• 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.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.17 no.5
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• pp.373-382
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• 1984

Processing conditions of retort pouched fried mackerel fish meat paste and quality stability during storage were investigated. The reasonable amounts of added ingredients to the frozen mackerel meat paste were $10\%$ of corn starch, $1\%$ of soybean protein, $1.5\%$ of sodium chloride, $0.6\%$ of monosodium glutamate, $0.3\%$ of alcoholic extract of red pepper, and $0.1\%$ of sodium erythorbate as an antioxidant and also added water corresponding to $10\%$ of the frozen mackerel meat paste. After grinding the defrosted mackerel fish meat paste with ingredients, the meat paste was molded in bar type and fried in soybean oil at $170-180^{\circ}C$ for 3 minutes. The fried mackerel meat paste was cooled, vacuum-packed in laminated plastic film bag (polyester/polyvinylidene chloride/unoriented polypropylene : $12{\mu}m/15{\mu}/50{\mu}m,\;14{\times}19cm$) and finally sterilized at $120^{\circ}C$ for 20 minutes in a hot water circulating retort. The pH, volatile basic nitrogen, moisture content, water activity, color, thiobarbituric acid value, peroxide value, texture and viable bacterial count of products were examined during 100 days of storage at $25{\pm}3^{\circ}C\;and\;5^{\circ}C$. The results showed that products could be preserved in good condition for 100 days at $25{\pm}3^{\circ}C$. Judging from sensory evaluation, the quality of products was not inferior to that of market products.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.17 no.5
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• pp.353-360
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• 1984

As an effort to expand the utilization of mackerel which has been thought disadvantageous to processors due to the defects in bloody dark color of meat, high content of lipid, and low stability of protein, and to develope a new type of product, so called, preservative fish meat paste, the processing method was studied in which dielectric heating was applied by means of cooking, pasteurization, dehydration, and control of water activity. The principle of this method is based on that dielectric heating can initiate a rapid dispersion or displacement of moisture in the meat tissue so that the level of water acivity can be controlled by dehydration with hot air meanwhile the product is cooked, pasteurized, and texturized. And the product is finally heated with electric heaters and vacuum sealed to stabilize water activity and storage stability. In present paper, a formula for preparing the fish meat-stach paste, the conditions of dielectric heating and dehydration, shape and size of the product, and other parameters were tested to optimize the process operation. A formula of the fish meat-starch paste to provide proper textural properties and water activity was $10\%$ starch, $1.5\%$ salt, $3\%$ soybean, $0.6\%$ MSG, $2\%$ sucrose, and $3\%$ sorbitol against the weight of fish meat. A proper shape and size of the product to avoid foaming and case hardening during heating was sliced disc of 8 cm $diameter{\times}0.8$ cm thickness or $10{\times}10$ cm square plate with 1.0 cm thickness. The disc shape was recommended because it resulted more uniform heating, minimum foaming and case hardening. And it was also advantageous that disc was simply provided when the fish meat disc was stuffed in the same, solidified in boiling water for 2 to 3 minutes, and sliced. Condition of dielectric heating was critical to decide the levels of sterility, water activity, and textural property of the product. The temperature at the center of the meat disc slices was raised up to $95^{\circ}C$ in 1.5 minutes so that continuous exposure to microwave caused expanded tissue and hardening ending up with a higher water content. Heating for 5 to 6 minutes was adequate to yield the final water activity of 0.86 to 0.83(35 to $40\%$ moisture). It is important, however, that heating had to be done periodically, for instance, in the manner of 2.0, 1.5, 1.5, and 1.0 minute to give enough time to displace or evaporate moisture from the meat tissue. The product was dehydrated for 2 to 3 minutes by hot air of $60^{\circ}C$, 3 to 5m/sec and finally exposed to electric heaters for 5 to 6 minutes until the surface was roasted deep brown. These conditions of heating and dehydration resulted in a complete reduction of total plate count from an initial count of $5.3{\times}10^6/g$ to less than $3{\times}10^2/g$. General composition of the product was $40.1\%$ moisture, $20.8\%$ protein, $17.4\%$ lipid, $16.2\%$ carbohydrate, and $5.5\%$ ash. Textural properties revealed folding test AA, hardness 42, cohesiveness 0.53, toughness 4.6, and elasticity 0.8.

