• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.142-149
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• 2014

Purpose: There are 15 types of Glycogen storage disease (GSD) that have been identified, and GSD type Ia is the most common type. There are several studies of Korean GSD type Ia patients' long-term complications. The aim of this study to find out clinical symptoms and prognosis of GSD type Ia patients. Methods: We performed clinical, biochemical and genetic analysis retrospectively on five patients diagnosed with GSD type Ia in a Soonchunhyang University Hospital from July 2002 to July 2014. Results: All patients had hepatomegaly at diagnosis, and they were all confirmed to have fatty liver at abdomen USG. They had no developmental delay, but two of them had growth retardation. Elevated blood lactate, triglyceride, and uric acid levels can find out all patients, but only one patient had hypoglycemia. They are diagnosed with GSD through gene analysis, and by gene analysis, they have c.648G>T (homozygote, splicing mutation), c.122G>A/c.648G>T, c.248G>A/c.648G>T mutations. Treatment with three times meals, three times snacks and four to six times use of uncooked constarch for all patients. Following the progress, one of them resulted in hypothyroidism, other one had renal stones. A patient diagnosed at 16 years old had liver cirrhosis and started having hemodialysis for ESRD. Conclusion: GSD type Ia patients had hepatomegaly, hyperlipidemia, hyperuricemia, and lactacidemia. Therefore patients who have such these symptoms are recommended gene analysis. A patient diagnosed at 16-years-old had liver cirrhosis and ESRD in progress, early diagnosis and treatment are important for GSD type Ia patients.

• Journal of Chest Surgery
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• v.40 no.3 s.272
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• pp.180-192
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• 2007

Background: As determined from the recent investigations of discordant cardiac xenotransplantation, hyperacute rejection occurs mainly at the endothelial cells in donor microvascular systems, but this does not occur at cardiac valve leaflets or at medium-to-large caliber vessels. On the basis of this background, this study was performed to look into the biocompatibility for transplantation of a middle or large diameter xenogenic blood vessel by conducting xenogenic arterial transplantation with the carotid artery in a pig-to-goat model. Material and Method: The experimental group was composed of 10 pairs of pig-to-goat combinations. They were divided into each period of 1 week, and 1, 3, 6 and 12 months. Four carotid artery grafts obtained through collection of the bilateral carotid arteries from two pigs were preserved at $-70^{\circ}C$ without other treatment, and then they were transplanted into the bilateral carotid arteries of two goats. Doppler ultrasonography was done on a periodic basis after transplantation to evaluate the patency of the grafted blood vessel. At the ends of a predetermined period, the grafts were explanted from the goats and they underwent gross examination. Hematoxylin-eosin and Masson's trichrome staining were conducted. In addition, in order to examine the immunological rejection of the grafted xenogenic blood vessel, immunohistochemical staining was conducted with T-lymphocyte indicator and von Willebrand factor. Result: Two goats at the each one-week period and the one-year period died during the experimental period because of a reason unrelated to the experimental procedure, and the remaining 8 goats survived until the end of each experiment period. On Doppler ultrasonography, unilateral carotid artery occlusion was found in a goat, whose period was specified as 3 months, among the 8 survived goats. However, the vascular patency was maintained well and there was no graft that formed aneurysms in the other goats. On gross examination, the region of vascular anastomosis was preserved well, and calcification of the grafted blood vessel was not shown. Histologically, the endothelial cells of the graft disappeared one week after transplantation, and then there was progressive spread of the recipients' endothelial cells from the anastomotic site. The reendothelialization occurred over the whole graft at one month after transplantation. The neointimal thickening and adventitial inflammation became severe by 3 months after transplantation, but this lessened at 6 months and 12 months, respectively. The rate of CD3 positive cells was very low among the infiltrated inflammatory cells. Conclusion: The fresh-frozen xenogenic artery kept its patency without being greatly influenced by xenogenic immune reaction.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.746-752
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• 2007

