• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.14-22
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• 2012

Objectives: This study was performed to review clinical manifestations of hyperphenylalaninemia patients in Korean. Methods: 178 cases of hyperphenyalaninemia were diagnosed at department of pediatrics, Soonchunhyang University Hospital from January, 1983 to August. We reviewed DNA analysis, MR imaging, EEG, radiography of the left hand and wrist, bone densitometry (BMD), IQ test of hyperphenyalaninemia patients. Results: Out of 178 cases, 161 cases were diagnosed classic phenylketonuria and 17 cases were diagnosed BH4 deficiency. 122 cases performed DNA analysis. R243Q (10.3%), Y204C (9.9%), and IVS4-1G>A (8.1%) mutations were predominant. 22 cases underwent MR imaging. Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the periventricular deep white matter of 15 cases. 23 cases were performed EEG. 12 cases (52.3%) showed abnormal pattern. EEG abnormalities showed in 11 cases. On lumbar BMD four of 11 cases (36%) showed reduced bone density of more than 1 S.D. in four of 11 cases, bone age was less than chronological age by at least one year. 18 cases were performed IQ test. Mean IQ scores was $84{\pm}21.6$. Among older than 15 years (9 cases), Mean IQ scores was $72{\pm}21.2$. PTPS deficiency was 14 cases, DHPR deficiency was 2 cases, and GTPCH deficiency was 1 case. Conclusion: We confirmed there were varieties of DNA mutations. And MR imaging and EEG were nonspecific in PKU patients. Older children showed lower IQ score. Low phenylalanine diet prevents brain damage in PKU patient. Not only first few years of life but also lifetime, Keeping low phenylalanine diet is important.

• Korean Journal of Environmental Agriculture
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• v.29 no.3
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• pp.273-281
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• 2010

Concentration of antibiotics including a tetracycline group (TCs) of tetracycline (TC), chlortetracycline (CTC), and oxytetracycline (OTC), a sulfonamide group (SAs) of sulfamethoxazole (SMX), sulfathiazole (STZ), and sulfamethazine (SMT), an ionophore group (IPs) of lasalocid (LSL), monensin (MNS), and salinomycin (SLM), and a macrolide group (MLs) of tylosin (TYL) was determined from samples collected from the agricultural soil, stream water, and sediment. For the agricultural soil samples, the concentration of TCs had the highest value among all tested antibiotic's groups due to its high accumulation rate on the surface soils. The lower concentrations of SAs in the agricultural soils may be resulted from its lower usage and lower distribution coefficient (Kd) compared to TCs. The concentration of TCs in stream water was significantly increased through June to September. It would be likely due to soil loss during an intensive rainfall event and a reduction of water level after the monsoon season. A significant amount of TCs in the sediment was also detected due to its accumulation from runoff, which occurred by complexation of divalent cations, ion exchange, and hydrogen bonding among humic acid molecules. To ensure environmental or human safety, continuous monitoring of antibiotics residues in surrounding ecosystems and systematic approach to the occurrence mechanism of antibiotic resistant bacteria are required.

• Journal of the Korean Chemical Society
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• v.43 no.4
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• pp.438-446
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• 1999

The optimum analytical method of aldehydes, ozone by-products, was established by reverse phase liquid chromatography. Six aldehydes including formaldehyde, acetaldehyde, acrolein, propionaldehyde, butylaldehyde and benzaldehyde, and one ketone including acetone were selected as aldehyde test samples through preliminary experiments. Such analytical conditions as the pH of citrate buffer solution, reaction temperature, reaction time, and concentration of DNPH, the component and composition of desorption solvent were optimized. As the result, pH 3.0 of citrate buffer solution, 40$^{\circ}C$ of reaction temperature, 15 minutes of reaction time, and 0.012% of DNPH concentration were chosen as optimum conditions. Aldehydes-DNPH derivatives in water were concentrated on $C_18$ Sep-Pak cartridge and followed by elution of their derivatives fraction with THF/ACN(70/30) mixture, and showed recoveries of the range from 87 to 107%. Separation condition on Nova-Pak $C_18$ column with low pressure gradient elution from ACN/MeOH/water(30/10/60) of an initial condition to 80% ACN of a final condition was found to give a good resolution within 20 minutes of run time. 86% to 103% of recovery for aldehydes using this method was similar to that for aldehyde using EPA Method 554 which is ranged from 84% to 103%.

• Journal of the Korean Applied Science and Technology
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• v.35 no.4
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• pp.975-984
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• 2018

Fast pyrolysis bio-oil has unfavorable properties that restrict its use in many applications. Among the main issues are high acidity, instability, and water and oxygen content, which give rise to corrosiveness, polymerization during storage, and a low heating value. Esterification and azeotropic water removal can improve all of these properties. A 500 g of Quercus mongollica which grounded 0.8~1.4 mm was processed into bio-oil via fast pyrolysis for 2 seconds at $550^{\circ}C$. The esterification consists of treating pyrolysis oil with a high boiling alcohol like n-butanol at $70^{\circ}C$ under reduced pressure (100 hPa). All products are analyzed for water mass fraction, viscosity, higher heating value, pH, FT-IR and GC/MS. The water mass fraction can be reduced by 91.4 % (from 31.5 % to below 2.7 %), the viscosity by 65.8 % (from 36.5 to 12.5 cP) and the higher heating value can be increased by 96.8 % (from 3,918 to 7,712 kcal/kg), the pH by 1.3 (from 2.7 to 4.0). FT-IR and GC/MS analysis indicated that labile acids, aldehydes, ketones and lower alcohols were transformed to stable target products. Using this approach, the water content of the pyrolysis oil is reduced significantly. These improvements should allow the utilization of upgraded pyrolysis liquids in standard boilers and as fuel in CHP (Combined heat and power) plants.

