• Clinical and Experimental Pediatrics
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• 제58권10호
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of Korean Neurosurgical Society
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• 제48권4호
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• pp.335-341
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• 2010

Objective : Anterior cervical corpectomy with fusion (ACF) or laminoplasty may be associated with substantial number of complications for treating multilevel cervical ossification of the posterior longitudinal ligament (OPLL) with significant cord compression. For more safe decompression and stabilization in multilevel cervical OPLL with prominent cord compression, we propose circumferential cervical surgery (selective ACF and laminoplasty) based on our favorable experience. Methods : Twelve patients with cervical myelopathy underwent circumferential cervical surgery and all patients showed multilevel OPLL with Signal change of the spinal cord on magnetic resonance imaging (MRI). A retrospective review of clinical, radiological. and surgical data was conducted. Results : There were 9 men and 3 women with mean age of 56.7 years and a mean follow up period of 15.6 months. The average corpectomy level was 1.16 and laminoplasty level was 4.58. The average Japanese Orthopedic Association score for recovery was 5.1 points and good clinical results were obtained in 11 patients (92%) (p < 0.05). The average space available for the cord improved from 58.2% to 87.9% and the average Cobb's angle changed from 7.63 to 12.27 at 6 months after operation without failure of fusion (p < 0.05). Average operation time was 8.36 hours, with an estimated blood loss of 760 mL and duration of bed rest of 2.0 days. There were no incidences of significant surgical complications, including wound infection. Conclusion : Although the current study examined a small sample with relatively short-term follow-up periods, our study results demonstrate that circumferential cervical surgery is considered favorable for safety and effectiveness in multilevel OPLL with prominent cord compression.

• Journal of Gastric Cancer
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• 제18권2호
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• pp.172-181
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• 2018

Purpose: This study aimed to evaluate the surgical outcomes and investigate the feasibility of reduced-port laparoscopic gastrectomy using learning curve analysis in a small-volume center. Materials and Methods: We reviewed 269 patients who underwent laparoscopic distal gastrectomy (LDG) for gastric carcinoma between 2012 and 2017. Among them, 159 patients underwent reduced-port laparoscopic gastrectomy. The cumulative sum technique was used for quantitative assessment of the learning curve. Results: There were no statistically significant differences in the baseline characteristics of patients who underwent conventional and reduced-port LDG, and the operative time did not significantly differ between the groups. However, the amount of intraoperative bleeding was significantly lower in the reduced-port laparoscopic gastrectomy group (56.3 vs. 48.2 mL; P<0.001). There were no significant differences between the groups in terms of the first flatus time or length of hospital stay. Neither the incidence nor the severity of the complications significantly differed between the groups. The slope of the cumulative sum curve indicates the trend of learning performance. After 33 operations, the slope gently stabilized, which was regarded as the breakpoint of the learning curve. Conclusions: The surgical outcomes of reduced-port laparoscopic gastrectomy were comparable to those of conventional laparoscopic gastrectomy, suggesting that transition from conventional to reduced-port laparoscopic gastrectomy is feasible and safe, with a relatively short learning curve, in a small-volume center.

• Asian Pacific Journal of Cancer Prevention
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• 제16권17호
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• pp.7935-7941
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• 2015

The aim of this study was to implement massive parallel sequencing (MPS) technology in clinical genetics testing. We developed and tested an amplicon-based method for resequencing the BRCA1 and BRCA2 genes on an Illumina MiSeq to identify disease-causing mutations in patients with hereditary breast or ovarian cancer (HBOC). The coding regions of BRCA1 and BRCA2 were resequenced in 96 HBOC patient DNA samples obtained from different sample types: peripheral blood leukocytes, whole blood drops dried on paper, and buccal wash epithelia. A total of 16 random DNA samples were characterized using standard Sanger sequencing and applied to optimize the variant calling process and evaluate the accuracy of the MPS-method. The best bioinformatics workflow included the filtration of variants using GATK with the following cut-offs: variant frequency >14%, coverage ($>25{\times}$) and presence in both the forward and reverse reads. The MPS method had 100% sensitivity and 94.4% specificity. Similar accuracy levels were achieved for DNA obtained from the different sample types. The workflow presented herein requires low amounts of DNA samples (170 ng) and is cost-effective due to the elimination of DNA and PCR product normalization steps.

