• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1056-1060
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• 2006

Purpose : As hearing ability affects language and cognitive development, early detection and intervention of congenital hearing defects is very important. We analyzed the result of newborn hearing screening using automated auditory brainstem response and estimated the incidence of congenital hearing defects in newborn infants in Korea. Methods : Hearing screening tests were done on 7,218 newborn infants who were delivered at Cheil General Hospital from July 1, 2004 to June 30, 2005. The first screening test was done on the second day of life with automated auditory brainstem response(AABR) using $ALGO{\bigcirc}^{(3)}$ Newborn hearing screener($Natus^{(R)}$ Medical Incorporated, San Carlos, USA) with 35 dB sound level. The newborn infants who did not pass the initial screening test took the second screening AABR test before discharge from the nursery. Infants who did not pass these screenings at the nursery were followed up at the Department of Otorhinolaryngology, Samsung Seoul Hospital. Results : Total 7,218 infants(83.3 percent of total 8,664 live births of the Cheil General Hospital) were screened in the nursery, and 55 of them failed to pass the newborn screening. Among 55 infants who were referred, six were lost during follow-up, and 14 were confirmed as hearing impaired. Six of them(42.8 percent) do not have any risk factors for hearing impairment. We can estimate that the incidence of hearing defects is about 1.9-2.8 per 1,000 live births. Conclusion : Automated auditory brainstem response is an effective tool to screen the hearing of newborn infants. Congenital hearing loss is more frequent than metabolic diseases on which screening tests are available in the newborn period. About 40 percent of infants who have hearing defects do not have any risk factors for hearing impairment. Therefore, universal newborn hearing screening must be recommended to all neonates.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.505-511
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• 2002

Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may predict the clinical course. Methods : From December 1995 to May 2001, 71 patients with ITP were admitted to Ajou University Hospital. They were treated with IVIg 400 mg/kg for five days and MPV, PDW, platelet count were analysed. Normal control group(n=38) was compared. The study group was divided into acute and chronic forms, and also divided into group A, good early responders whose platelets increased more than $100,000/{\mu}L$ within 5 days and group B who did not. Results : Mean value of MPV at diagnosis in ITP patients was lower than the normal control group(P<0.05). In group A, MPV was abruptly increased on the first day after IVIg and then started to decrease. But in group B, MPV was steadily increased until the fourth day after IVIg. In the normal control group, there were inverse correlations between platelet count and MPV(r=-0.415, P<0.05), but in ITP patients, there were positive relationships between platelet count and MPV(r=0.646, P<0.05) at diagnosis. Conclusions : MPV at diagnosis of ITP was lower than the normal control. MPV and PDW could not predict the course of ITP patients, but MPV could distinguish good early responders. More research is needed to find out the reasons of decreased MPV at diagnosis of ITP.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.183-191
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. Methods : Btk protein expression was analyzed by flow cytometry. Isolation of the coding sequence of the Btk gene was performed by amplification using the reverse transcription-polymerase chain reaction(RT-PCR) technique. Sequence alterations were screened by the single-stranded conformation polymorphism(SSCP) method and characterized by standard sequencing protocols. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in three patients with XLA. In addition, Btk protein analysis clearly showed cellular mosaicism in monocytes from four obligate carriers, findings further supported by SSCP. A single base pair mutation(T to C) in Btk-exon three, which encodes the PH domain, was identified in four XLA patients. A diagnostic sequencing analysis was established to detect heterozygotic pattern in 4 carrier females. Furthermore, we found significant clinical heterogeneity in individuals with the same gene mutation. Conclusion : The implicating genetic alteration provided valuable clues to the pathogenesis of XLA in Korea and the flow cytometric analysis was suggested as a useful tool for rapid detection of XLA patients and carriers. The present study has identified a genetic mutation in the Btk coding region and demonstrated heterogeneity in clinical manifestations among patients with the same mutation. A flow cytometric analysis was found to be informative in establishing a deficiency of Btk protein in both patients and carriers and is recommended as a frontline procedure in the molecular diagnosis and work-up of XLA.

