• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.63-67
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• 2005

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous palpable purpura, gastrointestinal(GI) symptoms, arthritis and renal involvement. In general, the prognosis is determined by GI complication as well as the severity of nephritis. In this study, we analyzed the statistical relationship between the GI symptom and other clinical findings for assessing the prognosis, and evaluated abdominal ultrasonographic findings for early diagnosis of this disease with atypical clinical presentation and early detection of serious GI complications. Methods : One hundred seventy seven patients with HSP in the Department of Pediatrics, Wonkwang University Hospital from January 1994 to June 2004, were enrolled. We retrospectively analyzed charts about clinical and abdominal ultrasonographic findings, and classified our patients into two groups(GI-Sx(-), GI-Sx(+)) for statistical analysis. Results : The ratio of female to male is 1.5 : 1. The peak age incidence was five to eight years in 95 cases(53%). The GI symptoms appeared in 117 cases(66%), which include abdominal pain 115 (98 %), tenderness 45(38%), nausea and vomiting 35(30%), bloody stool 10(8.5%), diarrhea four(3.4%), rebound tenderness four(3.4%), and also intussusception and appendicitis were complicated in five and two cases respectively. GI-Sx(+) group had an increased risk of renal involvement and relapse than the GI-Sx(-) group. But there were no relationships about sex and age incidence, or other clinical and laboratory findings between two groups. Ultrasonographic findings in 98 patients with GI symptoms included small bowel thickening in 70 cases(71%) in which duodenum, jejunum and ileum were involved in 71%, 45.7%, 40% respectively, small bowel dilatation in 41 cases(42%), lymph node swelling in 46 cases(47%), and ascites in 25 cases(25.5%). Conclusion : GI symptoms in patients with HSP suggested increased risk of renal involvement and relapse. Abdominal ultrasonography could be helpful in the early diagnosis on atypical clinical presentation and early detection of serious GI complication in these patients.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1247-1251
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• 2007

Purpose : Pathologically, Kawasaki disease (KD) is associated with widespread vascular endothelial damage in the acute phase. The vasculitis induced endothelial injury leads to coagulation abnormalities. Abnormalities of endothelial function, platelet activation, and fibrinolysis are present during acute phase and long after the onset of KD. The aim of study is to evaluate the change of hemostatic markers in the clinical stages of KD and to assess the hemostatic markers to be a useful indicator of the development of coronary artery lesion (CAL). Methods : Seventy four KD patients diagnosed in Chungnam National University Hospital from November 2004 to June 2007. Eleven febrile control and eleven healthy children were selected for healthy control. All blood samples were collected before and after Intravenous gammaglobulin (IVGG), $2^{nd}$ week, and $4^{th}-8^{th}$ week of illness of KD. Results : Initial D-dimer level of Kawasaki disease showed meaningful difference compared to control group (P<0.05). D-dimer and fibrinogen degradation products (FDP) before IVGG increased compared with normal control group and decreased after IVGG administration. It is normalized until 2 weeks later, and continue to decreasing. D-dimer and FDP were significantly different according to the CAL before IVGG. Conclusion : The hemostatic markers may change to the clinical stage of KD, which may suggest the degree of endothelial injury. Increased some hemostatic markers may be the predictors for development of CAL.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.500-505
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• 2005

Purpose : This study was designed to estimate the prevalence of Mycoplasma pneumoniae infection during an epidemic period by means of examining the antibody conversion rate and to investigate the association of the antibody conversion with age, initial antibody titer, and atopy. Methods : We chose 191 children whose antibody titer to M. pneumoniae was negative, 1 : 40, or 1 : 80 during the first half of 2003. After the second half of 2003 when the M. pneumoniae epidemic occurred, follow-up collection of sera was performed during the first half of 2004. M. pneumoniae antibody titer was measured by Serodia-Myco II particle agglutination test. Results : Of 191 children, antibody conversion was detected in 83 children(43.5 percent). No significant difference was found between the conversion and non-conversion group with respect to age, sex and atopy. Dividing the subjects into four groups by age, results on the antibody conversion rate revealed no significant differences between the groups. Assessed by initial antibody titer, a diminished trend of conversion rate was observed in children with 1 : 80 titer but the difference was not significant. There was no significant difference in the antibody conversion rate between atopic and non-atopic children. Conclusion : Based on the antibody conversion rate in this study, the prevalence of M. pneumoniae infection during an epidemic period was estimated to be 43.5 percent. This high infection rate suggests that during an epidemic, we should bear in mind M. pneumoniae as an important etiologic agent for respiratory infection in children.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.512-517
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• 2005

Purpose : In adults, endoscopic tracheobronchial balloon dilatation and stenting have become valuable methods to establish and maintain an adequate airway lumen when tracheomalacia or neoplastic growth compromise the airways. But in children, only a few cases were reported due to technical problems. We report six children who were treated with stent implantation and describe the use and safety of airway stents. Methods : Six patients with severe airway obstruction were treated. We investigated the underlying medical problems, stenotic site, symptomatic improvement and complications, and the size and location of stent. Results : The median age of the six patients was 21 months. Three of them were mechanically ventilated and one had an endotracheal tube to maintain the patency of airway. Diagnoses were : congenital tracheal stenosis with or without bronchomalacia, granulation tissue formation after right upper lobectomy by bronchial carcinoid or after prolonged intubation, endobronchial tuberculosis, and airway compression by mediastinal undifferentiated sarcoma. Nitinol stents were implanted in the airway guided by bronchoscopy and fluoroscopy simultaneously. Three cases were placed in trachea, the others were in the bronchus. After stent implantation, all patients showed marked improvements of their airway obstructive symptoms. Four patients are doing well, although two expired due to underlying diseases. Four patients had granulation tissue formation around stents, but that was tolerable after removing the stent. Conclusion : We suggest that the use of expandible metallic stent implantation can offer safe therapeutic option even in extremely severe, life threatening and inoperable airway stenosis in children.

• 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.