• Clinical and Experimental Pediatrics
• /
• v.48 no.11
• /
• pp.1212-1218
• /
• 2005

Purpose : Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. Methods : Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. Results : Ten(67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. $O_2$ saturation and pH were lower and cardiothoracic(CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia(P<0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but $O_2$ saturation was lower in functional atresia group(P<0.05). 13 patients(87%) were managed with $PGE_1$. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period(mean 37 months). 3 patients(20%) died and none of patients without pulmonary atresia died. Conclusion : We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.

• Clinical and Experimental Pediatrics
• /
• v.49 no.5
• /
• pp.507-512
• /
• 2006

Purpose : Early detection and intervention of hearing impairment is believed to improve speech and language development and behavior of children. The aim of this preliminary study was to determine the prevalence of hearing impairments, and to identify the association of risk factors relating to refer response in high risk neonates who were screened using distortion product otoacoustic emissions (DPOAE). Methods : The subjects included 871 neonates who were admitted to the neonatal intensive care unit of the Pediatric Department in Soonchunhyang University Bucheon Hospital from May, 2001 to December, 2004. They were screened using DPOAE. Based on DPOAE, we divided the neonates in two groups : 'Pass' and 'Refer'. The differences in risk factors between the pass group and the refer group were analyzed. Results : The incidence of the refer group was 12.1 percent(106 out of 871). The bilateral refer rate was 5.4 percent(47 out of 871). And the unilateral refer rate was 6.7 percent(59 out of 871). Gender, birth place, family history of hearing loss, small/large for gestational age, obstetrical factor, hyperbilirubinemia and use of gentamicin were not statistically related to the refer rate. Statistically related to refer rate were birth weight, resuscitated neonates, Apgar score, craniofacial anomaly, mechanical ventilator application, sepsis, using of vancomycin(P<0.05). The prevalence of hearing impairment (${\geq}60dB$) in this study was 2 percent(18 out of 871). Conclusion : This study showed a higher prevalence of hearing impairment in high-risk neonates. Thus neonatal hearing screening should be carried out in high-risk neonates.

• Clinical and Experimental Pediatrics
• /
• v.49 no.6
• /
• pp.617-622
• /
• 2006

Purpose : High-Sensitivity C-reactive protein(hs-CRP) has been recognized as a very useful and sensitive predictor of the future risk of myocardial infarction. But the clinical significance of hs-CRP in children remains uncertain. To confirm the existence of obesity-induced vascular inflammation and the association between metabolic syndromes and elevation of CRP in children, we investigated the relationship among CRP, obesity, blood pressure(BP), and serum lipids in schoolboys. Methods : Twenty-eight obese(BMI $29.61{\pm}3.29kg/m^2$) and 93 non-obese(BMI $18.99{\pm}2.21kg/m^2$) boys aged 14 years were examined. Serum CRP levels was measured by the high sensitive latex turbidimetric immunoassay and subjects with CRP levels below 0.3 mg/dL were adopted to avoid the influence of acute infection. Results : Obese children had significantly higher hs-CRP levels than their non-obese group($0.104{\pm}0.075$ vs. $0.054{\pm}0.005mg/dL$). In the obese group, BMI, systolic blood pressure, diastolic blood pressure, apolipoprotein B, atherogenic index, and triglyceride were significantly higher than in nonobese. The BMI, diastolic blood pressure, apolipoprotein E, atherognic index, and triglyceride showed positive correlation with log CRP by simple regression. Multiple regression analysis indicated that BMI and apolipoprotein E were strongly related to CRP. Conclusion : This study revealed that obese children tended to have higher levels of serum hsCRP, BP elevation and dyslipidemia than the control group and that BMI and apolipoprotein E were strongly related to CRP. These results indicate that obesity related metabolic syndrome can be developed in children.

• Clinical and Experimental Pediatrics
• /
• v.48 no.1
• /
• pp.34-39
• /
• 2005

Purpose : Perinatal complications associated with maternal smoking and alcohol ingestion in pregnancy have been well documented. But until now there has been no clinical data on it collected in our country. In this paper, we tried to research the association between maternal smoking and alcohol ingestion in pregnancy and the physical indices of the neonate at delivery among unmaried mothers from one charitable institution. Methods : We enlisted 125 unmarried with a history of smoking and alcohol consumption during pregnancy who delivered a baby in National Medical Center from March, 2001 to March, 2004 as a study group. As a control, 174 unmarried mothers without a history of smoking and alcohol were enlisted. Then, we compared the physical indices - birth weight, height, and the head circumference - of the neonates from both groups. Furthermore, we investigated the effects of the period and the quantity of maternal smoking and alcohol ingestion on the physical indices of the neonates from the both groups. Results : Low physical indices of neonates were found in the study group(either smoking group, or drinking group) compared with the control group. The quantity of smoking or drinking and the period of smoking or drinking have no significant effect on physical indices. Conclusion : We found that maternal smoking and alcohol result in the low physical index of neonates, and educational initiatives must be directed at expectant mothers to emphasize the harmful effects of smoking and alcohol ingestion in pregnancy.

