• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.85-91
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• 2017

Purpose: The aim of this study was to describe the clinical and biochemical features as well as the molecular analysis of a newly diagnosed illustrative case with ML II and to analyze the clinical features of 11 Korean patients with ML II/III. Method: Including a newly diagnosed patient, total 11 patients in 10 families were diagnosed as ML II (n=7) or ML III (n=4) were enrolled in the study. A diagnosis of ML II or III was made by demonstrating increased lysosomal enzyme activities in the plasma and sequence analysis of GNPTAB with characteristic clinical features. Result: A illustrative case of ML II patient was a 17 month-old boy showing characteristic facial appearance, multiple joint contractures with cardiac involvements. The enzyme assay showed increased lysosomal enzyme activities in the plasma. We identified compound heterozygous mutations in GNPTAB sequence analysis, including a frameshift (c.3428dupA [pAsn1143Lysfs*3]) and a nonsense variant c.673C>T (p.Gln225*). In total 11 patients with ML II/III, the patients with ML II showed severe growth retardation (height standard deviation score -3.2 [${\pm}1.5$]), compare to patients with ML III. Furthermore, patients with ML II patients had serious cardiac problem (n=4), hepatomegaly (n=3) and underwent tracheostomy (n=3) with further respiratory support due to respiratory distress. To improve osteoporosis and bone pain, all patients with ML III and four of 7 patients with ML II treated with intravenous pamidronate. Conclusion: Here we showed a newly diagnosed case of ML II and clinical features of 11 Korean patients with ML II or III. These data could be helpful for further diagnosis of mucolipidosis, a rare inherited metabolic disease, in Korea.

• Radiation Oncology Journal
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• v.14 no.1
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• pp.53-59
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• 1996

Purpose : Radiation-induced alteration in the immune function is well known phenomenon in cancer patients. Our purpose is to evaluate the extent of immune suppression immediately after mediastinal or pelvic irradiation, which include significant volume of active bone marrow in adults. Materials and Methods'48 cancer patients with mediastinal(N=29) and pelvic irradiation(N=19) were the basis of this analysis. Age ranged from 36 to 76 and mean and median value was 57 years, respectively Sex ratio was 1.3(M: F=27/21). The immunological parameters were the complete blood cell(CBC) with differential cell(D/C) count, T cell subset(CD3, CD4, CD8 CDl9), NK cell test(CDl6, CD56), and serum immunoglobulin(IgG, IgA, IgM) level. Results : The mean value of white blood cell(WBC) was reduced from 7017 to 4470 after irradiation(p=0.0000). In the differential count, the number of lymphocyte, neutrophil, and basophil was markedly reduced with statistical significance(p<0.01) and the number of monocyte was not changed and, on the contrary, that of eosinophil was increased by irradiation. In the lymphocyte subpopulation analysis, the number of all subpopulations, CD3(T cell), CD4(helper T cell), CD8(suppressor T cell), CDl6(NK cell), CDl9(B, cell) was reduced with statistical significance. The mean ratio of CD4 to CD8 in all patients was 1.09 initially and reduced to 0.99 after radiotherapy(p=0.34) , but the proportional percentage of all subpopulations was not changed except CD19(B cell) after irradiation. In the immunoglobulin study, initial values of Ig G, Ig A, and Ig M were relatively above the normal range and the only Ig M was statistically significantly reduced after radiotherapy(p=0.02). Conclusion : Mediastinal and pelvic irradiation resulted in remarkable suppression of lymphocyte count in contrast to the relatively good preservation of other components of white blood cells. But the further study on the functional changes of lymphocyte after radiotherapy may be necessary to conclude the effects of the radiation on the immunity of the cancer Patients.

• Journal of Animal Science and Technology
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• v.48 no.2
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• pp.211-218
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• 2006

This experiment was conducted to study the effects of the natural deep sea water, which contained approximately 2.3% salt, and various minerals of K, Mg, Ca, Na, Fe, Mn, Zn, Cu etc, on the immune response and antioxidant activity in rats. 24 Sprague Dawley rats were randomly allotted to a control group and 3 treatment groups. Control rats were supplied with filtered tap water, and each treatment group rats were supplied with 0.5% deep sea water, 1% deep sea water and Jijangsoo, respectively, which is upper clear water separated from sediment by the clay. Feed and water were provided ad libitum throughout the experiment that lasted for 4 weeks. The results showed that 1% deep sea water group showed the highest values in weight gain, feed intake, and feed efficiency than those of other groups. The levels of water intake of 1%- and 0.5%-deep sea water, and Jijangsoo group were 49.1%, 22.8%, and 40.5% higher than that of control group, respectively. The Jijangsoo group rats showed that perirenal and epididymal adipose tissue weights were decreased by 32% and 25%(p<0.05), respectively, when compared to control group rats. There were no remarkable differences of serum glucose concentration among all experimental groups. However, insulin concentration of experimental groups were remarkably increased in order of Jijangsoo (4.54), 1% deep sea water (3.70), 0.5% deep sea water (3.25)(p<0.05). B cell and T cell stimulation were increased about 44.7% and 207%, respectively, by 0.5% deep sea water in comparison with control (p<0.05). TBARS values of 0.5 % deep sea water group were significantly lower than that of control(p<0.05). Catalase and SOD activities of 0.5 % deep sea water group were 200% and 47% higher than that of control, respectively. From the results, it can be concluded that the supply of natural deep sea water can slightly improve the physiological activity which modulates immune response and antioxidant activity in rats.

