• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.15-21
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• 2021

Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.

• Journal of Bio-Environment Control
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• v.27 no.2
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• pp.140-146
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• 2018

This research was conducted to determine the influence of incorporation levels of sulfur into a coir dust+pine bark medium (1:1, v/v) on the changes in the bicarbonate ($HCO_3{^-}$) concentrations and pH of soil solution, crop growth, and nutrient uptake of 'Ssanta' strawberry. In the preparing of the mixed medium, sulfur powder was added with the rate of 0 (control), 0.23, 0.45, 0.90, and $1.80g{\cdot}L^{-1}$ and Hoagland nutrient solution containing $240mg{\cdot}L^{-1}$ $HCO_3{^-}$ was supplied during the crop cultivation. The growth measurements and tissue analysis for the determination of nutrient contents were carried out 140 days after solution application and the soil solution analysis was performed every two weeks. As the level of sulfur was elevated, the overall growth of mother plants became better showing that the growth indexes except chlorophyll contents were the lowest in control treatment but the statistical differences were not found among the three treatments of $0.45g{\cdot}L^{-1}$ or higher sulfur. The higher the levels of sulfur incorporation, the higher the occurrence of runners and the growth of daughter plants. The length of the runners and the number of daughter plants occurred per mother plants were higher in the treatments of 0.90 and $1.80g{\cdot}L^{-1}$ than the three treatments of 0, 0.23, and $0.45g{\cdot}L^{-1}$, but the statistical differences were not observed between the 0.90 and $1.80g{\cdot}L^{-1}$ treatments. The rose of pH and $HCO_3{^-}$ concentrations in soil solution of root media continued all the cropping period, but those decreased slightly in the treatments of $0.90g{\cdot}L^{-1}$ or higher. The soil solution concentrations of $K^+$ and $PO_4{^3-}$ in the treatments of 0.90 and $1.80g{\cdot}L^{-1}$ was lower than those in other treatments and the statistical differences in the $Ca^{2+}$ and $Mg^{2+}$ concentrations were not observed among all treatments. The nutrient contents in tissue analyzed in this experiment were the lowest in the control treatments and those increased as incorporation rates of sulfur were elevated. Above results indicated that when 'Ssanta' strawberry is grown hydroponically and the root medium is coir dust and the pine bark (5:5, v/v) mix, the sulfur incorporation rate as pre-planting fertilizer has to be higher than $0.9g{\cdot}L^{-1}$ root medium to reduce the $HCO_3{^-}$ injury.

• Horticultural Science & Technology
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• v.32 no.2
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• pp.149-156
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• 2014

The objective of this research was to investigate the influence of bicarbonate ($HCO_3{^-}$) concentrations in nutrient solution on the growth and physiological disorders in mother plants and occurrence of daughter plants in propagation of 'Seolhyang' strawberry (Fragaria ${\times}$ ananassa Duch.). To achieve this, the mother plants were transplanted to coir dust + perlite (7:3, v/v) medium and Hoagland solution was modified to contain 60, 90 (control), 120, 180, and $240mg{\cdot}L^{-1}$ of $HCO_3{^-}$. The symptoms of Fe, Zn and B deficiencies appeared on the mother plants in the $HCO_3{^-}$ of $240mg{\cdot}L^{-1}$ from the 60th day after treatment. The symptoms spread to all plants in the $240mg{\cdot}L^{-1}$ $HCO_3{^-}$ including daughter plants at 90 days after treatment. The$HCO_3{^-}$ concentrations higher than $120mg{\cdot}L^{-1}$ suppressed the growth of mother plants such as leaf number, chlorophyll content, fresh weight and other growth parameters. While the mother plants in $60mg{\cdot}L^{-1}$ $HCO_3{^-}$ produced 23 daughter plants, while mother plants in $240mg{\cdot}L^{-1}$ $HCO_3{^-}$ produced 13 daughter plants. The final pH 126 days after treatment in the $HCO_3{^-}$ of 60, 90, 120, 180 and $240mg{\cdot}L^{-1}$ were 5.4, 5.8, 7.3, 7.9, and 8.3, respectively. The elevation of $HCO_3{^-}$ concentrations in nutrient solution resulted in the decrease of Fe, Mn, Zn and Cu contents of above ground tissue 126 days after treatment. These results indicate that the $HCO_3{^-}$ concentrations higher than $120mg{\cdot}L^{-1}$ inhibited the growth of mother plants and occurrence of daughter plants in vegetative propagation of 'Seolhyang' strawberry.