• Clinical and Experimental Pediatrics
• /
• v.46 no.5
• /
• pp.467-473
• /
• 2003

Purpose : The Epstein-Barr virus(EBV), gamma herpesvirus, is an important pathogen that is widespread around the world. The EBV causes various diseases depending on the geographic location, and on the immunity or the premorbid condition of the person exposed to EBV. To evaluate EBV typing may be the most important step to figure out the pathogenesis of EBV associated diseases, and we need to re-evaluate the pathologic role of human leukocyte antigen(HLA) in developing Epstein- Barr virus associated acute infectious mononucleosis by using newly developed methods. Methods : This study included 24 children(age range : 6 to 13 years), serologically confirmed with acute infectious mononucleosis. The control group for the HLA type consisted of 200 age-matched healthy children. To classify HLA I, modified ARMs-PCR was used, while modified PCR-SSOP was utilized in typing of HLA II. Also, we performed EBV typing in study patients by using a one-step PCR. Results : The results of HLA types : In HLA class I, HLA-A24 was positive in 69 of 200 healthy children and positive in 14 of 24 patients in the study group(relative risk : 3.5724, chi-square; 5.26, P<0.05). In HLA class II, HLA-DRB1*07 was detected in 18 of 200 healthy children, and eight of 24 patients in the study group(relative risk; 506173, chi-square; 9.73, P<0.01). The results of EBV types : In the research group, 20(83.8%) of 24 patients were shedding type A virus, while 4(16.7%) were type B. Conclusion : We conclude that development of infectious mononucleosis may be associated with HLA types, and these results suggest that acute infectious mononucleosis could have hereditary traits. And we confirm that type A EBV is highly prevalent in patients with acute infectious mononucleosis in Korea. Also, our results suggest that further large scale studies, including adult groups, regarding the association between pathogenesis of EBV with HLA-DP or HLA-DQ will be warranted.

• Clinical and Experimental Pediatrics
• /
• v.52 no.6
• /
• pp.680-688
• /
• 2009

Purpose : Cysteinyl leukotrienes are important proinflammatory mediators in asthma. Recently, it was suggested that a promoter polymorphism in the genes encoding for leukotriene C4 synthase (LTC4S), a key enzyme in the leukotriene synthetic pathway, and cysteinyl leukotriene receptor 1 (CysLTR1) might be associated with aspirin-intolerant asthma. We investigated whether polymorphisms in LTC4S and CysLTR1 genes or their interactions were associated with the asthma phenotype, lung function, or bronchial hyperreactivity (BHR) in Korean children. Methods : A total of 856 asthmatic children and 254 non-asthmatic controls were enrolled; a skin prick test, lung function test and bronchial provocation test were performed. Of those enrolled, 395 children underwent exercise challenge tests. The LTC4S A(-444)C and CysLTR1 T(＋927)C were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis. Results : Of those enrolled, 699 children were classified as having atopic asthma and 277 children, as having exercise-induced asthma (EIA). LTC4S and CysLTR1 polymorphisms were not associated with atopic asthma, EIA, or asthma per se. Lung function and BHR were not significantly different between the wild type (AA or TT) and the variant (AC＋CC or TC＋CC) genotypes in asthmatics, atopic asthmatics, and EIA (＋) asthmatics, while total eosinophil counts were higher in the variant type of LTC4S than in the wild type in atopic asthmatics. There were no associations between the gene-gene interactions of LTC4S and CysLTR1 genotypes and the asthma phenotypes. Conclusion : LTC4S A(-444)C and CysLTR1 T(＋927)C polymorphisms and their gene-gene interactions are not associated with asthma phenotype, lung function, or BHR in Korean children.

