• Korean Journal of Heritage: History & Science
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• v.56 no.1
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• pp.100-116
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• 2023

The Sinamsa Temple was built in the late Goryeo Dynasty and a gilt-bronze seated Buddha is enshrined in Geungnakjeon hall in the precinct. Various damages occurred in the gilt layer of the Buddha, such as peeling of the gilt layer and deteriorating gloss. In the study, the conservation conditions of the inside and outside on the statue were accurately investigated, and the making technique was interpreted through the material characteristics and non-destructive diagnosis of the statue. As a result, it is estimated that gold-gilding layer is pure gold, coloration pigment of black is carbon, green is malachite, atacamite and verdigris, red is red lead and cinnabar, respectively. In the deterioration evaluation, peeling, cracking, break out and exfoliation of the gilt layer are confirmed as damages, but the conservation condition is relatively wholesome. However, the gloss of the gilt layer is calculated to be wider in the poorer part than the maintenance part. The ultrasonic velocity of the statue was calculated to be 1,230 to 3,987 (mean 2,608) m/s and showed a relatively wide range. In infrared thermography, peeling was not confirmed, and no special bonding marks were found. In endoscope, some biological damage and corrosion were observed on the surface of the internal metal, and sealed artifacts were identified. Manufacturing technique based on the study, it is considered that the gilt-bronze seated Buddha was cast at once, and the mold was inverted to inject molten metal.

• Journal of Chest Surgery
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• v.34 no.9
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• pp.672-679
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• 2001

Background: Surgical correction of partial anomalous pulmonary venous connection to the superior vena cava has been associated with postoperative venous obstruction and sinus node dysfunction. In this paper we describe our current approach and its short-term results. Material and Method: Between April 1999 and January 2000, 5 consecutive patients, ranging from 2 months to 66 years old, underwent corrective operation for partial anomalous pulmonary venous connection to the superior vena cava at Sejong General Hospital and Daegu Catholic University Medical Center. Surgical correction involved diversion of the pulmonary venous drainage to the left atrium using a right atrial flap(2 patients) or prosthetic patch(3 patients) with division of the superior vena cava superior to the restore site of the pulmonary veins and reimplantation on the right atrial appendage to restore systemic venous drainage. Result: All patients were discharged between postoperative day 9 and 15 without complications. One Russian boy returned to his country, therefore, he was lost to follow-up after discharge. Remaining 4 patients were asymptomatic and in normal regular sinus rhythm at a mean follow-up of 17.75$\pm$4.27 months. Follow-up echocardiographic study (range, 12 to 24 months) revealed no incidence of narrowing of the venous pathways or of residual shunt. Conclusion: Our current approach is relatively simple and reproducible in achieving unobstructive pulmonay venous and SVC pathways. By avoiding incision across the cavoatrial junction, surgical injury to the sinus node and its artery may be minimized. The presented surgical technique can be safely and effectively applied to the selected patients.

• 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 The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.87-94
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• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.

• 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.