• 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.45 no.9
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• pp.1114-1119
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• 2002

Purpose : The purposes of this study were to determine short- and long-term fractal correlation behavior of heart rates during daily activity in patients with neurocardiogenic syncope. Methods : Twenty five patients with histories of neurocardiogenic syncope episodes were included. Their analogue 24h ambulatory Holter electrocardiograms were analyzed. The tape was digitized and the digitized electrocardiograms were partioned into sections of one hour. Then their RR intervals were measured and 20,000 points of RRI were used. To quantify the fractal correlation behavior, we employed the detrended fluctuation analysis, and short-term($n{\leq}16$, ${\alpha}_1$) and long-term(n>16, ${\alpha}_2$) fractal scaling exponents were calculated. Results : When compared to control, 24-hour average values of ${\alpha}_1$ and all ${\alpha}_1$ values at quarters of each day were significantly higher in patients with syncope. On the contrary, their 24-hour average value of ${\alpha}_2$ and all ${\alpha}_2$ values at quarters of each day were lower in patients with syncope. However, statistical significances were found in 24-hour average value of ${\alpha}_2$ and in ${\alpha}_2$ value at MN-6AM. Conclusion : In the syncope patients with neurocardiogenic syncope, short-term fractal scaling exponents of RR interval was significantly high throughout the day. Therefore, their RR intervals were smoother in the short term scale and had a tendency to continue in the same direction of increase or decrease, which may contribute to persistent decrease in heart rate during a syncopal attack.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.80-84
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• 2010

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

• The Sea:JOURNAL OF THE KOREAN SOCIETY OF OCEANOGRAPHY
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• v.20 no.3
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• pp.119-130
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• 2015

Impacts of Argo temperature assimilation on the analysis fields in the East Sea is investigated by using DAESROM, the East Sea Regional Ocean Model with a 3-dimensional variational assimilation module (Kim et al., 2009). Namely, we produced analysis fields in 2009, in which temperature profiles, sea surface temperature (SST) and sea surface height (SSH) anomaly were assimilated (Exp. AllDa) and carried out additional experiment by withdrawing Argo temperature data (Exp. NoArgo). When comparing both experimental results using assimilated temperature profiles, Root Mean Square Error (RMSE) of the Exp. AllDa is generally lower than the Exp. NoArgo. In particular, the Argo impacts are large in the subsurface layer, showing the RMSE difference of about $0.5^{\circ}C$. Based on the observations of 14 surface drifters, Argo impacts on the current and temperature fields in the surface layer are investigated. In general, surface currents along the drifter positions are improved in the Exp. AllDa, and large RMSE differences (about 2.0~6.0 cm/s) between both experiments are found in drifters which observed longer period in the southern region where Argo density was high. On the other hand, Argo impacts on the SST fields are negligible, and it is considered that SST assimilation with 1-day interval has dominant effects. Similar to the difference of surface current fields between both experiments, SSH fields also reveal significant difference in the southern East Sea, for example the southwestern Yamato Basin where anticyclonic circulation develops. The comparison of SSH fields implies that SSH assimilation does not correct the SSH difference caused by withdrawing Argo data. Thus Argo assimilation has an important role to reproduce meso-scale circulation features in the East Sea.

• Clinical and Experimental Reproductive Medicine
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• v.34 no.3
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• pp.179-188
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• 2007

Objectives: Many neurological diseases are known to be caused by expansion of trinucleotide repeats (TNRs). It is hard to diagnose the alteration of TNRs with single cell level for preimplantation genetic diagnosis (PGD). In this study, we describe methods optimized for PGD of TNRs related diseases such as Huntington's disease (HD), spinocerebellar ataxia 3 (SCA3) and fragile X syndrome (FXS). Methods: We performed the preclinical assays with heterozygous patient's lymphocytes by single cell PCR strategy. Fluorescent semi-nested PCR and fragment analysis using automatic genetic analyzer were applied for HD and SCA 3. Whole genome amplification with multiple displacement amplification (MDA) method and fluorescent PCR were carried out for FXS. Amplification and allele drop-out (ADO) rate were evaluated in each case. Results: The fluorescent semi-nested PCR of single lymphocyte showed 100.0% of amplification and 14.0% of ADO rate in HD, and 94.7% of amplification and 5.6% of ADO rate in SCA3, respectively. We could not detect the PCR product of CGG repeats in FXS using the fluorescent semi-nested PCR alone. After applying the MDA method in FXS, 84.2% of amplification and 31.3% of ADO rate were achieved. Conclusions: Fluorescent semi-nested PCR is a reliable method for PGD of HD and SCA3. The advanced MDA method overcomes the problem of amplification failure in CGG repeats of FXS case. Optimization of methods for single cell analysis could improve the sensitivity and reliability of PGD for complicated single gene disorders of TNRs.

