• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.674-679
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• 2009

Purpose : The authors aimed to analyze the long-term effects of treatments, especially external beam radiotherapy (EBRT), in bilateral retinoblastoma patients. Methods : This retrospective study analyzed the medical records of 22 bilateral retinoblastoma patients who were registered between October, 1987 and October, 1998 and followed-up for more than 10 years. They were treated by enucleation, EBRT, and systemic chemotherapy. Age at diagnosis, sex, delay prior to treatment, Reese-Ellsworth (RE) classification, and the local treatment modalities were analyzed in relation to recurrence-free survival (RFS) and complications. Results : Median age at diagnosis was 7.0 months (range 1.7-31.6 months). Leukocoria was the most common presenting feature. Two patients had a familial history. The RE classifications of the 44 eyes were group II in 4, III in 14, IV in 4, and V in 22. At the end of a median follow-up period of 141 months (range 55-218 months), 20 patients were alive. The 10-year ocular survival rate of the 44 eyes was $56.8{\pm}7.5%$. The 10-year RFS and ocular survival rate of the 29 eyes treated by combined EBRT and chemotherapy were 75.9% and 86.2%, respectively. Treatment delay (>3 months) was found to be related to higher risk of recurrence. Complications after EBRT were cataract, retinal detachment, phthisis bulbi, and facial asymmetry. No patient developed a second malignancy during the follow-up period. Conclusion : Early detection and prompt treatment can increase ocular survival rates. In addition, careful attention should be paid to possible long-term sequelae in these patients.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.661-666
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• 2009

Purpose : To determine the prevalence and clinical features of codetected respiratory etiological agents for acute respiratory infection in hospitalized children. Methods : Nasopharyngeal aspirates were obtained from hospitalized children with acute respiratory infection at Dankook University Hospital from September 2003 through June 2005. Immunofluorescent staining and culture were used for the detection of respiratory viruses (influenza virus [IFV] types A, B; parainfluenza virus [PIV] types 1, 2, 3; respiratory syncytial virus [RSV]; adenovirus [AdV]). Polymerase chain reaction (PCR) assays were used for Mycoplasma pneumoniae (MP) and Chlamydia trachomatis (CT) detection, and PCR and culture were performed for enterovirus detection. Acid-fast staining and culture were performed for tuberculosis detection. The demographic and clinical characteristics were reviewed retrospectively from the patients medical records. Results : Evidence of two or more microbes was found in 28 children: RSV was detected in 14, PIV 3 in 10, AdV in 10, MP in 8, PIV 2 in 8, CT in 4, and PIV 1 in 3. Codetected agents were found as follows: RSV＋PIV 2, 6 patients; AdV＋MP, 4 patients; AdV＋PIV, 3 patients; RSV＋MP, 3 patients; PIV 1＋PIV 3, 3 patients. Distinct peaks of codetected agents were found in epidemics of MP and each respiratory virus. Conclusion : The codetected infectious agents were RSV, PIV, AdV, and MP, with distinct peaks found in epidemics of MP and each respiratory virus. Although advances in diagnostic methods have increased the prevalence of codetection, its clinical significance should be interpreted cautiously.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.655-660
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• 2009

Purpose : To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. Methods : Live-born infants with SUA (n=59) detected by physical examination from among 15,193 live births in seven university hospitals in Korea between January 1, 2004, to August 1, 2007, were reviewed retrospectively, with 236 normal infants serving as the control group. Results : A statistical difference was observed between the groups in birth weight and in vitro fertilization. The incidence of infants with SUA was 0.37%. Congenital malformations were observed in 21 infants with cardiovascular (n=15, 25.4%), gastrointestinal (n=2, 3.4%), genitourinary (n=9, 15.3%), neuromusculoskeletal (n=6, 10.2%), central nervous system (n=1, 1.7%), chromosomal (n=1, 1.7%), and other (n=3, 5.1%) abnormalities. There were 49 (83.1%) infants with isolated SUA in this study population; among them, the associated congenital malformations were cardiovascular (n=6, 12.2%) and genitourinary (n=6, 12.2%) abnormalities. Two infants with cyanotic heart disease were operated and four infants with acyanotic heart disease showed improvements without any treatment. Six infants with genitourinary abnormalities on renal ultrasound had mild hydronephrosis without further consequences. Conclusion : The incidence of structural abnormalities in the cardiovascular and genitourinary systems is high and the genitourinary anomalies associated with isolated SUA have relatively good prognosis.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.667-673
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• 2009