Purpose : Total hydroperoxide (TH), free radical-mediated oxidation product can be used as a measure of free radical injury. The aim of the present study was to see if preterm newborns are at increased risk for oxidative stress compared with term newborns, and to determine whether oxidative stress during postnatal first 1 week is associated with clinical outcomes in preterm infants. Methods : Serum TH levels of preterm infants (n=39) were compared with those of term infants (n=24) on the postnatal day 1. Among the preterm infants, serum TH levels of uncomplicated group (n=23) were also compared with those of complicated group (n=16) who developed oxygen radical related diseases on the postnatal day 1 and 7. Retrospective analysis was performed to find out risk factors for oxygen radical injuries based on birth history, laboratory data, neuroimaging findings and clinical progress in two preterm groups. Results : Serum TH levels on postnatal day 1 were higher in the preterm infant group than the term infant group. Serum TH levels on postnatal day 1 in the complicated preterm infant group were significantly higher compared with uncomplicated group, but there was no significant difference in serum TH levels on postnatal day 7. Also, there was no significant difference in serum TH levels between uncomplicated preterm infants and term infants. Serum TH level on postnatal day 1 was independently associated with higher morbidity after adjusting for gestational age, Apgar score (5 min), arterial blood gas analysis. Conclusion : Complicated preterm newborns are at increased risk for oxidative stress compared with uncomplicated newborns and term newborns. Oxidative injury during the prenatal or postnatal day 1 is associated with adverse outcomes in preterm infants. Elevated TH levels on postnatal day 1 may have a value to predict clinical outcomes in preterm infants.

• Journal of Chest Surgery
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• v.34 no.12
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• pp.901-908
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• 2001

Background : Coronary artery bypass graft(CABG) in patients with advanced left ventricular dysfunction has often been regarded as having high mortality rate, despite the great improvement in operative result of CABG. With recent advances in surgical technique and myocardial protection, surgical revascularization improved the symptom and long-term survival of these high risk patients more than the medical conservative treatment. Material and Methold : Clinical data of 31(4.1％) patients with preoperative ejection fraction less than 30% among 864 CABGs performed between January 1995 and March 1999 were retrospectively analyzed and pre- and postoperative changes of the ejection fraction on echocardiography were analyzed. There were 26 men and 5 women. The mean age was 60.7 years(range 41 to 72 years). History of myocardial infarction(30 cases, 98％) was the most common preoperative risk factor. There were seven irreversible myocardial infarction on thallium scan. Most patients had triple vessel diseases(26 cases, 84％) and first degree of Rentrop classification(16 cases, 52％) on coronary angiography. The mean number of distal anastomosis during CABG was per patient was 4.9${\pm}$0.8 sites in each patient. In addition to long saphenous veins, the internal mammary artery was used in 20 patients. Total bypass time was 244.7${\pm}$3.7 minutes(range, 117 to 567 minutes), and mean aortic cross-clamp time was 77.9 ${\pm}$ 1.6 minutes(range, 30 to 178 minutes). There were five other reparative procedures such as two left ventricular aneurysrmectomy, two mitral repair, and one aortic valve replacement. There were twelve postoperative complications such as three cardiac arrhythmia, two bleeding(re-operation), one delayed sternal closure, eleven usage of intra-aortic balloon counterpulsation for low cardiac output. Two patients died, postoperative mortality was 6.5% . Twenty-nine patients were relieved of chest pain and left ventricular ejection fraction after operation was significantly higher(38.5${\pm}$11.6%, p 0.001) as compared with preoperative left ventricular ejection fraction(25.3${\pm}$2.3%). The follow up period of out patient was 25. 3 months. Conclusion: In patients with coronary artery disease and advanced left ventricular dysfunction, coronary artery bypass grafting can be performed relatively safely with improvement in left ventricular function, but it will be necessary to study long term results.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.158-165
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• 2002

Purpose: We designed this retrospective study to establish the incidence of diagnosic delay in children diagnosed with acute appendicitis and to identify associated factors with delayed diagnosis and its impact on the clinical course. Methods: All cases of children under 15 years of age who underwent appendectomy from 1996 to 2001 at Gangneung Asan Hospital were reviewed. We reviewed signs and symptoms, type of health professional first contacted, the advice given by the health professional and a history of appendicitis in first degree relatives. Diagnostic period is the time elapsed between first complaints and definitive diagnosis. Delay was defined as diagnostic period exceeded the 48 hours. Postoperative course and complications were also reviewed. Results: Incidence of diagnostic delay differed by whether diarrhea and fecalith on X-ray were present. Also children whose parents were advised to observe them at home were more likely to have a diagnostic delay. In almost half of the cases in delayed group, initial diagnosis was not acute appendicitis but gastroenteritis. The perforation rate in non-delayed group was 22%, whereas 87% in delayed group. The delayed group showed a higher number of postoperative complication and a longer hospitalization period. Conclusions: Diarrhea with abdominal pain and fever in children should not be dismissed as gastroenteritis, respiratory infections or other common disorders. Our study suggests that physicians have a responsibility to prevent diagnostic delay and resultant perforation of acute appendicitis in children by having a high index of suspicion about acute appendicitis.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.64-68
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• 2005