• The Korean Journal of Malacology
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• v.24 no.3
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• pp.229-241
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• 2008

Changes of growth and histopathological feature in the mantle structure of the equilateral venus, Gomphina veneriformis exposed to tribultyltin chloride (TBTCl) for 36 weeks were observed. Concentrations of TBTCl were 0, 0.4, 0.6, and $0.8{\mu}g/L$. A regression analysis by power function of SPSS was shown that the growth of experimental groups was significantly decreased after 12 weeks of exposure. For histological analysis, mantle tissues were characterized using H-E stain, AB-PAS (pH 2.5) reaction and Masson's trichrome stain, and epidermal layer thickness and mucous cell distribution were analysed using the image analyser. The mantle had 4-folds (inner-inner, inner-outer, middle, and outer) and its epidermal layer consisted of simple epithlia. A periostracum was observed in the periostracal groove between middle and outer fold. Inner epidermal layer consisted of simple ciliated columnar epithelia, but the outer epidermal layer consisted of simple non-ciliated columnar epithelia. Alcian blue positive mucous cells showed blue color (7462c, 653c) in the inner fold, violet color (2583c) in the middle fold, and blue color (647c, 7455c) in inner epidermal layer (numbers in the parenthesis are codes of Pantone process coated color). Hemolymph sinus in the mantle was extended, and mucous cells in inner plica of the middle fold were stained as blue (7455c) and violet (2587c), after 12 weeks of TBTCI exposure. Cilia and striated border were disappeared, and number of mucous cells in the inner epidermal layer was reduced. Serious histopathological changes in middle and outer fold near the periostracum were observed after 36 weeks. Moreover, epidermal layer thickness and mucous cell distribution were showed decreasing tendency as exposure time to TBTCI was increased. Results of this study suggested that TBTCl induced growth disorder with histopathological changes.

• Journal of Bio-Environment Control
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• v.29 no.4
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• pp.320-325
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• 2020

This study aimed to analyze thermo-keeping and economic feasibility by utilizing silica aerogel, which has been attracting attention as a new material, complementing the disadvantages of the conventional multi-layered thermal screen, and producing and installing multi-layered thermal screen. The multi-layered thermal screen used in the experiment was produced in two combinations using a non-woven fabric containing silica aerogel and measured and compared the temperature and fuel consumption changes due to differences in practice with the multi-layered thermal screen being sold and used on the market. Experimental results show that the temperature and relative humidity changes due to the differences of the multi-layered thermal screens in the single-span greenhouse and the multi-span greenhouse were small but remained almost the same temperature and relative humidity. It is judged that this shows that the multi-layered thermal screen using silica aerogel is not inferior to the conventional multi-layered thermal screen. As a result of a comparative analysis of heating energy, the aerogel-based multi-layered thermal screen reduced fuel consumption by about 15% in the single-span greenhouse and about 20% in the multi-span greenhouse compared to the conventional multi-layered thermal screen. It is clear that heating energy is saved as a greenhouse size and duration increase. It was found that the silica aerogel-based multi-layered screen was more breathable and warmer than the conventional multi-layered thermal screen, but It was found that the multi-layered screen used in the multi-span greenhouse was heavier and stiff compared with the conventional multi-layered thermal screen, indicating less workability and operability. Therefore, improvements were applied to the multi-layered screens used in the single-span greenhouses. It was confirmed that the replacement of internal insulation materials reduced thickness and improved stiffness so that there could be sufficient possibility for farmers to use.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.310-314
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• 2009

Purpose : Pneumothorax is an important factor responsible for increased mortality and morbidity in neonates. Here, we compared the clinical findings and prognosis for neonatal pneumothorax induced by respiratory distress syndrome (RDS) and pneumonia. Methods : Between January 2001 and December 2005, 80 patients with neonatal pneumothorax induced by RDS and pneumonia and admitted to the NICU of Dongsan Medical Center, Keimyung University, were enrolled. They were assigned to the RDS group (30 cases) or the pneumonia group (50 cases). Admission records for gestational age, onset day of life, rate of ventilatory care and thoracostomy, and prognosis were retrospectively reviewed and statistically analyzed for both groups. Results : The mean gestation age was significantly shorter in the RDS group (32.3 weeks) than in the pneumonia group (38.1 weeks) (P<0.001), and the mean onset day of life were later in the RDS group (4.6 days) than in the pneumonia group (1.8 days) (P<0.05). Rates of ventilatory care and thoracostomy were higher for the RDS group than for the pneumonia group (100% vs. 44%, and 66.7% vs. 48%, respectively). Total mortality rates and pneumothorax specific mortality rates were higher for the RDS group than for the pneumonia group (46.7% vs. 18%, P<0.01 and 33.3% vs. 16%, P<0.05, respectively). After logistic regression analysis, preterm significantly increased the mortality rate (OR 7.44, 95% CI: 1.99-27.86, P<0.005), but bilateral involvement (OR 1.17, 95% CI: 0.82-1.67, P>0.5) and the RDS group itself (OR 1.70, 95% CI: 0.52-5.54, P>0.3) did not increase mortality rates significantly. Conclusion : Our study suggests that neonatal pneumothorax in the RDS group tends to have a later onset, higher mortality rate, and needs a higher rate of thoracostomy than the pneumonia group. However, after logistic analysis, only preterm significantly and independently increased the mortality rate.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.34 no.1
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• pp.1-11
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• 2012