• Korean Journal of Food Science and Technology
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• v.42 no.1
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• pp.90-96
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• 2010

In this study, the anti-oxidant, anti-tumorigenic, anti-hypertensive, anti-thrombic, anti-diabetic, and anti-inflammatory properties of 18 different species of genus Pleurotus were investigated. In addition, the amino acid, $\beta$-glucan, and polyphenol content were also measured. All species contained more than 20 mg% of polyphenol with the highest contents found in Pleurotus cornucopiae var. citrinopileatus (yellow pleurotus) ($39.13{\pm}0.82\;mg%$). The $\beta$-glucan contents was also the highest in yellow Pleurotus ($37.67{\pm}0.22%$) followed by Won-Hyeong1 (C, $28.75{\pm}0.61%$) and Jang-an PK (A, $27.95{\pm}0.33%$). The yellow Pleurotus exhibited the highest antioxidant activity as assessed by the DPPH scavenging rate with an $IC_{50}$ value of $2.94{\pm}0.44\;mg/mL$. Ethanol extracts from the yellow Pleurotus treated at 1% concentration showed cytotoxic activity up to 36.9% in the human embryonic kidney 293T cell lines. The yellow Pleurotus also showed the highest inhibitory effects on ACE activity ($60.52{\pm}0.2%$). Finally, the yellow Pleurotus exhibited anti-diabetic and anti-inflammatory properties as shown by inhibition of $\alpha$-amyloglucosidase activity ($50.5{\pm}0.8%$) and nitric oxide production ($68.4{\pm}0.3%$). Taken together, our data indicate the yellow pleurotus is a promising functional food ingredients.

• Journal of the East Asian Society of Dietary Life
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• v.17 no.6
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• pp.834-839
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• 2007

This study was designed to determine the optimal ratio of dropwort powder in castella by adding the powder at levels of 0, 3, 6, 9, and 12% respectively. The properties of the castella were analyzed by specific gravity, specific volume, color determinations, texture properties and sensory evaluation. The Specific gravity increased with increasing amount of dropwort powder. However, the specific volume decreased with increasing dropwort powder. For the color values, as more dropwort powder was added, the L-value decreased. The castella with 9% dropwort powder had a higher hardness, gumminess, and chewiness. A sensory panel perceived that the external and internal color of the castella become darker with the dropwort powder substitution and the grain size decreased with increasing amount dropwort powder, while sweet taste showed no significant difference. The order of overall preference was DP 9>DP 6>DP 12>CON>DP 3. Therefore, the substitution of 9% of wheat flour with dropwort powder was recommended in the production of castella.

• Korean Journal of Food Science and Technology
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• v.13 no.4
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• pp.314-318
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• 1981

In order to improve the storage stability of spray and freeze dried red ginseng extract powder packed in a bottle, the water vapor permeability of Al-foil laminate paper used for cap closure and shelf life of those products on various storage temperatures and relative humidities were investigated. The thickness of the laminate paper was $93{\pm}3\:{\mu}m$ and its physical properties were equal to standard of ASTM (B-377-66) The absorption rate of the freeze dried powder was 2-6 times greater than that of the spray dried powder at $37^{\circ}C$. Therefore it was considered that the laminate could be used for cap closures for the spray dried powder but unsuitable for the freeze dried powder. The shelf life of the spray dried powder was longer than that of the freeze-dried powder at $37^{\circ}C$.

