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A case of simultaneously identified glycogen storage disease and mucopolysaccharidosis (당원병과 뮤코다당체침착증이 동시에 발견된 증례 1예)

  • Lee, Ju Young;Shim, Jeong Ok;Yang, Hye Ran;Chang, Ju Young;Shin, Choong Ho;Ko, Jae Sung;Seo, Jeong Kee;Kim, Woo Sun;Kang, Gyeong Hoon;Song, Jeong Han;Kim, Jong Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.650-654
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    • 2008
  • Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

Effects of Low-power Laser and TDP on the Cutaneous Wound Healing (피부상처 치유에 대한 저에너지레이저와 특정전자파의 효과)

  • Ahn So-Youn;Park Sang-Ock
    • The Journal of Korean Physical Therapy
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    • v.9 no.1
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    • pp.1-18
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    • 1997
  • The purpose of this study is to investigate the effects of the low power Helium Neon-Infra Red(HeNe-IR) laser and the special electromagnetic therapeutic apparatus stimulation, which is usually designated as TDP by using the initial of Tending Diancibo Pu which is the Chinese inscribed with English, on wound healing in rat. The seventy-five Sprague-Dawley adult female and male rats were assigned to the experimental and control groups. Each rat was anesthetized with pentobarbital sodium, and three full-thickness incisions with 12mm length wert made on the back of the half cf the rats and three deep second degree burns were made on the back of the remainder of the rats. From 34 hours after being injured, the rats of the experimental laser group were irradiated with the 157mW electric power HeNe-IR laser for 2 minutes every day and the rats of the experimental TDP group were stimulated with TDP irradiation with the 4km spot distance for 20 minutes every day during the 17 days. The rats were sacrificed and the wound parts of its were incised on the first day, 4th day, 7th day, 10th day and 17th day respectively after the beginning of wound treatment with laser and TDP irradiation. The incised wound parts were processed appropriately for the light microscopic and electron microscopic examination. The length of incised wound was measured with microcaliper before the wound part was incised. There was a significant decrease in the length of the incised wound of the experimental laser and TDP group, compared with that of control group at 4th day, 7th day and 10th day(p<0.01) after surgery. Through the histological examination of the wound site, the more rapid epithelialization and collagen formation in experimental groups were showed, compared with control group. The histological results were analyzed and summarized as the follows; The epidermis begins to be regenerated and the granulation tissue begins to be changed to the mature pattern in the H-E stained incised skin of the laser and TDP treatment group on the 4th day. The epidermis shows the complete regeneration and the granulation tissue in the dermis in mostly to be matured in the laser and TDP treatment group on the 7th day, compared with control group. The chronic inflammatory cells are oberved and the necrosis of the collagen fibers are partially observed in control group on the 10th day. The dermis of the laser and TOP treatment group reveals relatively compactly arranged collgen bundles with the mature collagen fibers on the 10th day. The epidermis and dennis of the laser and TDP group are repaired normally and the hair follicles are well regenerated on the 17th day. The mild edema and the granulation tissue is observed in the dermis of the control groups and the delayed treatment process is observed on the 17th day. The Most of proliferated collagen fibrils are found to be compact and regular in electron micrograph of burn skin of the laser treatment group on the 10th day hut the interstitial eadema and some inflammatory cells are found in the control group. The above results suggest that through the visual and histological examination the epithelized epithelium and the proliferation of the collagen liters in the dermis occur very effectively with the low power laser treatment and the TDP treatment in the incised wound healing and the burn wound healing.

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Organic acid and quality change in Flammulina velutipes fruit body by various storage temperature treatments and packaging films application (포장필름을 달리한 팽이버섯의 저장 온도별 품질변화 및 유기산 함량)

  • Kim, Kyung-Je;Jin, Seong-Woo;Choi, Bong-Suk;Kim, Jin-Kyeong;Koh, Young-Woo;Kim, A-Rum-Chan;Seo, Kyoung-Sun
    • Journal of Mushroom
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    • v.13 no.2
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    • pp.119-124
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    • 2015
  • This study was carried out to characterize the chemical components of Flammulina velutipes during storage by different packaging film application for developing export winter mushroom to south east asia area. Changes of weight and growth rate from Flammulina velutipes were the lowest in testing packaging film at $15^{\circ}C$ among the films. The pH changes of Flammulina velutipesg was the highest in imported packaging film at $25^{\circ}C$. The changes of colors was measured by Hunter's color value, L value and yellowness (+b) were slightly decreased during storage. But the L value and yellowness (+b) were not affected by packaging films. Six organic acids were detected such as oxalic acid, lactic acid, malic acid, citric acid, acetic acid and succinic acid. The content of total organic acids were increased by storage period.