Purpose : Toll-like receptor 2 (TLR2) is critical in the immune response to mycobacterial infections. The purpose of this study was to analyze TLR2 surface expressions and TLR2-mediated tumor necrosis factor-alpha ($TNF-{\alpha}$) and interleukin-6 (IL-6) production in patients with BCG vaccine-associated suppurative lymphadenitis. Methods : Peripheral monocytes were separated from 16 patients with BCG vaccine-associated suppurative lymphadenitis and 10 healthy controls using a magnet cell isolation kit. Monocytes ($1{\times}10^6$ cells/well) were incubated with a constant amount of $Pam_3CSK_4$ ($100{\mu}g/mL$) for 24 hours. TLR2 surface expression on monocytes was analyzed by FACS analysis and TLR-2 mRNA expression was determined by RT-PCR. TLR2-mediated $TNF-{\alpha}$ and IL-6 production were measured by ELISA. Results : In patients with BCG vaccine-associated suppurative lymphadenitis, low TLR2 expression on monocytes ($3.39{\pm}$1.2% versus $4.64{\pm}2.6%$) together with significantly lower TLR2 mRNA expression than in the healthy controls was seen after $Pam_3CSK_4$ stimulation. TLR2-mediated $TNF-{\alpha}$ and IL-6 production in patients with BCG vaccine-associated suppurative lymphadenitis ($TNF-{\alpha}$, $775.5{\pm}60.8pg/mL$; IL-6, $4,645.8{\pm}583.9pg/mL$) were also lesser than that in healthy controls ($TNF-{\alpha}$, $1,098.5{\pm}94.3pg/mL$; IL-6, $6,696.3{\pm}544.3pg/mL$). Conclusion : These findings suggest that low TLR2 expression on monocytes might be associated with increased susceptibility to BCG vaccine-associated suppurative lymphadenitis.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1188-1193
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• 2007

Purpose : The prevalence of rickets in the world is on the rise not only in developing but also in developed countries. In Korea, breastfeeding has increased. There have been few studies on the possible association of rickets with breastfeeding. The purpose of this study was to identify the development and the clinical presentation of subclinical rickets in breastfed infants. Methods : We investigated patients who were breastfed and had hypovitaminosis D in the blood from May 2006 to April 2007, and who were diagnosed with vitamin D deficient rickets from May 2003 to April 2006. We evaluated the results of blood tests, x-rays and other relevant information in the medical record. A questionnaire that included questions on the diet of patients, the mothers activity during pregnancy and place of residence was administered. Results : Twelve patients (66%: male, 34%: female) were enrolled in this study. There were eight in the asymptomatic and four in the symptomatic group. The median age for each group was 8 months (range 4-11 month) and 5.5 months (5-8 month). All patients in the symptomatic group were breastfed until diagnosed. In the asymptomatic group, they were breastfed for four to six months, and then weaned with only thin rice soup and vegetables. Nine patients had a vitamin D concentration below 20 ng/mL and three patients had levels between 20 and 29 ng/mL. Alkaline phosphatase (ALP) and parathyroid hormone (PTH) were elevated in both groups. There were statistically significant $25-OHD_3$ levels in the blood in both groups (P=0.008). Ten of the patients (83%) also had iron deficient anemia. Conclusion : Vitamin D deficiency and subclinical rickets has been identified in Korea. However, the prevalence of this disease has not been determined. The main limitation of this study was the small number of patients and the absence of a control group.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1077-1084
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• 2008