• Clinical and Experimental Pediatrics
• /
• v.52 no.8
• /
• pp.910-916
• /
• 2009

Purpose : The arterial switch operation (ASO) has become the preferred procedure for the surgical management of transposition of the great arteries (TGA). We conducted a retrospective evaluation of our experience in 30 patients seen from January 2003 to July 2008, in order to determine outcomes and related risk factors after the arterial switch operation. Methods : Patients charts, surgical reports, and echocardiograms were retrospectively reviewed. And they were analyzed in 2 different groups: complex (n=16) versus simple TGAs (n=14). Complex TGAs are TGAs with VSD or the Taussig-Bing anomaly with or without aortic arch anomalies. Simple TGAs are defined as TGAs with intact ventricular septum having no such anomalies. Median follow-up time was 44 months (3-63 months). Results : Hospital mortality was 0%. However, follow-up echocardiographies revealed potential complications, including stenosis of the branch pulmonary arteries, neo-aortic and/or neo-pulmonary valvar regurgitation, and right or left ventricular outflow tract obstructions. Great arterial relationship (side-by-side), association of aortic arch anomalies, and the existence of the Taussig- Bing anomaly were assessed as significant risk factors of neo-aortic and/or neo-pulmonary valvar regurgitation in this series. On the other hand, right or left ventricular outflow tract obstructions were more frequently found in patients demonstrating VSD, side-by-side positioned great arteries, or associated coronary anomalies. Conclusion : The ASO is the procedure of choice in the treatment of TGA. However, special attention and follow-ups are needed to detect residual problems like the stenosis of the branch pulmonary arteries, neo-aortic and/or neo-pulmonary valvar regurgitation, as well as ventricular outflow tract obstructions.

• Clinical and Experimental Pediatrics
• /
• v.52 no.8
• /
• pp.875-880
• /
• 2009

Purpose : Iron-deficiency anemia remains the most common nutritional deficiency in young infants. This study aimed to survey the actual condition of feeding patterns and weaning diet and to study the association between these factors and the prevalence of anemia in infants aged over 9 months. Methods : We studied 171 infants aged 9-24 months who were hospitalized in the general ward with mild to moderate acute illnesses. The mothers answered a questionnaire about the feeding patterns and the status of the weaning diet of their infants. The infants were divided into three groups: infants who were exclusively breast-fed, those who had been given mixed feeding, and artificial milk feeders. Results : The incidence of anemia was significantly higher in exclusively breast-fed infants (23/68, 33.8%) than in the infants with mixed feeding (11/62, 17.7%) and artificial milk feeders (5/41, 12.1%). The mothers' awareness about the state of their infants󰡑 weaning diet was not related to the presence of anemia in the exclusively breast-fed infants. About 70% of the infants had started the weaning diet before age 6 months in all three groups, without any difference according to feeding patterns. Conclusion : The incidence of anemia was significantly higher in the breast-fed group than in the other infants. Many mothers of breast-fed infants with anemia also believed that their infants were taking sufficient weaning foods. Therefore, further education of the mothers about iron-rich weaning foods and the importance of iron intake during infancy is needed to prevent anemia, especially in breast-fed infants.

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

• Korean Journal of Poultry Science
• /
• v.36 no.3
• /
• pp.207-213
• /
• 2009

Adenylosuccinate lyase (ADSL) deficiency is a disease of purine metabolism which affects patients both biochemicall and behaviorally. An obstacle of this purine nucleotide cycle(PNC) can be caused brain functional disorder and growth disorder. So ADSL deficiency, which is associated with sever mental retardation, autistic features and energy metabolism. This study was performed to identify SNP on ADSL gene in chicken. The nucleotides were observed as T to C ($7724^{th}$ nucleotide), C to T ($7732^{nd}$ nucleotide), G to T ($10108^{th}$ nucleotide), A to T ($10356^{th}$ nucleotide), G to A($10375^{th}$ nucleotide), A to C ($10402^{nd}$ nucleotide), A to T ($12716^{th}$ nucleotide), T to A ($12717^{th}$ nucleotide), C to T ($15491^{st}$ nucleotide), C to T ($15542^{nd}$ nucleotide) and C to T ($15550^{th}$ nucleotide). The nucleotide substitutions at $15542^{nd}$ and $15550^{th}$ (GeneBank accession no. AY665559) were found as missense mutation (alanine$\rightarrow$valine, proline$\rightarrow$serine, respectively). This study will be useful for farther researches for identifying association between these SNPs and energy metabolism in chicken. The C15550T SNP showed three genotypes, CC, CT, TT by digestion with the genotype TT had significantly faster the first lay day (150.0) than CT (162.0, P<0.05) and genotype TT (150.0, P<0.05) had significantly higher the egg production rate than CT (172.4, P<0.05). According to result of this study, a C15550T was found to have a significantly effect first lay day and mean egg production. It will be possible to use SNP marker on selecting chicken to improve important economic traits, which is the first lay day and mean egg production.