Study on Life Style of Health Promotion for the Elderly - Centering on farming villages in Jeollabuk-do Province - (노인들의 건강증진생활양식에 관한 연구 - 전북 농어촌지역을 중심으로 -)

  • Lee Jin-Woo;Chong Myung-Soo;Lee Chun-Woo;Kwon So-Hee;Ko Kwang-Jae;Jeoung Jae-Yeal;Jahng Doo-Sub;Song Yung-Sun;Lee Ki-Nam
    • Journal of Society of Preventive Korean Medicine
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    • v.5 no.2
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    • pp.8-28
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    • 2001
  • This investigation grasps the level and relevant elements of performance of health promotional activities for the elderly in Korea. It provides fundamental data on health promoting projects targeting the elderly population from farming villages. Hence, this study gropes for an effective approach and measures of health promoting programs. The program needs to be developed with a focus on elderly people from farming villages. In addition, it was carried out in order to provide basic data for development of health projects for local communities. Data gathering was based on survey data targeting patients from the free clinic service. Service was rendered for the residents of farming villages, and conducted at the Offices of CheonBuk Province from October 2000 to December 2000. Analytical results were used to examine the health promotional method for the elderly in the aspect of Oriental Medicine. SPSS 9.0 version as well as T-test and ANOVA were used for survey data analysis. Piersons correlation coefficient was utilized for the relationship for each area, obtaining the following analytical results. 1. The average score for the activities of health promotion was 2.28. Looking at each subcategory, stress management was the highest at 3.65; interpersonal relationship, 3.00; nutrition, 2.55; health responsibility, 2.15; self-realization, 2.03; and exercise was the lowest at 1.89. 2. With respect to lifestyle of the health promotion secondary to general features of elderly people from farming villages, the level of activities of health promoting lifestyle was shown to be higher for males than that of females. Self-realization area was high among males in detailed particulars while the level of execution was high as age decreases in the stress area. 3. Regarding health promoting life style secondary to socioeconomic characteristics, the level of execution was higher for the individuals with a higher level of education and further utilization of spare time. With respect to occupation, the level was highest for people from the fishery. The level decreased in the order of other occupations such as trade, unemployed and agriculture, which was shown to be the lowest. In detailed particulars, it revealed that higher the individuals educational level, the higher the self-realization and stress management areas. The level of interpersonal relationship was the highest among people with little or no education. With respect to self-realization area, the level was highest among the cases where one paid living expenses along with their children. The lowest level of living expenses was seen in the cases where an individual pays for living expenses by himself/herself. There were significant results in all areas except for nutrition areas depending on occupation. The fishery was shown to be the highest. The level of activities was higher as one utilizes more spare time in all areas except for the area of interpersonal relationship.

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Inferring the Transit Trip Destination Zone of Smart Card User Using Trip Chain Structure (통행사슬 구조를 이용한 교통카드 이용자의 대중교통 통행종점 추정)

  • SHIN, Kangwon
    • Journal of Korean Society of Transportation
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    • v.34 no.5
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    • pp.437-448
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    • 2016
  • Some previous researches suggested a transit trip destination inference method by constructing trip chains with incomplete(missing destination) smart card dataset obtained on the entry fare control systems. To explore the feasibility of the transit trip destination inference method, the transit trip chains are constructed from the pre-paid smart card tagging data collected in Busan on October 2014 weekdays by tracing the card IDs, tagging times(boarding, alighting, transfer), and the trip linking distances between two consecutive transit trips in a daily sequences. Assuming that most trips in the transit trip chains are linked successively, the individual transit trip destination zones are inferred as the consecutive linking trip's origin zones. Applying the model to the complete trips with observed OD reveals that about 82% of the inferred trip destinations are the same as those of the observed trip destinations and the inference error defined as the difference in distance between the inferred and observed alighting stops is minimized when the trip linking distance is less than or equal to 0.5km. When applying the model to the incomplete trips with missing destinations, the overall destination missing rate decreases from 71.40% to 21.74% and approximately 77% of the destination missing trips are the single transit trips for which the destinations can not be inferable. In addition, the model remarkably reduces the destination missing rate of the multiple incomplete transit trips from 69.56% to 6.27%. Spearman's rank correlation and Chi-squared goodness-of-fit tests showed that the ranks for transit trips of each zone are not significantly affected by the inferred trips, but the transit trip distributions only using small complete trips are significantly different from those using complete and inferred trips. Therefore, it is concluded that the model should be applicable to derive a realistic transit trip patterns in cities with the incomplete smart card data.