• Journal of Chest Surgery
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• v.43 no.4
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• pp.394-398
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• 2010

Background: The overexpression of transforming growth factor-beta 1 receptor II (TGF-${\beta}1$RII) and transforming growth factor-beta 1 (TGF-${\beta}1$) ligand may be involved in the formation of a bulla. In this study, we tested if serum TGF-${\beta}1$ ligand levels correlated with the expression level of TGF-${\beta}1$RII and TGF-${\beta}1$ in bullous tissues from patients with spontaneous pneumothorax. Material and Method: Bullous lung tissues and blood samples were obtained from 19 patients with spontaneous pneumothorax, 18 males and 1 female, aged 17 to 35 years old. The bullous tissues were obtained by video-assisted thoracic surgery (VATS), fixed in formalin, embedded in paraffin, and cut into $5{\sim}6{\mu}m$ thick slices. Sections were immunohistochemically stained with primary antibodies against TGF-${\beta}1$ or TGF-${\beta}1$RII, and serum levels of TGF-${\beta}1$ in patients and normal controls was measured by enzyme-linked immunosorbent assay (ELISA). Result: Of the 19 patients, 16 were TGF-${\beta}1$ positive and 10 were TGF-${\beta}1$RII positive. Among the 16 TGF-${\beta}1$ positives, 9 were also TGF-${\beta}1RII$ positive. As seen previously, strong immunohistochemical staining of TGF-${\beta}1$RII and TGF-${\beta}$ was detected in the boundary region between the bullous and normal lung tissues. Average TGF-${\beta}1$ blood levels of both TGF-${\beta}1$ and TGF-${\beta}1$RII positive patients was $38.36{\pm}16.2ng/mL$, and that of five controls was $54.06{\pm}15ng/mL$. Conclusion: These results suggest that overexpression of TGF-${\beta}1$ and TGF-${\beta}1$RII expression may be involved in the formation of bullae. TGF-${\beta}1$ blood levels in patients with primary spontaneous pneumothorax is lower than normal people, suggesting that the high level of local TGF-${\beta}1$ expression in the bullous tissue region, but not in the whole blood, may contribute more in the formation of bullae.

• Journal of Genetic Medicine
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• v.5 no.2
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• pp.139-144
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• 2008

We report on two cases of pericentric inversion of X chromosome. The cases were found in a 40-year-old man with azoospermia and in a family of a 38-year-old pregnant woman. The first case with 46,Y,inv(X)(p22.1q27) had concentrations of LH, prolactin, estradiol, and testosterone that were within normal ranges; however, FSH levels were elevated. Testis biopsy revealed maturation arrest at the primary and secondary spermatocytes without spermatozoa. There were no microdeletions in the 6 loci of chromosome Y. For the second case, the cytogenetic study of thepregnant woman referring for advanced maternal age and a family history of inversion X chromosome was 46,X,inv(X)(p22.11q27.2). The karyotype of her fetus was 46,X,inv(X)(p22.1q27). Among other family members, the karyotypes of an older sister in pregnancy and her fetus were 46,X,inv(X)(p22.11q27.2), and 46,Y,?inv(X), respectively. The proband's father was 46,Y,inv(X)(p22.11q27.2). All carriers in the family discussed above were fertile and phenotypically normal. In addition, the ratio of inactivation of inv(X) by RBG-banding was discordant between the two sisters, with the older sister having only 4.1% of cells carrying inactivated inv(X) while the proband had a 69.5% incidence of late replicating inv(X). Therefore, we suggest that the cause of azoospermia in the first case might be related to inversion X chromosome with positional effect. Also, the family of the second case showing normal phenotype of the balanced inv(X) might be not affected any positional effect of genes.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.268-272
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• 2006