Purpose : Despite the seriousness of bacterial meningitis in children, there is little information on the incidence, causative organisms, mortality rate and age distribution. We studied the frequency by age group and causal pathogens, and clinical characteristics in children with bacterial meningitis in the private sector in Korea. Methods : The medical records containing the data on bacterial meningitis patients under 18 years of age confirmed by cerebrospinal fluid (CSF) findings were retrospectively analyzed from September, 1993 to August, 2006 at Ewha Womans University Mokdong Hospital. Results : Eighty-one cases of bacterial meningitis were observed. Overall the most common organism was Streptococcus agalactiae (group B streptococcus, GBS) (30 cases, 37.0%) followed by Haemophilus influenzae (22 cases, 27.2%), Streptococcus pneumoniae (12 cases, 14.8%), Escherichia coli (3 cases, 3.7%), Neisseria meningitidis (1 case, 1.2%) and others (13 cases, 16.0%). In neonates and young infants under 2 months, the most common organism was GBS. In children between 3 months, and 5 years, the most common organism was H. influenzae. S. pneumoniae was the most common organism in children over 5 years of age. Thirty-one patients (38.3%) had complications. Of all ages, the mortality rate of bacterial meningitis markedly decreased compared with the previously reported rate. Conclusion : In neonates, GBS meningitis was most common. The frequency of H. influenzae meningitis decreased after the introduction of H. influenzae type b vaccination. A strategy for the prevention of GBS meningitis in neonates should be established. The influence of the pneumococcal conjugate vaccine on S. pneumoniae meningitis should be studied.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1200-1205
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• 2007

Purpose : We performed this study to investigate the outcome of surfactant replacement therapy (SRT) in above nearterm neonates who were required mechanical ventilatory care due to meconium aspiration pneumonia (MAP), respiratory distress syndrome (RDS) or other severe pneumonia (PN). Methods : 48 patients, gestational period ${\geq}36weeks$, who were admitted in NICU of Dongsan Medical Center, Keimyung University between July 1999 and June 2004 were enrolled. They were divided into three groups, MAP group (15 cases), RDS group (27 cases) and PN group (6 cases). All patients were received SRT and evaluated several clinical data (gestational age, oxygen index, duration of ventilator care) and outcome (complications and mortality rate) between pre-SRT and post-SRT. The mean dose of surfactant (modified bovine surfactant, Newfacten, Yuhan Co., Seoul, Korea) was 120 mg/kg. Results : Among each groups, mean pre-SRT OI was higher in MAP group ($21{\pm}3.2$) than other groups, mean duration (days) of ventilatory care and oxygen therapy were similar distributions. Compared with pre-SRT values, significant improvements (P<0.05) in mean values for FiO2 and oxygenation index were documented at 12 hours after SRT. Early complications (persistent pulmonary hypertension of newborm, pneumothorax) and survival rate were lower in MAP group. Within RDS group, earlier SRT (given before 12 hours of life) revealed significantly lower early complication rate than later SRT (given after 12 hours of life) (13.3% vs 58.3%, P<0.05) Conclusion : Our study suggested that SRT seems to be an effective therapy in above nearterm neonates with severe pulmonary disease, and earlier SRT tends to reduce complications in RDS group than later therapy.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1096-1101
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• 2008

Purpose : Patients with chronic granulomatous disease (CGD) have genetic mutations in a component of the NADPH oxidase enzyme that is necessary for the generation of the superoxide anion. The profound defect in innate immunity is reflected by the patients susceptibility to catalase-positive bacteria and fungi. In addition, CGD patients display signs of persistent inflammation, which is not associated only with deficient superoxide anion production. The aim of this study was to elucidate the cytokine responses in CGD patients after $TNF-{\alpha}$ stimulation. Methods : Heparinized blood samples were collected from 8 CGD patients and 10 healthy volunteers. Monocytes ($1{\times}10^6cell/well$) isolated by the magnet cell isolation system were incubated with a constant amount of $TNF-{\alpha}$ (10 ng/mL) at $37^{\circ}C$ for 6 h. Incubated cells were harvested at 60-min intervals for IL-8 and IL-10 mRNA analysis, and the supernatant was collected at the same intervals to determine IL-8 and IL-10 expression. Monocytes from healthy volunteers were also incubated with antioxidants followed by $TNF-{\alpha}$ stimulation for IL-8 and IL-10 expression. Results : In CGD patients, a high expression of IL-8 together with a significantly higher IL-10 expression than in the healthy controls was seen after $TNF-{\alpha}$ stimulation. Moreover, normal monocytes treated with antioxidants exhibited increased IL-8 responses. Conclusion : The absence of phagocyte-derived reactive oxidants in CGD might be associated with a dysregulated production of pro- and antiinflammatory cytokines. Additional research related to reactive oxidants is needed to clarify the role of cytokines in CGD patients.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.622-628
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• 2008