Cloning and Characterization of a 5-Enolpyruvyl Shikimate 3-Phosphate Synthase (EPSPS) Gene from Korean Lawn Grass (Zoysia japonica) (들잔디 5-Enolpyruvyl Shikimate 3-Phosphate Synthase(EPSPS) 유전자 클로닝 및 특성)

  • Lee, Hye-Jung;Lee, Geung-Joo;Kim, Dong-Sub;Kim, Jin-Beak;Ku, Ja-Hyeong;Kang, Si-Yong
    • Horticultural Science & Technology
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    • v.28 no.4
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    • pp.648-655
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    • 2010
  • This study is the first comprehensive report on the molecular cloning, structural characterization, sequence comparison between wild and mutant types, copy number in the genome, expression features and activities of a gene encoding 5-enolpyruvylshikimate-3-phosphate synthase (EPSPS) in Korean lawn grass ($Zoysia$ $japonica$). The full length cDNA of the EPSPS from Korean lawn grass ($zj$EPSPS) obtained from a 3' and 5' RACE method was 1540 bp, containing a 1176 bp ORF, a 144 bp leader sequence (5' UTR) and a 220 bp 3' UTR, which was eventually decoded 391 amino acid residues with a molecular mass of 41.74 kDa. The Southern blot detection of the $zj$EPSPS showed that the gene exists as a single copy in the Korean lawn grass genome. Sequence comparison of the $zj$EPSPS gene demonstrated that the glyphosate-tolerant mutant (GT) having a Pro-53 to Ser substitution in the gene seems to have a preferred binding activity of the enzyme to phosphoenol pyruvate(PEP) over glyphosate, which allows the continuous synthesis of aromatic amino acids in the shikimate pathway. From the Northern blotting analysis, the $zj$EPSPS was found to be highly expressed, with continuous increase until 36 hours after 0.5% glyphosate treatment in both wild and mutant samples, but 1.5-fold higher EPSP synthase activity was observed in the tolerant mutant when exposed to the glyphosate treatment. The molecular information of the $zj$EPSPS gene obtained from this study needs to be further dissected to be more effectively applied to the development of gene-specific DNA markers and zoysiagrass cultivars; nevertheless, the glyphosate-tolerant mutant having the featured $zj$EPSPS gene can be provided to turfgrass managers for weed problems with timely adoptable management options.

Grain Yield Potential of a Low-Tillering Large Panicle Type in Rice (벼 소얼 수중형 초형의 수량성)

  • Kim, Je-Kyu;B.S., Vergara
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.37 no.4
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    • pp.361-371
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    • 1992
  • For the increase of grain yield potential in rice, a low-tillering large panicled type has been suggested as an ideotype. A low-tillering plant type may have different yield potential and needs different cultural practices from that used in a high-tillering type for the maximum yield. This study was conducted to evaluate the grain yield performances of a low-tillering large panicled rice and high-tillering small panicled rice at different plant spacings, nitrogen(N) levels and seedling numbers per hill. A low-tillering large panicled genotype, IR25588 was compared with a high-tillering small panicled IR58. The grain yield of IR25588 was significantly higher than that of IR58 under a narrow spacing with high N level. The maximum yields of IR58 and IR25588 were reached at about 35,000 and 40,000 spikelets per m$^2$, respectively. The increased grain yield in IR25588 was mainly due to the increase in spikelet number per unit area which is the most precise indicator of grain yield in rice. The optimum spacing for the maximum yield was denser for IR25588 than that for IR58 under high N level. The intra-hill competition of the low-tillering type was lower than that of the high-tillering type. The higher dry matter production and bigger leaf area and culm weight were the main factors for increased grain yield in a low-tillering panicle weight type. Based on the results, the yield potential of a low-tillering panicle weight type was higher than that of a high-tillering panicle number type, especially under a close spacing with high N level.

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Improvement of Rice Quality Using Grain Color Sorter During Early Transplanting Cultivation in the Southern Plain of Korea (색채선별기 이용한 남부평야지 조기재배 해담쌀의 품질향상)