• The Korean Journal of Malacology
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• v.24 no.3
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• pp.229-241
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• 2008

Changes of growth and histopathological feature in the mantle structure of the equilateral venus, Gomphina veneriformis exposed to tribultyltin chloride (TBTCl) for 36 weeks were observed. Concentrations of TBTCl were 0, 0.4, 0.6, and $0.8{\mu}g/L$. A regression analysis by power function of SPSS was shown that the growth of experimental groups was significantly decreased after 12 weeks of exposure. For histological analysis, mantle tissues were characterized using H-E stain, AB-PAS (pH 2.5) reaction and Masson's trichrome stain, and epidermal layer thickness and mucous cell distribution were analysed using the image analyser. The mantle had 4-folds (inner-inner, inner-outer, middle, and outer) and its epidermal layer consisted of simple epithlia. A periostracum was observed in the periostracal groove between middle and outer fold. Inner epidermal layer consisted of simple ciliated columnar epithelia, but the outer epidermal layer consisted of simple non-ciliated columnar epithelia. Alcian blue positive mucous cells showed blue color (7462c, 653c) in the inner fold, violet color (2583c) in the middle fold, and blue color (647c, 7455c) in inner epidermal layer (numbers in the parenthesis are codes of Pantone process coated color). Hemolymph sinus in the mantle was extended, and mucous cells in inner plica of the middle fold were stained as blue (7455c) and violet (2587c), after 12 weeks of TBTCI exposure. Cilia and striated border were disappeared, and number of mucous cells in the inner epidermal layer was reduced. Serious histopathological changes in middle and outer fold near the periostracum were observed after 36 weeks. Moreover, epidermal layer thickness and mucous cell distribution were showed decreasing tendency as exposure time to TBTCI was increased. Results of this study suggested that TBTCl induced growth disorder with histopathological changes.

• Journal of Bio-Environment Control
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• v.30 no.1
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• pp.85-93
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• 2021

In hydroponic cultivation, in order to investigate the change of lettuce growth and physiologically active substances through CO2 tablet treatment in nutrient solution, we used a solid carbonated tablets commercially available in the Netherlands. The experiment consisted of 0.5-fold, 1-fold, and 2-fold treatment groups with no treatment as a control. As a result, the atmospheric CO2 concentration in the chamber after CO2 tablet treatment showed the highest value at 472.2 µL·L-1 in the 2-fold treatment zone immediately after treatment, and the pH in the nutrient solution decreased the most to pH 6.03 in the 2-fold treatment zone. After that, over time, the CO2 concentration and pH recovered to the level before treatment. Leaf width and leaf area of lettuce showed the highest values of 17.1cm and 1067.14 ㎠ when treated 2-fold with CO2 tablet, while fresh weight and dry weight of the above-ground part were highest at 63.87 g and 3.08 g in 0.5-fold treatment. The root length of lettuce was the longest (28.4 cm) in the control, but there was no significant difference in the fresh weight and the dry weight among the treatments. Apparently, it was observed that the root length of the lettuce was shortened by CO2 tablet treatment and a lot of side roots occurred. In addition, there was a growth disorder in which the roots turned black, but it was found that there was no negative effect on the growth of the above-ground part. As a result of analyzing the bioactive compounds of lettuce by CO2 tablet treatment, chlorogenic acid and quercetin were detected. As a result of quantitative analysis, chlorogenic acid increased by 249% compared to the control in 1-fold treatment, but quercetin decreased by 37%. As a result of comparing the DPPH radical scavenging ability showing antioxidant activity, the control and 0.5-fold treatment showed significantly higher values than the 1-fold and 2-fold treatments. This suggests that carbonated water treatment is effective in increasing the growth and bioactive compounds of hydroponic lettuce.

• Asian-Australasian Journal of Animal Sciences
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• v.9 no.3
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• pp.319-329
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• 1996