• Korean Chemical Engineering Research
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• v.50 no.4
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• pp.672-677
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• 2012

Lipids in microalgal biomass were recovered by using pyrolysis method. The pyrolysis experiments of two Chlorella sp. KR-1 samples, which have triglyceride contents of 10.8% and 36.5%, respectively were carried out at $600^{\circ}C$ to investigate the effects of lipid contents in the cells on the reaction characteristics. The conversion and liquid yield of the lipid-rich sample were higher than those of the lipid-lean sample since its carbon to hydrogen ratio was low. There were low molecular weight organic acids, ketones, aldehydes and alcohols in the liquid products from both KR-1 samples, but the pyrolysis oil of the lipid-rich sample was abundant in free fatty acids, particularly palmitic acid, oleic acid and stearic acid while the content of nitrogen containing organic compounds was low. The microalgal pyrolysis oil had two layers composed of the light hydrophobic fraction and the heavy hydrophilic fraction. The light fraction might be originated from triglycerides and the heavy fraction might be from carbohydrates and proteins. In the light fraction of the liquid products, there were considerable linear alkanes such as pentadecane and heptadecane as well as free fatty acids, implying that deoxygenation reaction including decarboxylation was occurred during the pyrolysis. The yield of the liquid products from the pyrolysis of the KR-1 sample having triglyceride content of 36.5% was 56.9% and the light fraction in the liquid products was 68.2%. Also more than 80% of the light fraction was free fatty acids and pure hydrocarbons, thus showing that most triglycerides could be extracted in the form of suitable raw materials for biofuels.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.79-85
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• 2016

Purpose: This study aimed to analyze genetic mutations, clinical manifestations, and treatment of patients with benign HPA in Korea. Methods: This case series study involved ten HPA patients who were referred to our hospital because of high phenylalanine concentration. We investigated their demographic features, clinical manifestations, and mutations of the PAH gene through direct DNA sequencing. Results: Among ten patients with benign HPA, two pairs of patients were related (father-daughter, mother-daughter relationship) cases, and all of them showed no specific clinical manifestations or notable past history. Their plasma phenylalanine levels ranged between 1.2 and 4.2 mg/dL. In the tetrahydrobiopterin (BH4) loading test, all patients were nonresponsive to BH4. In the confirmation test of PAH mutation analysis, we identified eleven different alleles out of twelve. The most common allele was R53H (c.158G> A). In addition, two novel PAH gene mutations, V423A (c.1268T>C) and V51A (c.152T>C), were identified. Although the patients did not receive any pharmacologic treatment or continuous phenylalanine restriction dietary therapy, their neurocognitive development was normal. Moreover, on serial outpatient follow-up tests, all patients maintained phenylalanine levels below 6 mg/dL. Conclusion: This study is the first in Korea to analyze benign HPA patients. All patients with benign HPA could maintain phenylalanine levels below 6 mg/dL with normal neurocognitive development, without continuous therapy. Therefore, performing mutation analysis and distinguishing benign HPA from phenylketonuria (PKU) are important to help improve life quality in patients with benign HPA by avoiding unnecessary lifelong therapy.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.18 no.6
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• pp.511-518
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• 1985

As one of the sensory factors for characterizing food quality, volatile compounds have been particularly contributed to sensory evaluation of fermented sea foods in Korea. But no chemical investigation of the volatile compounds of fermented anchovy as one of the most favored fermented sea food products has been reported. Accordinglry, for a series study of processing of low salt fermented sea foods, changes in volatile compounds and fatty acid composition of fermented anchovy with low salt contents ($4\%$ of salt contents) were experimented fermentation comparing with conventional fermented anchovy ($20\%$ of salt contents). Total lipid of raw anchovy was composed of $77.6\%$ of neutral lipid, $22.1\%$ of phospholipid and $0.3\%$ of glycolipid. And polyenoic acid was held $39.8\%$ of fatty acid composition of total lipid and the major fatty acids in those were $C_{22:6},\;C_{20:5}$. During the fermentation of anchovy saturated fatty acid ($C_{16:0},\;C_{18:0},\;C_{l4:0}$) and monoenoic acid ($C_{16:1},\;C_{18:1}$) increased while polyenoic acid ($C_{22:6},\;C_{20:5}$) decreased greatly. Thirty-eight kinds of volatile component from the whole volatile compounds obtained from fermented anchovy after 90 days fermentation were identified, and composed of some alcohols (8 kinds), carbonyl compounds (9 kinds), hydrocarbons (8 kinds) and fatty acids (8 kinds). During fermentation 8 kinds of volatile acids, 5 kinds of amines, 9 kinds of carbonyl compounds were also detected. Those volatile acids such as acetic acid, isovaleric acid, propionic acid, n-butyric acid were the major portion of total volatile fatty acids of 60 days fermented anchovy prepared with low salt contents. On the other hand, carbonyl compounds such as ethanal, 3-methyl butanal, hexanal, 2-methyl propanal were the major ones, while TMA held the most part of volatile amines in fermented anchovy with low salt contents after 60 days. Conclusively, there was little difference in composition of volatile components, but merely a little difference in content of those between low salt fermented anchovy and conventional fermented ones.

