• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1126-1133
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• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.893-897
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• 2009

Purpose : Advances in neonatal intensive care have improved the survival rate of low-birth-weight infants, but mild bronchopulmonary dysplasia (BPD) with the accompanying need for prolonged oxygen supplement remains problematic. Maternal chorioamnionitis and neonatal ventilator care affect the development of BPD. This study aimed to examine whether maternal chorioamnionitis or neonatal ventilator care affect the development of BPD dependently or independently. Methods : We performed a retrospective study of 158 newborn infants below 36 weeks of gestational age and 1,500 gm birth weight admitted to the neonatal intensive care unit of Daegu Fatima Hospital between January 2000 and December 2006. We analyzed the incidence of BPD according to maternal chorioamnionitis and neonatal ventilator care. Result : Histologic chorioamnionitis was observed in 50 of 158 infants (31.6%). There were no significant differences in the development of BPD (P=0.735) between the chorioamnionitis (+) and chorioamnionitis (-) groups. In the multiple regression analysis, ventilator care (OR=7.409, 95% CI=2.532-21.681) and neonatal sepsis (OR=4.897, 95% CI=1.227-19.539) affected the development of BPD rather than maternal chorioamnionitis (OR=0.461, 95% CI=0.201-1.059). Conclusion : Ventilator care or neonatal sepsis may play a role in the development of BPD rather than maternal chorioamnionitis.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.434-440
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• 2009

Background: Surgical closure of a patent ductus arteriosus (PDA) can be considered when conservative medical treatment is ineffective or contraindicated. Low weight and earlier gestational age neonates who are treated with conservative medical therapy generally showed a higher failure rate. The morbidity of surgical PDA closure in such extremely low birth weight (ELBW) neonates is also high. Here we present the early results of a new technique for approaching the PDA through a dorsal minithoracotomy. Material and Method: From March 2006 to November 2008, 24 premature neonates underwent surgical PDA closure. The procedures were performed in the newborn intensive care unit via a 2 cm long dorsal minithoracotomy with the baby in the prone position with the left hemithorax elevated 30$^{\circ}$. Bimanual cotton swab blunt dissection completed the extrapleural accesstothe PDA and then two clips were applied. Tube thoracostomy was avoided if there was no meaningful pleural laceration. Result: The infants mean gestational age was 26.5$\pm$2.1 weeks (range: 23 to 30 weeks) and the average age at operation was 11$\pm$11 days. The mean body weight at operation was 933$\pm$271 grams (range: 570 to 1,700 grams). Eight patients expired, but there was no procedure-related death. Postoperative echocardiography revealed two cases of residual shunt but none of these shunts were detected on the follow up echocardiogram that was performed on the post operative 5 and 59 days. Conclusion: We concluded that the technique described here is an effective procedure in view of the satisfactory operative exposure and the low rate of complications.

• 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.

• Neonatal Medicine
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• v.17 no.1
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• pp.94-101
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• 2010

Purpose:In recent years, Korea has showed a steady increase in the frequency of teenage birth, while the overall birth rate has declined. As the teenage birth is known as a high risk pregnancy itself, we examined perinatal complications of teenage mothers and whose neonates in aspects of medical problem, and social status and support. Methods:We examined the perinatal characteristics of teenage mothers and whose babies, who were hospitalized at Korea University Ansan Hospital from January 2004 to July 2009 using medical records retrospectively. Twenty-seven teenage mothers and their 28 babies were enrolled in this study. Results:Teenage mothers were all unmarried and showed high rates of preterm labor, maternal anemia, and unexpected delivery. Among them, 11 (40.7%) were from families that were separated. Eleven mothers (40.7%) did not have any antenatal care. There were high rates of prematurity and low birth weight (60.7% and 64.3%, respectively). The complication included: respiratory distress syndrome, patent ductus arteriosus and necrotizing enterocolitis. Fourteen babies (51.9%) were not going to be brought up by their biological parents. Conclusion:Teenage pregnancy had high rates of preterm labor and associated complications, often caused by the lack of proper antenatal care. Babies from unmarried mothers were likely to be adopted and this could be a social burden. Therefore, to reduce unplanned teenage pregnancy and births, sex education and social supports should be provided to all teenagers.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.75-81
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• 1999

