• Journal of Genetic Medicine
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• v.4 no.1
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• pp.53-63
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• 2007

Purpose : Tandem mass spectrometry (MS/MS) is effective screening test for inherited metabolic diseases. In this study, we estimate potential costs and benefits of using tandem mass spectrometry (MS/MS) to screen new borns for inherited metabolic diseases (phenylketonuria, BH4 deficiency, citrullinemia, maple syrup urine disease, propionic aciduria, isovaleric aciduria, glutaric aciduria type 1, LCHAD deficiency) in Korea. Methods : From April 2001 to March 2004, 79,179 new borns were screened for amino acid disorders, organic acid disorders, and fatty acid oxidative disorders. Twenty-eight new borns were diagnosed with one of the metabolic disorder and the collective estimated prevalence amounted to 1 in 2,800 with a sensitivity of 97.67%, a specificity of 99.28%, a recall rate of 0.05%, and a positive preditive value of 6.38%. We calculated and compared the total costs in case when neonatal screening on pheny lketonuria, BH4 deficiency, citrullinemia, maple syrup urine disease, propionic aciduria, isovaleric aciduria, glutaric aciduria type 1, LCHAD deficiency is implemented, and when not. Results : If the neonatal screening on pheny lketonuria, BH4 deficiency, citrullinemia, maple syrup urine disease, propionic aciduria, isovaleric aciduria, glutaric aciduria type 1, LCHAD deficiency is implemented, total benefits far exceed costs at a ratio of 1.40:1. Conclusion : Although, this study only concerns the monetary aspects of the neonatal screening, tandem mass spcetrometry for neonatal screening is cost-effective compared with not screening. The study appears to support the introduction of tandem mass spectrometry into a Korea neonatal screening programme for inherited metabolic diseases.

• Analytical Science and Technology
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• v.28 no.6
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• pp.460-466
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• 2015

An isotope dilution liquid chromatography tandem mass spectrometry was developed as a primary method for the quantitative analysis of cholesterol in infant formula. Cholesterol-d₄ was used as an internal standard and spiked into the infant formula sample. In order to release cholesterol out of cholesteryl ester, which is cholesterol bound to fatty acids in infant formula, saponification was carried out. Saponification conditions were optimized with heating temperature, reaction time and the concentration of KOH. The optimum conditions were as follows; heating temperature was 70 ℃, reaction time was 180 min and the concentration of KOH was 0.8 mL of 8 M KOH for about 0.1 g infant formula sample. Extraction of cholesterol out of sample solution was carried out with hexane uisng liquid-liquid extraction. Chromatographic analysis was carried out using Phenomenex Kinetex C₁₈ column. Mobile phase was 0.1% acetic acid in methanol/water (v/v, 99/1) and flow rate was 0.3 mL/min. Cholesterol and cholesterol-d₄ were monitored at mass transfer m/z 369/259 and 373/263 respectively. Reproducibility of the method was evaluated to be 0.23% of the measurement result. The expanded uncertainty of the measurement result of cholesterol in infant formula was approximately 1.9% at a 95% confidence level. NIST standard reference material having certified values of cholesterol in infant formula, was analyzed in order to verify this method. The ID-LC/MS/MS results were well agreed with the certified values of NIST SRM within the uncertainty.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.9
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• pp.1318-1323
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• 2008

Corn distiller's dried grains with solubles (DDGS) is a completely new feed ingredient in the Korean feed market. There is an ever increasing need for the Korean feed industry to import and make the best of it as a high protein and high energy feed ingredient. A layer feeding trial was conducted for 10 weeks to investigate the effects of addition of light-colored DDGS to layer diets on laying performance, egg qualities and yolk fatty acid composition. Also, the economics of using DDGS in the Korean situation was analyzed. Nine hundred Hy-line Brown layers, 24 weeks of age, were employed in a feeding trial consisting of four dietary treatments (0, 10, 15, and 20% DDGS), and five replicates per treatment. All experimental diets were prepared as iso-protein (17%) and iso-calorie (TMEn 2,780 kcal/kg). The use of DDGS up to 20% in layer diets did not exert any influence on feed intake, laying rate, total egg mass, mean egg weight and feed conversion ratio (p>0.05). The color and breaking strength of eggshell, as well as the albumin height and Haugh unit were not affected by the addition of DDGS up to 20% in the diet. The yolk color was significantly increased by DDGS supplementation (p<0.05). As the DDGS level increased, the oleic acid content decreased, and the linoleic acid increased (p<0.05) in egg yolk. The degree of saturation of yolk fatty acids was not affected by DDGS supplementation. The inclusion of light-colored DDGS up to 20% in layer diets resulted in a decrease of feed cost per kg without any undesirable effect on laying performance. In conclusion, the light-colored DDGS (L* 56.65) could be used up to 20% in layer diets without any harmful effect on laying performance, and possibly provide economic benefits to the Korean poultry industry.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.35-42
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• 2018

Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

• Analytical Science and Technology
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• v.29 no.1
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• pp.49-55
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• 2016

A ketone body (acetoacetic acid, β-hydroxybutyric acid, and acetone) increases from blood or urine when bio-energy dependence pays more fatty acid than glucose. However, in case oxidation of fat is greater than the capacity of the citric acid cycle the fatty acid oxidation is made from acetoacetyl CoA to acetoacetate then, again form β-hydroxyburytic acid to acetone, the diffusion take place into the blood. Enzymes that oxidize ketone body in the brain and nerve tissue blood ketone dody is increased during prolonged fasting, brain used it as energy. In this study, we developed the rapid two step derivatization method for sensitive detection of the ketone body by GC-MS/SIM. The plasma was deproteinized and then the hydroxy and carboxyl groups of ketone body are subjected to extraction and drying then, keto-group were derivatized with hydoxylamine at 60℃ for 30 min for oximation. Then it was trimetyl-silylated with BSTFA at 80℃ for 30 min and analyzed using a GC-MS. The linear ranges were in between 0.001 μg/mL and 250 μg/mL for β-hydroxy butyrate, and acetoacetate. The method detection limits were below 0.1 pg over each target compound determined. The mean recoveries (%) of target compounds were ranged from 88.2 % to 92.3 % at 1 µg/mL, from 89.5 % to 94.8 % at 10 μg/mL, with RSD of 6.3-9.4 %. This method could be applied to quantification of ketone bodies which are seen in the keto-acidosis in children and adults from a variety of diseases that cause ketones in the blood and urine.

• Journal of Applied Biological Chemistry
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• v.66
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• pp.467-476
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• 2023

Schisandra chinensis (S. chinensis) is a deciduous broad-leaved cave plant belonging to the Schisandraceae family and is widely distributed in East Asia including Korea, Japan, China, and Taiwan. It has been reported that the main components contained in S. chinensis include lignan compounds and triterpenoid compounds. To distinguish the characteristics of S. chinensis by production region of Korea, a discriminant was established by performing metabolite profiling and principal component analysis, a multivariate statistical analysis technique. As a result, 16 types of triterpenoids, 9 types of lignan, and 1 type each of flavonoid, phenylpropanoid, and fatty acid were identified. In addition, through multivariate statistical analysis, it was confirmed that the four groups in Danyang, Moongyeong, Geochang, and Pyeongchang were divided, by applying the s-plot model of orthogonal partial least squares discriminant analysis. Biomarkers were identified: lanostane, cycloartane, schiartane triterpenoid, and dibenzocyclo-octadiene lignan were identified as chemical markers, respectively.

• Korean Journal of Food Science and Technology
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• v.26 no.6
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• pp.665-669
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• 1994

A rapid gas chromatographic (GC) profiling method was applied to dairy products (milk and cheeses) for the simultaneous analysis of volatile and nonvolatile organic acids. Cheese samples were first made into aqueous samples by dissolving in water. The aqueous samples were then extracted with organic solvents after the acidification and NaCI saturation. The organic layers (diethyl ether : hexane= 1 : 1) were extracted with $NaHCO_3$ saturated solution with subsequent solid-phase extraction of the aqueous phases using Chromosorb P column/diethyl ether followed by triethylamine treatment. The resulting triethylammonium salts of acids were directly converted into volatile tert.-butyldimethylsilyl derivatives, which were analyzed by dual-capillary column GC, and GC-mass spectrometry. From milk and four cheese samples studied, 31 organic acids including 21 fatty acids and other hydroxy and dioic acids were tentatively identified. The amounts of the fatty acids were different among the kinds of cheese and thus the simplified retention index (RI) spectra of organic acids were useful for the visual pattern recognition of each sample, when the Direct Comparision method between cheese and a blind sample were attempted, it was quickly recognized to be a gouda cheese with the 999 ppt match quality value.