• Journal of the Economic Geographical Society of Korea
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• v.8 no.1
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• pp.91-105
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• 2005

The assessment (or the evaluation) of spatial disparity is the main concern for the study of spatial disparities or spatial inequalities. In order to evaluate the spatial disparity, the regional differences have to be counted quantitatively. Several measurements have been introduced for evaluating the development potentials of each region. Most of them are the composite indices of the socio-economic variables rather than the real potentials of the region. This study attempts to investigate the spatial disparity in Korea. For the purpose, the levels of opportunity potentials of the cities have been calculated by the Potential Model redefined by Lee(1995). The opportunity potentials have been calculated for the educational, cultural, medical service, environmental sectors, income, and consumption sectors, and the spatial patterns of various opportunity potentials have been analyzed. The spatial patterns of opportunity potentials show the severe concentration on the Metropolitan Seoul area through all sectors. The next level concentration appears at the other end of the Keuyng-Bu axis. And the cities relatively high opportunity potential values are distributed along the Keuyng-Bu axis. Remain parts of the country show quietly low opportunity potential values. In particular, the southern-west and the northern-east parts show relatively very low values. This pattern appears for all sectors except for the opportunity potential of the environmental sector. It means that the spatial disparity in Korea have been promoted and enhanced by the national development policies concentrated the investment on the large cities along the Keuyng-Bu axis during the last 40 years.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.34 no.4
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• pp.389-393
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• 2001

For an effective utilization, quality stability of instant powdered oyster soup made of canned oyster processing waste water (IPSW) was determined. Instant powdered soup from oyster hot-water extracts (IPSE) was also prepared by mixing hot-water extract powder (15 g) with table salt (5 g), cream powder (19 g), milk replacer (12 g), wheat flour (20 g), corn flour (15 g), starch (5 g), glucose (7.5 g) and onion powder (1.5 g). In preparing IPSW, mixed powder from wash water and boiling liquid waste, instead of powder from hot-water extracts and table salt, was added (powder from boiling liquid waste: powder from wash water= 12: 8) and other additives were added in proportion to those in the IPSE. The moisture content, water activity, peroxide value and fatty acid composition showed little changes during storage of the IPSW. The pH, volatile basic nitrogen content and brown pigment formation increased slightly, while white index decreased slightly during storage of IPSW. No significant difference was observed in the changes of food component during storage between IPSW and IPSE. According to a sensory evaluation, the change in quality of IPSW was negligible during 12 months of storage. from the results of the chemical experiment and sensory evaluation, IPSW packed with laminated film bag (OPP, $20{\mu}m/PE,\;20{\mu}m/paper,\;45g/m^3/PE,\;20{\mu}\;m/Al,\;7{\mu}\;m/PE,\;20{\mu}m$) was revealed to be preserved in good quality during 12 months of storage.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.96-102
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• 2017

Glycogen storage disease type IX (GSD IX) is caused by deficiency of phosphorylase kinase which plays a role in breakdown of glycogen. Mutations in PHKA2 are the most common cause of GSD IX (GSD IXa). Clinical manifestations of GSD IXa include hepatomegaly, elevation of liver enzyme, growth retardation, fasting hypoglycemia, and fasting ketosis. However, the symptoms overlap with those of other types of GSDs. Here, we report Korean familial cases with GSD IXa whose diagnosis was confirmed by targeted exome sequencing. A 4-year old male patient was presented with hepatomegaly and persistently elevated liver enzyme. Liver biopsy revealed swollen hepatocyte filled with glycogen storage, suggesting GSDs. Targeted exome sequencing was performed for the differential molecular diagnosis of various types of GSDs. A hemizygous mutation in PHKA2 were detected by targeted exome sequencing and confirmed by Sanger sequencing: c.3632C>T (p.Thr121Met), which was previously reported. The familial genetic analysis revealed that his mother was heterozygous carrier of c.3632C>T mutation and his 28-month old brother had hemizygous mutation. His brother also had hepatomegaly and elevated liver enzyme. The hypoglycemia was prevented by frequent meals with complex carbohydrate, as well as cornstarch supplements. Their growth and development is in normal range. We suggest that targeted exome sequencing could be a useful diagnostic tool for the genetically heterogeneous and clinically indistinguishable GSDs. A precise molecular diagnosis of GSD can provide appropriate therapy and genetic counseling for the family.

• Applied Biological Chemistry
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• v.44 no.4
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• pp.224-229
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• 2001

This study was carried out to examine the quality stability of powdered soup using powder from oyster wash water (PSW). To compare the quality stability, powdered soup from oyster hot-water extracts (PSE) was also prepared by mixing hot-water extract powder (15 g), table salt (5 g), cream powder (19 g), milk replacer (12 g), wheat flour (20 g), corn flour (15 g), starch (5 g), glucose (7.5 g), and onion powder (1.5 g). In preparing PSW, powder from oyster wash water, instead of powder from oyster hot-water extracts, was added and other additives were the same proportion as PSE. The PSW and PSE were packed with laminated film bag (OPP,$20\;{\mu}m$; PE, $20{\mu}m$; paper, $45\;g/m^3$; PE, $20\;{\mu}m$; Al, $7\;{\mu}m$; PE, $20\;{\mu}m$), and then stored at ambient temperature for 12 months. The moisture content, water activity, peroxide value, and fatty acid composition showed little changes during storage of the PSW, The pH, volatile basic nitrogen content, and brown pigment formation increased slightly, while white index decreased slightly during storage of PSW. No significant difference was observed in the changes of food components between PSW and PSE during storage. According to a sensory evaluation, the change in quality of PSW was negligible during 12 months of storage. From the results of the chemical experiment and sensory evaluation, PSW packed with laminated film bag (OPP, $20\;{\mu}m$; PE, $20\;{\mu}m$; paper, $45\;g/{\mu}m$; PE, $20\;{\mu}m$) was revealed to be preserved in good quality during 12 months of storage.