• Journal of Applied Biological Chemistry
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• v.60 no.3
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• pp.283-291
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• 2017

Rice that the half of population in the world eats as a staple food is mostly produced and consumed in Asia. However, its consumption is nowadays decreasing mainly due to diet diversity. Accordingly, some attempts are in demand to enhance the utilization of rice. In this study, profiling of volatile and non-volatile flavor components in rice pastes obtained by ${\alpha}$-amylase was performed and compared according to nine different rice cultivars domestically cultivated in Korea using gas chromatography-mass spectrometry combined by solid phase microextraction and gas chromatography-time of flight-mass spectrometry after a derivatization, respectively. In total, 46 volatile compounds identified included 6 alcohols, 6 aldehydes, 4 esters, 4 furan derivatives, 4 ketones, 1 acid, 1 sulfur-containing compound, 7 hydrocarbons, 5 aromatics and 8 terpenes. The non-volatile flavor components found were composed of 12 amino acids, 6 sugars and 4 sugar alcohols. In principal component analysis, rice paste samples could be discriminated according to cultivars on the score plots of volatile and non-volatile flavor compounds. In particular, some volatile compounds such as pentanal and 4,7-dimethylundecane could contribute to distinguish Senong 17 white and Senong 17 brown, whereas ethanol, 6-methylhep-5-en-2-one, and tridecane could be highly related to the discrimination of Iipum from other cultivars. Among non-volatile compounds, some amino acids such as glycine, serine and ${\gamma}$-aminobutyric acid and some sugars such as sucrose and fructose were mainly responsible for the discrimination of Danmi from the other cultivars. On the other hand, galactose, arabitol and mannose were more closely related to Senong 17 white than Senong 17 brown.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.1-12
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• 2018

Purpose: Disorders of organic acid metabolism have various clinical manifestations and it may be life-threatening. The prognoses of affected children are dependent on early diagnosis and treatment. We report this study to find out detection rate of referred samples, clinical manifestations and age distribution after introduction of neonatal screening test using tandem mass spectrometry in Hallym University Chuncheon Sacred Heart Hospital during 8 years and 9 months. Methods: The 2,794 patients referred from Jan. 2007 to Sep. 2015 were divided into four groups according to age. We conducted organic acid analysis of urine samples of patients and analyzed clinical manifestations and distributions of age at the diagnosis. For patients with ambiguous results, reanalysis of urine organic acid after diet restriction, protein loading and restriction, has been done. Results: A total of 626 patients with 20 disorders were diagnosed. Mitochondrial disorders (482 patients) were the most common diagnosis, followed by ketolytic defects (67), 3-hydroxyisobutyric aciduria (32), EPEMA syndrome (8), 3-methylcrotonyl glycinuria (7), glutaric aciduria type II (6) and type I (4), methylmalonic aciduria (3), isovaleric aciduria (3) and medium chain acyl-CoA dehydrogenase deficiency (3). Conclusion: As neonatal screening test using tandem mass spectrometry is increasingly common and medical environment is changed, detection rate of disorders of organic acid metabolism in this study has decreased compared to previous report. Because the deterioration can be prevented by early diagnosis and treatment, many pediatricians have to pay special attention to possibility of the disorders and make an effort for early diagnosis in clinical setting.

• The Korean Journal of Pesticide Science
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• v.18 no.2
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• pp.79-87
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• 2014

Pyriofenone is an aryl phenyl ketone fungicide that is newly registered in Korea in 2013 to control powdery mildew on food. The objective of this study was to develop reliable and sensitive analytical method for determination of pyriofenone residue in agricultural products for ensuring the food safety. The pyriofenone residues in all samples(Korean melon, pepper, potato, mandarin, soybean, and hulled rice) were extracted with acetonitrile, partitioned with dichloromethane, and then purified with a silica cartridge. The purified samples were analyzed by HPLC-UVD and confirmed with LC-MS. The linear range of pyriofenone was 0.05~5 mg/kg with the correlation coefficient ($r^2$) > 0.999. Average recoveries of pyriofenone ranged from 72.8% to 99.5% at the spiked level of 0.05 and 0.5 mg/kg, while the relative standard deviation was 2.3%~6.4%. In addition, the limit of detection and limit of quantification were 0.01 and 0.05 mg/kg, respectively. The results revealed that the developed and validated analytical method was suitable for pyriofenone determination in agricultural products.