• Nutritional Sciences
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• v.9 no.2
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• pp.124-130
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• 2006

To examine the effects of alcohol consumption on body fluid restoration and fat mobilization following exercise induced dehydration, nine healthy collegiate men ($24{\pm}2yrs,\;177{\pm}5cm,\;72{\pm}8kg,\;10.5{\pm}2.3%$ body fat) underwent three experiments. In each experiment, subjects ran on a treadmill to reduce individual body mass to $2.2{\pm}0.1%$ and consumed one of three beverages containing 0, 4, or 8% alcohol over 60 min followed by 4 hr of resting recovery. They consumed approximately 150% of weight loss $(2053{\pm}204,\;2091{\pm}149,\;and\;1943{\pm}295mL)$ and content of alcohol was $9.9{\pm}1.0(0%),\;71.9{\pm}5.1(4%)$, and $132.2{\pm}20.1g$ (8% trial). Body weight, urine volume and samples, blood samples, and thirst sensation were measured five times; at baseline, immediately after exercise, and 0, 1st, and 4th hr of recovery. Blood alcohol concentration after ingestion was $0.0{\pm}0.0(0%),\;0.1{\pm}0.02(4%)$, and $0.2{\pm}0.03%$ (8% trial). No differences in blood sodium and potassium concentrations, and urine specific gravity were noticed over time periods and trials. Thirst sensation tended to be elevated in all trials immediately after exercises and urine output was elevated during the recovery. The magnitude of changes in these variables was proportional to the alcohol concentrations, but not statistically significant. While serum osmolality was not different among trials and time periods in 0 and 4% trials, it was higher during recovery than the baseline in the 8% trial (P<0.01). Triglycerides did not change throughout the time period and among trials. Free fatty acids were elevated after exercise in all trials and 4th hr of recovery in 0% (P<0.05). Subjects' net body fluid balance at 4th hr of recovery was negatively maintained and proportional to alcohol concentrations. Only 8% trials showed a significant reduction at 1st and 4th hr of recovery compared to 0 hr. The results suggested that diuretic effect of alcohol after moderate level of dehydration appeared dose dependent, but beverage containing alcohol up to 4% did not induce impaired rehydration than alcohol free drinks. Alcohol effects on fat mobilization during recovery appeared to be minimal and the mechanism is unclear.

• The Korean Journal of Food And Nutrition
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• v.33 no.6
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• pp.747-754
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• 2020

This study was conducted to evaluate the effect of high-oleate and normal-oleate peanut consumption on adipose mass and serum lipids in obese-induced C57BL/6J mice. After four weeks of the high-fat diet, mice were randomly divided into six groups: normal control (NC) diet, high-fat control (HFC) diet, high-oleate peanut-seed (HOPS) diet, normal-oleate peanut-seed (NOPS) diet, high-oleate peanut-oil (HOPO) diet, and olive-oil (OO) diet. After four weeks, all four experimental diet groups showed significantly lower body weight and epididymal fat weight than HFC group. In four experimental diet groups, serum triglycerides (TG), total cholesterol (TC) and low-density lipoprotein cholesterol (LDL-C) were significantly lower, and high-density lipoprotein cholesterol (HDL-C) was significantly higher than HFC group. TG was significantly decreased in HOPS group (92.1±1.2 mg/dL) than NOPS group (101.7±5.3 mg/dL, p<0.05). Similarly, LDL-C was significantly lower in HOPS group (66.1±2.8 mg/dL) than NOPS (76.9±1.5 mg/dL, p<0.05), on the other hand, HDL-C indicated a significant elevation in HOPS (50.5±2.1 mg/dL) than NOPS group (45.2±1.6 mg/dL, p<0.05). This result suggests that the consumption of high-oleate peanut has a favorable effect on the plasma lipid profile.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.13 no.2
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• pp.153-159
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• 1991

The use of the autogenous free fat is a well-known procedure to fill in superficial depressions resulting from the traumatic or congenital defects. The major donor site for this procedure was the abdominal subcutaneous fat or buttocks. In 1977, Egyedi was the first to report the use of the buccal fat pad as a pedicled graft. The buccal fat pad is a structure usually considered to be a nuisance when encountered in intraoral procedures such as facial bone osteotomies, elevation of buccal falp, or procedures on Stensen's duct. In these operations, appearance of the buccal fat pad complicates surgical exposure. The buccal fat pad is a lobulated convex mass of fatty tissue covered by a very delicate membrane, and is described as having a body from which four processes extend. These projection serve as a filling material between the various muscular structures in the area. Recently malar depression was augmented with the pedicled buccal fat pad in 3 cases, and it was used for the reconstruction of the nasolabial fold in one case.