• Journal of Animal Science and Technology
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• v.53 no.2
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• pp.147-154
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• 2011

This study was conducted to evaluate the effects of saponin contained plant extracts on in vitro rumen fermentation characteristics and methane production. Ruminal fluid was collected from rumen cannulated Hanwoo steers fed rice straw and concentrate (5:5). Collected rumen fluids, corn starch and buffer including saponin contained plant extracts (ginseng, Ogapi, soapwort, tea plant and yucca; 0.5%/15 ml) were incubated at $39^{\circ}C$ for 24 h. All incubations were repeated five times. Rumen pH in all treatments was lower (p<0.05) compared with that of the control (no addition) during incubation time. The concentration of total VFA in all treatments was higher (p<0.05) than that of the control after 12h incubation. Compared with the control, the concentration of acetate and propionate in all treatments was lower and higher after 6h incubation, respectively. The concentration of $NH_3$-N in all treatments was lower (p<0.05) than that of the control except for Ogapi or yucca extracts supplementation. The number of protozoa in all treatments was significantly (p<0.05) lower than that of the control except for soapwort extract supplementation. The total gas production and methane production in all treatments was higher (p<0.05) and lower (p<0.05) compared with the control, except for ogapi or soapwort extracts supplementation after 12h incubation, respectively. Therefore, reduction in methane production by saponins may could be results from decreased protozoal population without any negative in vitro fermentation.

• Journal of Animal Science and Technology
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• v.50 no.4
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• pp.499-508
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• 2008

This study was conducted to evaluate the effect of bio-starch from corn processing to replace dried whey in weaned pigs. A total of 120 crossbred [(Landrace×Yorkshire)×Duroc] pigs were weaned at 21 days of age weighing 6.01±0.34 kg in average. Five week feeding trial consisted of phase 1(0~2 wks) and phase 2(3~5 wks). Dietary treatments included 1) CON(basal diet), 2) BS5(whey 5%, partial substitution of bio-starch), 3) BS10(whey 10%, partial substitution of bio-starch) and 4) BS15(whey 15%, partial substitution of bio-starch). There were four dietary treatments with six replicate pens per treatment and five pigs per pen. During the overall period, there were no significant differences in the ADG(average daily gain) and gain/feed ratio among the treatments(P>0.05). However, the ADFI(average daily feed intake) was higher in BS5 and BS15 treatments than in CON treatment(P<0.05). At the 2nd week, dry matter and nitrogen digestibility were increased(quadratic effect, P=0.03 and P=0.01, respectively; cubic effect, P<0.001 and P=0.01, respectively) with the highest at 10% of bio-starch inclusion in the diets. At the last week of the experiment, dry matter, nitrogen and energy digestibility were increased(P<0.05) with the highest at 5% of bio-starch inclusion in the diets. At the 2nd week total protein concentration was increased(linear effect, P=0.04; cubic effect, P=0.01) with the highest at 10% of bio-starch inclusion in the diets. Also, BUN(blood urea nitrogen) was increased(linear effect, P=0.01) as the level of bio-starch inclusion increased in the diets. Fecal consistency score was inclined to lowers CON treatment than other treatments. In conclusion, the result of trial indicates that bio-starch can be included at the level of 5~10% of weaning pig diet replacing part of the dried-whey, and digestibilities were positively affected by bio-starch at growth stage.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.34 no.3
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• pp.285-290
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• 2001

To utilize canned oyster processing waste water effectively, this study was carried out to prepare instant powdered soup using the waste water (IPSW), Instant powdered souu from oyster hot-water extracts (IPSE) was prepared by mixing hot-water extracts powder (15 g) with table salt (5 g), cream powder (19 g), milk replacer (12 g), wheat flour (20 g), corn flour (15 g), starch (5 g), glucose (7.5 g) and onion powder (1.5 g). In preparing IPSW, mixed powder from wash water and boiling liquid waste, instead of powder from hot-water extracts and table salt, was added (powder from boiling liquid waste: powder from wash water= 12: 8) and other additives were added in proportion to those in the IPSE, The IPSW consists mainly of carbohydrates (about $72\%$). It was not different from the IPSE. The volatile basic nitrogen, viable cell counts, coliform group of the IPSW contains 33.4 mg/100g, $2.2\times10^4CFU/g$, <180 MPN/100g, respectively, and its water activity has 0.257. So it was a hygienically safe and conservable instant food. The main fatty acids of IPSW were 16: 0 and 18: 1n-9. Its chemical score of protein was $61.4\%$ and its main inorganic matter was iron. According to a sensual evaluation, in contrast to the IPSE, the IPSW had a bit lower aroma but better taste, It was concluded from the above chemical and sensory evaluation that even the boiling liquid waste which had been mostly abandoned because of its high table salt content can be used as a good material for instant powdered soup if it's powdered and mixed adequately with powder from wash water, and its table salt content is properly adjusted.