  • Lee, Jong-Hee;Lee, Ji-Yoon;Lee, Somyeong;Shin, Dongjin;Cha, Jinkyeong;Cho, Jun-Hyeon;Kwon, Young-Ho;Jo, Su-min;Park, Dong-Soo
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.65 no.2
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    • pp.79-83
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    • 2020
  • A photoelectric grain color sorter is commonly used in postharvest technology to detect and separate grains considered to be of good quality from those of poor quality based on color. In this study, the milled rice of Haedamssal cultivated under early transplanting conditions was evaluated using the color sorting device. Rice was classified according to standard grades of 64% for grade A, 18% for grade B, and 17.2% for grade C. By head rice ratio analysis, the milled rice of Haedamssal was recorded a grade A of 72.9%, representing a 24.5% improvement compared to the 48.4% of original grains. However, the grades B and C of Haedamssal rice were lower than those of original rice. In addition, grade A whiteness of Haedamssal rice was 45.3, which was lower than that of original grains. The color affinity redness was significantly lower in Haedamssal rice than in the screening control. No significant difference was found in the amylose and protein contents of rice before and after selection by the grain color sorter. In contrast, grain viscosity analysis revealed an increase in peak and final viscosities, while the consistence viscosity increased in low grade levels. Our results suggest that the photoelectric grain color sorter could improve the head rice ratio and palatability of early transplanted Hadamssal rice.

Validity and Reliability of the Korean Version of the Global Assessment of Recent Stress Scale (전반적인 스트레스평가 척도 한국판의 타당도 및 신뢰도)

  • Koh, Kyung-Bong;Park, Joong-Kyu
    • Korean Journal of Psychosomatic Medicine
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    • v.8 no.2
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    • pp.201-211
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    • 2000
  • Objectives : The purpose of this study was to determine the validity and reliablity of the Korean version of the Global Assessment of Recent Stress(GARS) Scale developed for assessing the severity of current perceived stressors, and then to use the scale in clinical practice. Methods : The questionnaire was completed by 215 healthy subjects. A comparison was made regarding the perception of stressors among 242 patients(71 with anxiety disorder, 73 with depressive disorder, 47 with somatoform disorder, and 51 with psychosomatic disorder) and 215 healthy subjects. Factor analysis was done using oblique rotation after maximum-likelihood factor analysis. Results : Factor analysis yielded 1 subscale. Reliability was computed by administering the GARS Scale to 69 healthy subjects during a 2-week interval. Test-retest reliability for 8 items and the total score was significantly high, ranging between 0.85-.95. Internal consistency was significant(Cronbach's a for 7 items : .86). Convergent validity was computed by correlating the 8 items and the total score with the total score of the stress response inventory(SRI), the perceived stress questionnaire(PSQ), and the somatization, anxiety, depression, hostility subscales and general indices of the symptom checklist-90-revised(SCL-90-R). The correlations were all at significant levels. Discriminant validity was computed by comparing the 8 item scores of the patient and control groups. Significant differences were found in area relevant to sickness or illness and the overill global area. The patients with anxiety disorder were significantly higher in area relevant to sickness or illness than the healthy group, whereas the patients with depressive disorder was significantly higher in the overall global score than healthy group. The depressive disorder group was the highest in overall global score of the GARS scale among 4 patient groups, and showed a significant higher scores in interpersonal relationship and overall global area than the psychosomatic disorder group. Conclusions : These results indicate that the Korean version of the GARS scale is highly reliable and valid, and that it can be utilized as an effective measure of perceived stressors for research in stress-related fields. It is suggested that depressive disorder patients are more likely to perceive recent stressors than psychosomatic disorder patients.

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Physicochemical properties of dacquoise made with sugar or sugar replacements, tagatose, and erythritol (타가토스와 에리스리톨로 제조한 다쿠아즈의 물리화학적 특성 연구)

  • Lee, Na Ra;Kang, Suna;Kim, Ji Hyun;Kim, Ha Hyoung;Lee, Jung Ah;Park, Sunmin
    • Journal of Applied Biological Chemistry
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    • v.60 no.1
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    • pp.87-93
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    • 2017
  • The objective of this study was to investigate the physicochemical characteristics and sensory evaluation of dacquoise made with sugar or sugar replacement (tagatose, erythritol). Sugar (S) was replaced with tagatose (T), and sugar powder was replaced with tagatose (TS), and erythritol (TE). S showed the highest sugar contents compared with TS and TE (p <0.05) but there were no significant differences with T. There were not significant differences in specific gravity and spread factor among the sweeteners. S and T tended to have higher loss factors than TS & TE. The more tagatose was increased, the more the redness value increased compared with S, and sucrose showed similar tendency for lightness (redness; TS > T > TE > S, lightness; S > T > TS > TE). TE showed good results for physical characteristics whereas T was better in the sensory test. The above results bear out that tagatose and erythritol both are suitable as alternative sweeteners for making dacquoise, but tagatose was better. Tagatose appeared to be a better replacement for sucrose in baking dacquoise, and dacquoise with 33% of the sugar replaced tagatose (T) could be suitable for use by diabetic and obese people. 95.1% tagatose (TS) maybe appropriate for diabetic patients. In conclusion, tagatose is a suitable sugar replacement for making dacquoise.