• Asian Pacific Journal of Cancer Prevention
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• 제16권4호
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• pp.1355-1360
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• 2015

Background: The number of cancer survivors is increasing globally and recently, higher rates of comorbidities in cancer survivors have been reported. However, no studies have investigated whether cancer survivors have a higher risk of chronic kidney disease (CKD). Accordingly, our study evaluated the association between cancer survivors and the risk of CKD using the 2010-2012 Korean National Health and Nutrition Examination Survey. Materials and Methods: A total of 11,407 participants aged 40 years and over were categorized into two groups according to cancer experience. Multiple variables were compared and the odds ratios (ORs) for CKD prevalence were calculated using a weighted logistic regression analysis between the two groups. Results: Cancer survivors were older than were those in the non-cancer group, on average, the percentages of glomerular filtration rate(GFR) lower than $60mL/min/1.73m^2$, proteinuria, and CKD were significantly higher in cancer survivors when compared to controls. Weighted logistic regression analyses demonstrated that cancer survivors had a higher risk for CKD after adjusting for multiple variables (OR (95% confidence interval), 2.88 (1.48-5.59)). Conclusions: Our study demonstrated a possible association between CKD and cancer survival in Korean adults. Identifying and correcting risk factors for cancer survivors would positively affect prevention of CKD and result in a better cancer prognosis.

• Childhood Kidney Diseases
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• 제22권2호
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• pp.52-57
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• 2018

Purpose: Rhabdomyolysis is a metabolic disorder in which the content of damaged muscle cells is released into plasma. Its manifestations include asymptomatic, myalgia, gross hematuria, and complications of acute kidney injury. Because of limited data on rhabdomyolysis in children, we performed this study to determine clinical characteristics of rhabdomyolysis in children. Methods: We retrospectively reviewed the records of patients with rhabdomyolysis who were treated at the Pusan National University Children's hospital from January 2011 to July 2016. The diagnostic criteria were serum myoglobin level of ${\geq}80ng/mL$, exclusive of acute myocardial injury, cardiac arrest, and brain damage. Results: Forty-five patients were enrolled; mean age, $116{\pm}68$ months. Of these, 35 were boys and 10 were girls. Twenty-six patients experienced myalgia and 12 patients showed gross hematuria. Among these, seven patients initially had both myalgia and gross hematuria. The most common causes of rhabdomyolysis were infection, physical exertion, prolonged seizures, metabolic abnormalities, and drug addiction. Acute kidney injury (AKI) was the most common complication, followed by disseminated intravascular coagulation. Thirty-seven patients improved with sufficient fluid supply but two patients underwent hemodialysis due to deterioration of kidney function. Gross hematuria, positive occult blood test, and positive urine protein were more common in patients with AKI than in those without AKI. Conclusions: In children, infection was the most common cause of rhabdomyolysis. Most patients recovered by sufficient fluid therapy. However, in severe cases, especially in patients with underlying kidney disease, hemodialysis may be necessary in the present study.

• 한국조경학회지
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• 제34권1호
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• pp.107-119
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• 2006

The Korea Water Resources Corporation(KOWACO) proposed bidding for an alternative design for Hwabuk Multi-purpose Dam in March of 2004. The site is located in Hakseong-ri, Goro-myeon, Gunwi-gun, Gyeongsangbuk-do and has adrainage area of $87.52km^2$. The purpose of this project is to establish an environmentally friendly plan for minimizing the damage that was caused by the construction of the Hwabuk Multi-purpose Dam. The design principle of KOWACO was the restoration of the natural environment, a harmonious landscape, and the creation of a space of regional and local culture. The basic concept of this project involves an ecological-restoration axis and a functional-connection axis. The site is divided into four spaces: (1) the space of memory and symbol, (2) the space of nature and ecology, (3) the space of regional and local culture, and (4) the space of the regional economy. There are four sub-spaces in the space of memory and symbol: the track forest, the time forest, the memory room, and the sun plaza. There are three sub-spaces in the space of nature and ecology: the habitat of aquatic birds, the wind forest, and the eco-corridor. There are five themed parks in the space of regional and local culture: the culture and relic room, the wildflower garden, the ecological pond, the insect observation park, and the pyogo maze. There are three areas in the space of the regional economy: the forest pension, the waterside pension, and the community center, as Dungdungi village was reorganized to serve as a lodging complex. These themed parks, working together, can offer an effective space for nature, culture, rest, and experience.