Purpose : The reinfection rate of H. pylori reported before $^{13}C$-urea breath test($^{13}C$-UBT) era was higher than that of the post $^{13}C$-UBT era. Children are usually reluctant to receive invasive endoscopic evaluation for the reinfection of H. pylori, particularly when they are asymptomatic. The aim of the study is to discover the reinfection rate by different diagnostic tests, and to find out what causes the difference. Methods : Children confirmed to be eradicated from H. pylori were included in the study. Reinfection was evaluated by endoscopic biopsy based tests(n=34, mean age $11.5{\pm}3.7$ years) and/or a $^{13}C$-UBT(n=38, mean age $10.0{\pm}3.6$ years) at the time of 18 months after eradication. At first visit, H. pylori infection had been diagnosed by positive results from a rapid urease test, Giemsa stain and Warthin-Starry stain and/or a positive culture. Eradication was defined as negative results from all above tests 1-3 months after eradication therapy. Results : Reinfection rate by endoscopic biopsy based tests was 35.3 percent(12/34). All patients had abdominal symptoms(P=0.000). Reinfection rate was 13.2 percent(5/38) by a $^{13}C$-UBT. Reinfection rate was higher in children with abdominal symptoms(P=0.008). There was no evidence that reinfection rate depended on the sex(P=0.694), age(P=0.827), diseases(peptic ulcers vs gastritis, P=0.730) and eradication regimen(P=0.087). Conclusion : Helocibacter pylori reinfection rate in Korean children was 13.2 percent per 18 months by a non-invasive test or $^{13}C$-UBT. Accurate determinations of the reinfection rate in children is affected by the compliance of the diagnostic tests. Non-invasive tests should be considered to investigate the reinfection rate in children.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.169-176
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• 2010

Purpose : We aimed to evaluate predictive parameters for non-response to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD) before IVIG use using two controls. Methods : We evaluated 229 consecutive KD patients who were treated with 2 g/kg of IVIG at a single center. Those who had persistent fever >24 hours after IVIG infusion made up the 23 IVIG non-responders; the first control included a total 206 defervesced cases and the second control included 46 cases that were matched for age and pre-treatment fever duration to non-responders. Results : Demographic and clinical characteristics were similar in IVIG non-responders and responders at presentation. As for laboratory findings, the neutrophil differential, CRP, AST, ALT, and LDH were higher, and lymphocyte differential, total protein, albumin, platelet count, and total cholesterol were significantly lower in IVIG non-responders compared to responders by univariate analysis in both study designs. However in multivariate analysis, non-responders showed a significantly higher neutrophil differential (cutoff value, >77%, sensitivity 68.4% and specificity 79.5%) and lower cholesterol (<124 mg/dL, sensitivity 79% and specificity 70.5%). Whereas plasma albumin (<3.6 g/dL, sensitivity 73.7% and specificity 60%) was the sole laboratory parameter of non-responders in the second study design. Conclusion : Severity of inflammation in KD was reflected by higher or lower laboratory values at presentation. Because the multivariate analysis for these indices may be influenced by some confounding factors, including the numbers of patients of different ages and fever duration, other assessment modalities are needed for KD patients with the greatest risk of coronary artery lesions.

• Journal of Animal Environmental Science
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• v.8 no.1
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• pp.9-16
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• 2002