A survey on the intestinal atresias was made among 34 members of Korean Association of Pediatric Surgeons. The response rate was 82.4 %. Two hundred and fifteen patients from the January 1, 1994 to December 31, 1996 were analyzed. The lesions were 73 cases of duodenum(DA), 72 cases of jejunum(JA), 71 cases ileum(IA) and 2 cases cecum and sigmoid colon respectively. There were 2 cases of combined anomalies (DA + JA + IA and DA + JA). Male to female ratio was 1:1 in DA, and 1.8:1 in JA. Seventy four cases(34.3 %) were premature babies(DA 35.2 %, JA:48.6 %, IA:19.2 %), and 62 cases(28.7 %) had low birth weight (DA:39.4 %, JA 33.0 %, IA:13.7 %). Antenatal diagnosis was made in 92 cases(43.6 %). However 22 cases (23.9 %) of them were transferred to pediatric surgeon after delivery. Maternal polyhydramnios was observed in 63 cases(28.9 %). Seventy· five cases(34.4 %) were taken only simple abdominal film for diagnostic studies. The associated malformations were observed in 54 aresia and were observed more frequently in DA(35 cases, 47.9 %). Meconium peritonitis due to intrauterine bowel perforation was more frequently associated with IA compared to DA and JA. The overall mortality rate was 30 %. (Abbreuations: $P_{T}$;p-value in total, $P_{DJ,DI,JI}$;p value between two groups among duodenal, jejunal and ileal groups).

• Neonatal Medicine
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• v.17 no.2
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• pp.245-249
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• 2010

Purpose: Numerous false alarms by pulse oximetry, which is widely used in neonatal intensive care unit, can delay response to true alarms. Masimo $SET^{(R)}$ was introduced lately, to overcome false alarms by motion. We compared the clinical performance of two devices (Nellcor $N-595^{(R)}$ and Masimo $SET^{(R)}$) for the evaluation of the false alarm frequency during usual motion artifacts and stable state. Methods: A total of 20 preterm infants weighing 1,000-2,500 g were enrolled in the study. The sensors of two devices were placed on the different feet on the same infants, and both devices were programmed to emit an alarm for episode of hypoxemia (SpO2$\leq$85%). The false alarms were defined as episodes of poor correlation with ECG heart rate, poor waveforms, and the absence of obvious signs of hypoxia. We compared the frequency of false alarms between the two devices. Results: The mean chronological age was 20.8 days and the mean body weight was 1,668 g on the study day. The frequency of total false alarm was significantly fewer for Masimo $SET^{(R)}$ pulse oximetry (48 in Nellcor $N-595^{(R)}$, 27 in Masimo $SET^{(R)}$) although the false alarm during usual motion artifacts was not significantly between two devices (32 in Nellcor $N-595^{(R)}$, 19 in Masimo $SET^{(R)}$). Conclusion: The Masimo $SET^{(R)}$ pulse oximetry has fewer false alarm rates and identified more true hypoxic events than Nellcor $N-595^{(R)}$ pulse oximetry. Therefore, it is useful for adequate oxygen therapy and helps to decrease unnecessary handling by clinicians and nurses.

• Journal of Environmental Health Sciences
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• v.33 no.4
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• pp.227-234
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• 2007

