. Then, in case that they had air space consolidation in posterior segment of both upper lobes and superior segment of both lower lobes(dependent position), we performed proximal and distal esophageal 24-hr pH monitoring. Results : The proximal and distal pH monitoring were performed in 17 infants(male 12; female five). The patients with positive pathologic reflux in proximal esophagus were 15 of 17(88.2%) and in distal esophagus were four of 17(23.5%). Reflux index and the total number of reflux episodes were statistically significantly lower in the proximal than in the distal esophagus(P<0.05). There was no correlation between each parameters of proximal and distal esophageal 24-hr pH monitoring. Conclusion : This study suggests that proximal esophageal 24-hr pH monitoring can be used as a very useful diagnostic tool in infants with chronic cough in which there are suspicions that it resulted from aspiration due to GERD.

• 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.51 no.4
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• pp.391-395
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• 2008

Purpose : This study was performed evaluate optimal dose for disimpaction, efficacy and safety of PEG 4000 in children with chronic functional constipation. Methods : Eighty six children with chronic functional constipation were enrolled in this prospective study at Konkuk university hospital March, 2003 through August, 2006. Success in disimpaction with PEG 4000 was defined as meeting at least two out of three criteria; resolution of chief complaint, getting easiness of defecation with respect to frequency of bowel movement and hardness of stool based on defecation diary, and decrease in fecal impaction on simple abdominal X-ray test. Adverse effects of PEG 4000 were monitored clinically and biochemically. Results : Eighty three out of 86 children completed the study, and success rate of disimpaction was 99% (82/83). The mean dose of PEG 4000 for disimpaction was $0.93{\pm}0.28g/kg/day$ (0.4-2.0 g/kg/day, Max.: 30 g/day). Frequency of bowel movement increased ($5.02{\pm}2.71/wk$ vs. $11.25{\pm}5.43/wk$) in most children (79/83). Fecal impaction on simple abdominal X-ray test improved with statistical significance in 25 children (P=0.0007). Because of adverse effect of PEG 4000, 3 children did not complete the study; urticaria, severe diarrhea, diarrhea and abdominal pain. One 6-year-old girl who completed the study complained tingling sensation in the hand and foot without laboratory abnormality (4/86, 4.7%). Laboratory test revealed hyperosmolality without clinical symptom in 1 child, and eosinophilia in 6 children. Conclusion : The average safe and effective dose of PEG 4000 for disimpaction was $0.93{\pm}0.28g/kg/day$ (0.4-2.0 g/kg/day, Max : 30 g/day) in children with chronic functional constipation.