This study was conducted to develope the new solid separating system which can be efficiently and economically removed the solid parts in high pollutants concentration of pig slurry. The pollutants concentration, BOD$_{5}$ , COD and SS of the slurry used in this study was 15,990($\pm$2,389)mg/l, 20,004($\pm$5,512)mg/l and 26,486($\pm$5,935)mg/l, respectively. After removal of solid part in slurry, the pollutants concentration, BOD$_{5}$, COD and SS was change into 5,617($\pm$690)mg/l, 5,553($\pm$633)mg/land 1,456($\pm$341)mg/l, respectively in the Fixed biological membrane tank. The reduction of the pollutants concentration of suspend liquid through membrane will be allowed to greatly improve the water purification by an Activated sludge method. This separating system consisted of a temporary storage, a circulating tank and a Fixed Biological membrane tank. A temporary storage which has a draining system of screw type and an aeration device played a tremendous role in draining the solid by filled an aeration of 0.3 l/min. A Fixed Biological membrane tank of which a styrofoam filled in a 2/3 volume as a Biological media was fixed by a stainless steel net (pore size : 0.5mm) to separate the liquid layer of influx in them. The separating system efficiency factors were the speed of screw motor, cycle number of slurries in a circulating tank and moisture contents of solid effluent through the screw path. Although the pollutants concentration was very variable in temporary storage, the final concentration of $BOD_5$ and SS, except COD of the suspended liquid in a Fixed biological membrane were not different regardless of cycle number of a circulating tank. Moisture contents of effluent from temporary storage was 73% under the speed 1 ppm of screw motor and 62% under the 1/4rpm of it.

• International Journal of Highway Engineering
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• v.1 no.1
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• pp.107-119
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• 1999

The joints in the jointed concrete pavement provide a control against transverse or longitudinal cracking at slab, which may be caused by temperature or moisture variation during or after hydration. Without control of cracking, random cracks cause more serious distresses and result in structural or functional failure of pavement system. However, joints nay cause distresses due to its inherent weakness in structural integrity. Thus, the evaluation at joint is very important. and the joint-related distresses should be evaluated reasonably for economic rehabilitation. The purpose of this paper was to develop an evaluation system at joints of jointed concrete pavement using finite element analysis program, ILLI-SLAB, and nondestructive testing device. FWD. To develop an evaluation system for JCP, a sensitivity analysis was performed using ILLI-SLAB program with a selected variables which might affect fairly to on the performance of transverse joints. The most significant variables were selected from precise analysis. An evaluation charts were made for jointed concrete pavement by adopting the field FWD data. It was concluded that the variables which most significantly affect to pavement deflections are the modulus of subgrade reaction(K) and the modulus of dowel/concrete interaction(G), and limiting criteria on the performance of joints at JCP are 300pci. 500,000 lb/in. respectively. Using these variables and FWD test, a charts of load transfer ratio versus surface deflection at joints were made in order to evaluate the performance of JCP. Practically, Chungbu highway was evaluated by these evaluation charts and FWD field data for jointed concrete pavement. For Chungbu highway, only one joint showed smaller value than limiting criterion of the modulus of dowel/concrete interaction(G). The rest joints showed larger values than limiting criteria of the modulus of subgrade reaction(K) and the modulus of dowel/concrete interaction(G).

• Journal of Chest Surgery
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• v.40 no.5 s.274
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• pp.341-350
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• 2007

Background: We present here the early and midterm surgical results for infective endocarditis and we especially focus on the effect of aggressive reconstruction or root implantation after wide debridement. Material and Method: Between January 1995 and Jun 2006, we enrolled 79 adult infective endocarditis patients who underwent surgical treatment. There were 63 and 16 native and prosthetic valve endocarditis cases, respectively. They included 27 cases of culture negative endocarditis. With performing valve replacement or repair, 28 of the patient underwent a more aggressive surgical option, for example, aortic root replacement or reconstruction, or heart base reconstruction etc. Result: There were statistical relationships between the in-hospital mortality and staphylococcal infection, urgent-based operation and operation during the active phase of endocarditis. Wide debridement and aggressive reconstruction were not related to either the post operative mortality or the early morbidity. Culture negative endocarditis was not related to the postoperative mortality and morbidity. Conclusion: Physicians must pay attention to patients' medical treatment during the preoperative period of the infective endocarditis. If surgery is considered for treating infective endocarditis, it should be performed before the downhill course of the disease so that the surgical outcome is improved. Wide debridement and more aggressive reconstruction are also warranted.