• 대한통합의학회지
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• 제7권1호
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• pp.81-88
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• 2019

Purpose : The purpose of this study was to examine if there is any correlation between pelvic tilt angle and trunk motion and trunk extensor during trunk forward flexion and to measure trunk motion, onset time of trunk motion, and onset time of trunk extensor activation. Methods : The subjects of this study were 42 healthy adults. The subjects had no back pain due to neurological disease and no experience of back surgery. After pelvic tilt angle was measured, each trunk forward flexion was performed three times. Trunk motion and onset time of trunk motion were measured using Myomotion. Four sensors were used, with one located at the upper thoracic (below $C_7$), the lower thoracic ($T_{12}-L_1$), the sacrum ($S_1$), and at the center of the anterior femur. Onset time of trunk extensors (spinalis, longissimus, gluteus medius, gluteus maximus, biceps femoris, and gastrocnemius) activation was measured using a wireless surface EMG. The EMG amplitude was normalized by using the reference voluntary contraction (RVC). The statistical significance of the results were evaluated using Pearson's correlation test. Results : The correlation between pelvic tilt angle and lumbar motion, onset time of pelvis motion, and onset time of gluteus medius activation was statistically significant in a positive direction (p<.05). The correlation between pelvic tilt angle with pelvis motion, onset time of lumbar motion, and onset time of longissimus activation showed a statistically significant negative correlation (p<.05). Conclusion : The study results provide a significant contribution to our understanding of the lumbar load at the initial stage of trunk flexion. Therefore, it may be possible to provide basic data for evaluation and treatment, such as orthodontic treatment for alignment of the spine and back pain. In addition, it is necessary to focus on normal exercise pattern reeducation as well as pelvic correction during exercise in daily life or in industrial fields.

• Childhood Kidney Diseases
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• 제3권2호
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• pp.123-129
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• 1999