Purpose : Renal abscess is very rare in children and its diagnosis is difficult because symptoms are often nonspecific. In previous studies, on]y 15% to 25% of patients were reported to be diagnosed at the time of admission. Early diagnosis and treatment are important be cause mortality rate correlates positively with the time of diagnosis. The purpose of this study is to clarify the clinical features of children with renal abscess and to investigate the possible indicators of this disease for early diagnosis and Proper treatment. Methods : Twelve children diagnosed with renal abscess from Jan. 1996 to Jul. 2004 were included. The age of patients ranged from S months to 15 years. We retrospectively analyzed the demographics of patients, their symptoms, predisposing factors, diagnostic methods and causative organisms and the treatment modalities. Results : Fever was the most common manifestation, Five children(42%) had vesicoureteral reflux. Renal ultrasonography and computerized tornography were the most frequently used imaging tools to detect renal abscess. Gram negative bacteria were isolated in 7 patients and Staphylococcus aureus grew in 2 patients. All patients received intravenous antibiotics and 4 patients underwent aspiration or drainage of renal abscess. The average admission duration was 30 days. Conclusion : Renal abscess should be included in the differential diagnosis of prolonged fever in children, especially when flank pain is combined. For early diagnosis and a better prognosis, patients should be promptly investigated with ultrasonography or computerized tomography.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.43-49
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• 2018

Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.

• Journal of Internet Computing and Services
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• v.25 no.1
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• pp.99-107
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• 2024

Along with economic growth and industrial development, there is an increasing demand for various electronic components and device production of semiconductor, SMT component, and electrical battery products. However, these products may contain foreign substances coming from manufacturing process such as iron, aluminum, plastic and so on, which could lead to serious problems or malfunctioning of the product, and fire on the electric vehicle. To solve these problems, it is necessary to determine whether there are foreign materials inside the product, and may tests have been done by means of non-destructive testing methodology such as ultrasound ot X-ray. Nevertheless, there are technical challenges and limitation in acquiring X-ray images and determining the presence of foreign materials. In particular Small-sized or low-density foreign materials may not be visible even when X-ray equipment is used, and noise can also make it difficult to detect foreign objects. Moreover, in order to meet the manufacturing speed requirement, the x-ray acquisition time should be reduced, which can result in the very low signal- to-noise ratio(SNR) lowering the foreign material detection accuracy. Therefore, in this paper, we propose a five-step approach to overcome the limitations of low resolution, which make it challenging to detect foreign substances. Firstly, global contrast of X-ray images are increased through histogram stretching methodology. Second, to strengthen the high frequency signal and local contrast, we applied local contrast enhancement technique. Third, to improve the edge clearness, Unsharp masking is applied to enhance edges, making objects more visible. Forth, the super-resolution method of the Residual Dense Block (RDB) is used for noise reduction and image enhancement. Last, the Yolov5 algorithm is employed to train and detect foreign objects after learning. Using the proposed method in this study, experimental results show an improvement of more than 10% in performance metrics such as precision compared to low-density images.

• Journal of Embryo Transfer
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• v.26 no.1
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• pp.19-25
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• 2011

This study was carried out to evaluate the effect of early pregnant cow as donor for Ovum Pick-Up (OPU) derived oocyte aspiration and embryo production in Holstein heifers. Four non-pregnant and 2 pregnant Holstein heifers were used as donor and then carried out total 17 OPU session for 10 weeks (2 times per week). Recovered cumulus-oocyte-complexes (COCs) were classified into 4 grade by oocyte cytoplasm and cumulus cells and matured in vitro in TCM-199 supplemented with 10% FBS, 10 mg/ml FSH and 1 mg/ml estradiol in 5% $CO_2$ and over 99% humidity for 24 h. After 24 h co-incubation with post-thaw sperm, the presumed zygotes were cultured in CR1aa medium with 4 mg/ml BSA for 3 days and then changed CR1aa medium with 10% of FBS for another 3~4 days. The Mean number of aspirated follicles and collected oocytes in the early stage pregnant and non-pregnant heifers were $13.0{\pm}4.3$ and $10.6{\pm}3.9$, $5.4{\pm}3.4$ and $7.7{\pm}3.6$ per session, respectively. Rate of collected oocyte from aspirated follicles were 59.2% and 50.5%, respectively. The average number of good quality oocytes (Grade I and II) in the early stage pregnant and non-pregnant heifers was $3.7{\pm}2.7$ and $4.9{\pm}2.6$ (Mean${\pm}$SD). Cleavage and blastocyst developmental rates in Grade I and II were 22.2% and 25.5%, and then $1.7{\pm}0.9$ and $1.4{\pm}1.1$ blastocyst per session, respectively. In conclusion, OPU technology can be used in early stage pregnant and non-pregnant heifers without any problem and so applied OPU derived embryo production to maximize the ability of genetically valuable females.