Recent epidemiologic studies show that gestational exposure to air pollution adversely affects pregnancy outcomes including low birth weight in preform birth. In this study, we evaluated the effect of air pollutants on LBW (low birth weight) on firstborn fetus throughout the gestational period using the birth cohort between 1999 and 2003 in Seoul. Using birth cohort data from the National Statistics Office of Korea we identified 288,346 firstborn births (excluded missing data on lack of information for birth weight and discordance between residential and certificated address from a total of 316,451) during 1999 to 2003 with complete covariate (gender, parity, date of birth, gestational age, parental age and educational level, maternal occupation etc.) and maternal residential history data. Our subjects were defined as more than 37 weeks and less than 44 weeks of completed gestation and we identified 5,457 persons (1.89%) by low birth weight (<2.5 kg) in this study. Using logistic regression, we estimated the risk of mean (entire pregnancy and trimester period) air pollution concentrations for CO, $O_3,\;PM_{10},\;NO_2\;and\;SO_2$. In terms of trimester-specific exposure, we found that some air pollutants exposure in each trimester would increase the risk for LBW. Results also showed that the effect size of air pollutants exposure during the first and third trimester is higher than during the second trimester. In all trimester, the estimated risk of LBW was 1.831 (95% CI=1.573-2.132) with unit increase for CO, 1.139 (95% CI=1.107-1.172) for 50, and 1.009 (95% CI=1.001-1.017) for $O_3$. Our results suggest that exposure during the gestation period to relatively low levels of some air pollutants may be associated with a reduction in birth weight on first-born fetus. These findings implicate the effective risk management strategies should be applied to minimize the public health impacts for pregnant women.

• Journal of Preventive Medicine and Public Health
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• v.36 no.2
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• pp.117-124
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• 2003

Objectives : This study was performed to examine the relationship between maternal exposure to environmental tobacco smoke (ETS) and pregnancy outcomes (low birth weight or preform baby) in a prospective cohort study. Methods : We made a pregnant women's cohort, and followed the pregnancy outcomes, between May 1st 2001 and August 31st 2002. We surveyed 2,250 women who visited our hospital during their 35th gestational week, with a self-administered questionnaire. The final total of mother-infant pairs analyzed in this study was 1,712. We used a multiple logistic regression analysis to analyze the effect of maternal ETS on the incidence of preform or low birth weight, and a linear regression analysis for the birth weight and gestational age. Results : Higher exposure to ETS (>=1 hours/day) during pregnancy was more negatively associated with the gestational age and birth weight, than no exposure to ETS (no or less than 1 hour). Maternal exposure to ETS was associated with preform baby(adjusted odds ratio (AOR) 1.7; 95% confidence interval (CI) 0.9, 3.3) and low birth weight (AOR 2.3; 95% CI 0.9, 5.5). In addition, we found that maternal ETS may reduce the birth weight by 70g after adjusting for potential confounding factors. Conclusions : This study suggests that maternal exposure to ETS during pregnancy may increase the frequency of low birth weights and preform births.

• Advances in pediatric surgery
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• v.14 no.1
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• pp.37-47
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• 2008

Recently, the survival rates of extremely low-birth-weight (ELBW) infants have improved with the development of neonatal intensive care. However, these infants were susceptible to intestinal perforation due to prematurity, fluid restriction, and injection of indomethacin, etc. Because of the risks of transportation, anesthesia and surgery itself, peritoneal drainage has been compared with laparotomy. Through our experience, we investigate the usefulness of peritoneal drainage retrospectively. From 1997 to 2007, six ELBW (M:F=5:1) underwent primary peritoneal drainage for intestinal perforation. Their median birth weight was 685g (405~870) and gestational age was $25^{+1}$ weeks ($24^{+3}{\sim}27^{+0}$). We noticed the intestinal perforation at median 10.5 days (8~18) after birth, and placed Penrose drain or Jackson-Pratt drain through right lower quadrant incision under local anesthesia. The cause of intestinal perforation was necrotizing enterocolitis in one patient, but that of the others was not clear. Three patients who showed normal platelet count and stable vital signs recovered uneventfully. Two patients (birth weight less than 500g) who showed unstable vital signs and low platelet count (12,000 / $mm^3$ to 30,000 / $mm^3$)expired despite aggressive resuscitation. One patient required laparotomy due to persistent intestinal obstruction after drain removal and survived. Our experience shows that peritoneal drainage was an acceptable treatment for ELBW infants and the prognosis was related to vital sign and platelet count at the time of intestinal perforation, and birth weight.