목적 : 연쇄상구균 감염후 급성사구체신염은 항원-항체계가 작용하여 증식병변과 염증반응을 가져오는 신질환을 가리킨다. 임상적으로 부종, 고혈압의 전신증상과 함께 육안적 혹은 현미경적 혈뇨를 보이며 대개 경한 단백뇨가 동반되는 것으로 알려져 있으나 소수의 환자에서 신증후군이 동반될 수 있고 이러한 경우 질환의 경과 및 예후 또한 전형적인 사구체신염과 다르게 나타날 수 있다. 따라서 본 연구에서는 신증후군을 동반한 연쇄상구균 감염후 급성사구체신염의 임상적인 양상 및 조직학적인 특성을 관찰하여 초기 단백뇨의 정도가 본질환의 예후에 미치는 영향을 파악하여 치료 및 경과 예측에 도움이 되고자 하였다. 대상 및 방법 : 1989년 3월부터 1999년 2월까지 연쇄상구균 감염후 급성사구체신염으로 진단된 71명의 환아를 대상으로 하여 신증후군을 동반하지 않은 환아군(A군)과 동반한 환아군(B군)으로 구분하여 두 군간의 임상양상의 차이를 비교하였다. 결과 : 대상기간 중 연쇄상구균 감염후 급성사구체신염으로 진단된 환아는 총 71명으로 신증후군이 동반되지 않은 환자(A군)는 64례(90.1%), 신증후군이 동반된 환자(B군)가 7례(9.9%)였고, 남녀비는 A군이 1:1, B군이 1.9:1, 발병평균연령은 A군이 $8.9{\pm}2.6$세, B군이 $8.8{\pm}2.6$세였다. 진단 당시 주소로 육안적 혈뇨는 A군에서 33/64명 (51.6%), B군에서 7/7명(100%)있었고, 전신부종은 A군에서 23/64명(35.9%), B군에서 2/7명 (28.6%), 안면부종은 A군에서 17/64명(26.6%), B군에서 1/7명(14.3%), 호흡곤란은 A군에서 1/64명(1.6%), B군에서 0/7명(0%)였다. 검사실소견상 백혈구수, 헤모글로빈, CRP, ASO, BUN, 크레아티닌, 사구체여과율, $C_3,\;C_4$는 두 군간의 유의한 차이가 없었다. 선행감염으로는 A군의 경우 급성인두편도염 39/64명(60.9%), 피부감염 0/64명(0%), 경부임파선염 1/64명 (1.6%), 알지못하는 경우가 24/64명(37.5%), B군의 경우 급성 인두편도염 4/7명(57.1%), 알지 못하는 경우가 3/7명(42.9%)이었다. 단백뇨의 지속기간은 A군이 $1.95{\pm}2.27$주, B군이 $13.3{\pm}21.1$주 통계적으로 유의한 차이(P=0.013)를 나타내었고, 현미경적혈뇨의 지속기간은 A군이 $4.5{\pm}5.8$주, B군이 $6.6{\pm}5.0$주(P=0.42), $C_3$감소의 지속기간은 A군 (33/38명)이 $1.9{\pm}2.9$주, B군(5/38명)이 $7.3{\pm}5.0$주(P=0.04)로 신증후군을 동반하는 경우 단백뇨, $C_3$의 감소 기간이 유의하게 증가하였고 전례에서 단백뇨가 모두 소실되었으며 신부전으로 진행되거나 사망한 례는 없었다. 신조직 검사는 15례(21.1%)에서 시행하였고 적응증으로는 신증후군, 신기능의 저하, 고혈압이 동반되었던 경우로 하였다. 결론 : 연쇄상구균 감염후 급성사구체신염 환아에서 신증후군을 동반하는 경우 질환의 경과(단백뇨, $C_3$감소)가 유의하게 길어지며, 이외의 임상양상에 있어서는 신증후군의 유무에 따른 차이가 없었고, 전례에서 단백뇨가 모두 소실되었으며 신부전으로 진행된 례는 없는 것으로 보아 신증후군의 동반이 이 질환의 최종적인 예후에는 별다른 영향을 나타내지 못하는 것으로 생각되었다.

• International Journal of Quality Innovation
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• 제8권3호
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• pp.123-155
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• 2007

This research treated the secondary school teachers as the research scale and the research targets included the following: secondary school (employ unit), center of teacher education, and qualified teachers. In terms of these three groups of people, the topics of exploration focused on the perception of suitable teachers, perception of evaluation standard during the process of teacher examination and the relationship among teacher examination systems. The research was managed by two phases: the first phase referred to literature reorganization, expert interview, the qualities and conditions of suitable teachers, important evaluation standard during the process of teacher examination and teacher examination system used; the second phase included questionnaire survey, employ school acquisition, the perception of centers of teacher education and qualified teachers toward the suitability condition and examination evaluation standard in the first phase and teacher examination system used. This research found out that as to the perception of suitable teachers, through data collection, there were six factors reorganized. The levels of their importance were as follows: education devotion, teaching capacity, class management, capacity to guide special students, capacity to communicate with the parents and the will to undertake administrative works. Noticeably, employee unit and centers of teacher education apparently valued class management more, compared with trained teachers; as to evaluation standard of examination, the analytical result found out that the perception of three groups were different in terms of the views toward educational works, written examination data, candidate's age, club experience at school and capacity to use multimedia support teaching materials. This research further proposed six suggestions for centers of teacher education and trained teachers: (l) employ schools considerably valued educational devotion; (2) trained teachers tended to neglect the importance of class management; (3) employee unit considerably cared about the new teachers' competence to use multimedia support teaching materials; however, trained teachers did not have the same view; (4) employee unit considerably cared about new teachers' views toward educational works as well as the candidates' ages; (5) generally speaking, trained teachers neglected the importance of club experience at school; (6) the data revealed that written examination data was not relatively important in terms of teacher examination.