Purpose : The purpose of this study was to evaluate the immunogenicity of the booster immunization with pneumococcal conjugate vaccine in Korean children. Methods : Thirty-nine children aged 12-23 months who visited Kangnam CHA Hospital between September 2006 and December 2006 were enrolled. The children were divided into primary and booster groups depending on their vaccination status for the 7-valent pneumococcal conjugate vaccine. The anti-pneumococcal antibody levels of each serotype included in the vaccine (4, 6B, 9V, 14, 18C, 19F, 23F) were determined by third-generation ELISA. Results : The geometric mean titer (GMT) of antibodies to each pneumococcal serotype in the booster group was higher than in the primary group (P<.05). The percentage of subjects with pneumococcal antibodies ${\geq}0.35{\mu}g/mL$ was 90.5-100% for all serotypes in both the primary and booster groups. The percentage of subjects with pneumococcal antibodies ${\geq}1.0g/mL$ in the booster group was 94.4-100%, which was higher than the primary group except for serotypes 6B and 14 (P<.05). The percentage of subjects with pneumococcal antibodies ${\geq}5.0{\mu}g/mL$ in the booster group was 50.0-94.4% which was higher than the primary group for all serotypes (P<0.05). Conclusion : The immunogenicity of a booster dose of the pneumococcal conjugate vaccine in Korean children was high and the immunogenicity of a primary series was also relatively high. To determine the feasibility of the introduction of the pneumococcal conjugate vaccine and the appropriate schedule for Korean children, further prospective investigation of the immunogenicity of the booster immunization is needed.

• Biomedical Science Letters
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• v.8 no.3
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• pp.115-125
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• 2002

Gram-negative septicemia, which continues to be a serious clinical problem, is one of the major causes of morbidity and mortality in hospitalized patients. Endotoxin of gram-negative bacteria is a pivotal pathogen of sepsis. To understand the effect of endotoxin on hematological aspect and the time course in early childhood, this study was designed with experimental septic model of young rabbits (8 week-old). Rabbits were divided into control (n=7) and endotoxin group (0.50 mg/kg of endotoxin). The endotoxin group was subdivided into six groups by the sampling times: 3, 6, 12, 24, 48 and 72 hr-group (E-G$_{3}$, E-G$_{6}$, E-G$_{12}$, E-G$_{24}$, E-G$_{48}$ and E-G$_{72hrs}$, each n=7). The evaluation of CBC, activated partial thromboplastin time (APTT), prothrombin time (PT), fibrinogen concentration, coagulation factors and D-dimer were taken from the bloods. The number of leukocytes was lower in E-G$_{3}$ and E-G$_{6hrs}$ (due to pantocytopenia), whereas it was higher in E-G$_{24}$ and E-G$_{48}$ (due to neutrophilia and/or lymphophilia) than in control group (P<0.05). Platelet counts in E-G$_{3}$, E-G$_{6}$, E-G$_{12}$, E-G$_{24}$ and E-G$_{48hrs}$ were lower than those of control group (P<0.05). Normoblast counts in E-G$_{3}$, E-G$_{6}$, E-G$_{12}$, E-G$_{24}$ and E-G$_{48hrs}$ were higher than those of control group (p<0.01). APTT in E-G$_{3}$, E-G$_{6}$, E-G$_{12}$, E-G$_{24}$ and E-G$_{72hrs}$ were longer while PT in E-G$_{3}$, E-G$_{6}$, E-G$_{48}$ and E-G$_{72hrs}$ were higher than those of control group (p<0.05). Fibrinogen concentrations were lower in E-G$_{3}$, E-G$_{6}$ and E-G$_{12}$ but higher in E-G$_{48}$ and E-G$_{72hrs}$ than those of control (p<0.05). Intrinsic coagulation factors (XII, XI, IX, VIII) in all endotoxin groups were significantly lower than those of control group (p<0.05). Extrinsic coagulation factor (X, VII, V, II) were lower in E-G$_{3}$, E-G$_{6}$, E-G$_{12}$ and E-G$_{24hrs}$ whereas they were higher in E-G$_{48}$ and E-G$_{72hrs}$ than in control group (p<0.05). D-dimer concentrations in E-G$_{48}$ and E-G$_{72hrs}$ were higher than those of control group (P<0.001). We concluded that endotoxin led to extensive hematological disturbances including disseminated intravascular coagulation in the young rabbits and that this pathologic condition in the infant and childhood groups will cause the grave results.