• Horticultural Science & Technology
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• v.31 no.5
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• pp.607-616
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• 2013

Light with two faces, beneficial and harmful effects is an important signal for every living cell. Optimal adaptation to light environment enhances the fitness of an organism and survival in nature. Understandings of light quality and plant growth provide with the economical guides for artificial light sources like LEDs. Compared with those under white light, the 1 week seedlings of Chinese cabbage (Brassica rapa) under monochromic red and blue light showed normal development and growth. In contrast to extremely long and etiolated hypocotyls of the seedlings under dark, those under far-red etiolated were extremely short. Based on the microarray analysis, blue light induced the vigorous development and growth and two fold changes of transcripts than red light condition. To have insight of gene products under different light qualities conditions, GO term enrichments were calculated and each gene according to their GO terms were categorized. The blue and red lights affected the expressions of genes related to biological process. Especially, the genes related to metabolic process and developmental process and plastid and chloroplast in the cellular component category were induced under blue light. This study provided the molecular biological evidence for various light qualities on the growing process of B. rapa.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)

• Journal of Chest Surgery
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• v.32 no.2
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• pp.97-107
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• 1999

Background: This study was designed to develop a Korean version of the heparin-coated vascular bypass shunt by using a physical dispersing technique. The safety and effectiveness of the thrombo-resistant shunt were tested in experimental animals. Material and Method: A bypass shunt model was constructed on the descending thoracic aorta of 21 adult mongrel dogs(17.5-25 kg). The animals were divided into groups of no-treatment(CONTROL group; n=3), no-treatment with systemic heparinization(HEPARIN group; n=6), Gott heparin shunt (GOTT group; n=6), or Korean heparin shunt(KIST group; n=6). Parameters observed were complete blood cell counts, coagulation profiles, kidney and liver function(BUN/Cr and AST/ ALT), and surface scanning electron microscope(SSEM) findings. Blood was sampled from the aortic blood distal to the shunt and was compared before the bypass and at 2 hours after the bypass. Result: There were no differences between the groups before the bypass. At bypass 2 hours, platelet level increased in the HEPARIN and GOTT groups(p<0.05), but there were no differences between the groups. Changes in other blood cell counts were insignificant between the groups. Activated clotting time, activated partial thromboplastin time, and thrombin time were prolonged in the HEPARIN group(p<0.05) and differences between the groups were significant(p<0.005). Prothrombin time increased in the GOTT group(p<0.05) without having any differences between the groups. Changes in fibrinogen level were insignificant between the groups. Antithrombin III levels were increased in the HEPARIN and KIST groups(p<0.05), and the inter-group differences were also significant(p<0.05). Protein C level decreased in the HEPARIN group(p<0.05) without having any differences between the groups. BUN levels increased in all groups, especially in the HEPARIN and KIST groups(p<0.05), but there were no differences between the groups. Changes of Cr, AST, and ALT levels were insignificant between the groups. SSEM findings revealed severe aggregation of platelets and other cellular elements in the CONTROL group, and the HEPARIN group showed more adherence of the cellular elements than the GOTT or KIST group. Conclusion: Above results show that the heparin-coated bypass shunts(either GOTT or KIST) can suppress thrombus formation on the surface without inducing bleeding tendencies, while systemic heparinization(HEPARIN) may not be able to block activation of the coagulation system on the surface in contact with foreign materials but increases the bleeding tendencies. We also conclude that the thrombo-resistant effects of the Korean version of heparin shunt(KIST) are similar to those of the commercialized heparin shunt(GOTT).

• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.289-297
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• 2006

Background: Surgery for Stanford type A aortic dissection shows a high operative mortality rate and frequent postoperative brain injury. This study was designed to find out the risk factors leading to operative mortality and brain injury after surgical repair in patients with type A aortic dissection. Material and Method: One hundred and eleven patients with type A aortic dissection who underwent surgical repair between February, 1995 and January 2005 were reviewed retrospectively. There were 99 acute dissections and 12 chronic dissections. Univariate and multivariate analysis were performed to identify risk factors of operative mortality and brain injury. Resuit: Hospital mortality occurred in 6 patients (5.4%). Permanent neurologic deficit occurred in 8 patients (7.2%) and transient neurologic deficit in 4 (3.6%). Overall 1, 5, 7 year survival rate was 94.4, 86.3, and 81.5%, respectively. Univariate analysis revealed 4 risk factors to be statistically significant as predictors of mortality: previous chronic type III dissection, emergency operation, intimal tear in aortic arch, and deep hypothemic circulatory arrest (DHCA) for more than 45 minutes. Multivariate analysis revealed previous chronic type III aortic dissection (odds ratio (OR) 52.2), and DHCA for more than 45 minutes (OR 12.0) as risk factors of operative mortality. Pathological obesity (OR 12.9) and total arch replacement (OR 8.5) were statistically significant risk factors of brain injury in multivariate analysis. Conclusion: The result of surgical repair for Stanford type A aortic dissection was good when we took into account the mortality rate, the incidence of neurologic injury, and the long-term survival rate. Surgery of type A aortic dissection in patients with a history of chronic type III dissection may increase the risk of operative mortality. Special care should be taken and efforts to reduce the hypothermic circulatory arrest time should alway: be kept in mind. Surgeons who are planning to operate on patients with pathological obesity, or total arch replacement should be seriously consider for there is a higher risk of brain injury.