Purpose: The purpose of this study was to evaluate the bone regeneration capacity of silk fibroin (SF) when combined with beta tricalcium phosphate (${\beta}$-tricalcium phosphate [TCP]) and rh-bone morphogenetic protein (BMP) in vivo by micro-computed tomography (CT), soft x-ray, and histological analysis. Methods: A total of 56 critical size defects formed by a trephine bur made on 28 adult female Spague-Dawley rats were used for this study and the defect size was 5.0 mm in diameter. The defects were transplanted with (1) no graft material (raw defect), (2) autogenous bone, (3) SF ($10{\mu}g$), (4) SF-BMP ($10{\mu}g$, $0.8{\mu}g$ each), and (5) SF+${\beta}$-TCP ($10{\mu}g$). At 4 and 8 weeks after operation, the experimental animals were sacrificed. Samples were evaluated with soft x-ray, histological examinations and 3-dimensional micro-CT analysis. Results: In the 3-dimensional micro-CT evaluation, bone volume and bone surface data were higher in the SF-BMP ($12.8{\pm}1.5$, $138.6{\pm}45.0$ each) (P<0.05) and SF-TCP ($12.3{\pm}1.5$, $144.9{\pm}30.9$ each) group than in the SF group ($6.1{\pm}3.3$, $77.2{\pm}37.3$ each) (P<0.05), except for the autogenous group ($15.0{\pm}3.0$, $190.7{\pm}41.4$ each) at 4 weeks. At 8 weeks, SF-BMP ($16.8{\pm}3.5$, $173.9{\pm}34.2$ each) still revealed higher (P<0.05) bone volum and surface, but SF-TCP ($11.3{\pm}1.5$, $1132.9{\pm}52.1$ each) (P=0.5, P=0.2) revealed the same or lower amount compared with the SF group ($13.8{\pm}2.7$, $127.5{\pm}44.8$ each). The % of bone area determined by radiodensity was higher in the SF-TCP ($31.4{\pm}9.1%$) and SF-BMP ($36.2{\pm}16.2%$) groups than in the SF ($19.0{\pm}10.4$) group at the period of 4 weeks. Also, in the histological evaluation, the SF-BMP group revealed lower inflammation reaction, lower foreign body reaction and higher bone healing than the SF group at postoperative 4 weeks and 8 weeks. The SF-TCP group revealed lower inflammation at 4 weeks, but accordingly, as the TCP membrane was absorbed, inflammatory and foreign body reaction are increased at 8 weeks. Conclusion: The current study provides evidence that the silk fibrin can be used as an effective grafted material for tissue engineering bone generation through a combination of growth factor or surface treatment.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1325-1331
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• 2002

Purpose : We tried to check the accuracy of references in the Journal of the Korean Pediatric Society and Journals of the Korean Pediatric Subspecialty Societies. We also wanted to know the citation patterns of authors by analyzing the frequency of cited materials. Methods : Three journals were randomly selected from the 2000 issues of Journal of the Korean Pediatric Society and nine journals were selected mainly from the second half of 2000 issues of each Journal of the Korean Pediatric Subspecialty Societies for the study. Then the accuracy of references was checked with Medline. Journals before 1964, books, and journals which were not written in English were used only in the citation pattern analysis. Results : Author errors were the most common(21.3%) among the reference elements. The next was title errors, followed by page errors, journal errors, volume errors and year errors. Total average error rate was 34.7% and we were unable to find journals in 89 cases(2.2%). The journal of the Korean Society of Neonatology had the lowest error rate(17.4%) and the journal of the Korean Pediatric Cardiology Society had the highest error rate(53.2%). The reference journals which were published and quoted in the most recent three years were only 612 cases(15%). Foreign journals were selected as reference(78.4%) more than domestic journals; The Journal of the Korean Pediatric Society was the most frequently cited reference(43.3%) among domestic journals. Conclusion : Authors are ultimately responsible for the accuracy of references and they should check the reference list with responsibility. Hopefully, authors also will have to use more domestic journals and recent journals.