During the monsoon in Bangladesh, the possibility of preserving wet ($700g\;H_2O$) straw by urea (50 g/kg straw DM) with or without polythene cover has been studied. The quality of preserved straw (PS) in terms of colour, smell and fungal infestation were recorded. Nutritive value of the PS was compared to that of a dry straw (DS) in two separate feeding trials on growing bulls (about 290 kg) without (Expt. 1) or with (Expt. 2) concentrate supplements. Over 96% of the wet straw was excellently preserved for over 5 months when covered with polythene in horizontal heaps (of appx. 4 tons). Whereas only 33% of the straw was well preserved in the uncovered (dome shaped) heaps (of approximately 9.5 tons). Each ton of wet straw costed Tk. 1413 and its preservation cost incurred Tk. 345. Urea preservation increased the crude protein content (95 vs. 50 g/kg), dry matter (DM) degradability at all (8, 16, 24, 48, 72 and 96) hours of incubation and at 48 hours, DM degradability (%) were 45 and 25 respetively for the PS and the DS. When fed alone, DM intake (75 vs. $106g/kg\;W^{0.75}/d$), total microbial N yield (27 vs. 54 g/d) and growth rate (-379 vs. 283 g/d) were higher (p < 0.01) in the PS than the DS. Supplementation of concentrate reduced the straw DM intake both in the DS ($51g/kg\;W^{0.75}/d$) and the PS ($958g/kg\;W^{0.75}/d$), but the substitution rate (SR%) was higher in the PS (42) than the DS (27). Higher substitution rate was probably responsible for the reduction in the differences between the DS and PS in their nutrient digestibilities, total microbial N yield (62 vs. 64 g/d) and growth rate(669 vs 339 g/d) when supplemented with concentrate. On 28th day of Expt. 2, feeding PS from one of the polythene covered heaps resulted nervous disorder due to unknown reason(s). Further studies on the effect of size and shape of heap on the preservation quality need to be determined.

• Korean Journal of Organic Agriculture
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• v.14 no.2
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• pp.179-189
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• 2006

After examining the difference in the photosynthesis rate of corn according to the planting distance, the distance of $40{\times}40cm$ showed the lowest rate by 23 days after transplanting (May 31); however, there was no significant difference in the photosynthesis rate due to increased salt tolerance in the plant as time went by. As for the difference in growth features of a plant, the planting distance of $40{\times}40cm$ showed a growing disorder due to the influence of salt by 23 days after trans-plantation (May 31); however, there was a desirable growth as time went by. For the difference in the salt content within a plant, the planting distance of $40{\times}40cm$ tended to be higher than other planting distances, and the K+ content is much higher than other kinds of salt after examining the difference in salt absorption. As for the correlation between saline components within a plant, there was a significant negative correlation among $K^{+},\;Ca^{2+},\;and\;Na^{+}$ while there was a significant positive correlation among $Mg^{2+},\;Ca^{2+}$, and Na+. After examining the effectiveness of salt removal from soil according to com cultivation, the planting distance of $30{\times}30cm$ showed a remarkable decrease by 37 days, compared with 23 days, after transplantation in $K^{+}$ by 28%, $Ca^{2+}$ by 36.6%, $Mg^{2+}$ by 30.6%, and $Na^{+}$ by 22.9%. And the salt content is higher in surface soil than in subsoil.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.639-644
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• 2009

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-$\beta$ receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.601-606
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• 2009

Amelogenesis Imperfecta (AI) is a genetic disorder which retards the development of enamel and it can be classified into three types: hypoplastic, hypomaturation, hypocalcified type. This can occur both in deciduous and permanent dentition. A 8 year 8 month old patient with a chief complaints of delayed eruption on upper anteriors, calculus deposit on lower anteriors and anterior openbite visited the clinic. Anteriors had thin layer of enamel and were very narrow. Especially lower anteriors had rough surface and were in bad shape. Teeth were very hypersensitive to thermal changes. Upper and lower first molars showed severe attrition on the occlusal surface. Radiographs also verified hypoplastic enamel in the whole dentition including the teeth in the tooth bud. The patient was diagnosed as hypoplastic AI, and is being treated at the pediathc and prosthodontic department of the Kyunghee dental university hospital. To improve the function, esthetics, hypersensitivity of the AI patients, restorations on the posteriors and the anteriors with oral hygiene instruction are necessary, Constant follow-up check is needed until full growth and after full growth, cooperative care with the other department is needed.

• The Korean Journal of Physiology and Pharmacology
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• v.22 no.4
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• pp.409-417
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• 2018

Pre-eclampsia (PE) is a pregnancy disorder that is characterised by severe hypertension and increased risks of foetal and maternal mortality. The aetiology of PE not completely understood; however, maternal nutrition and oxidative stress play important roles in the development of hypertension. The treatment options for PE are currently limited to anti-hypertensive drugs. Punicalagin, a polyphenol present in pomegranate juice, has a range of bioactive properties. The effects of supplementation with punicalagin on angiogenesis and oxidative stress in pregnant rats with induced hypertension were investigated. The pregnant rats were randomly divided into five experimental groups (n=12 per group). Hypertension was induced using an oral dose of NG-nitro-L-arginine methyl ester (L-NAME, 50 mg/kg/day) on days 14-19 of pregnancy. Punicalagin (25, 50 or 100 mg/kg) was given orally on days 14-21 of pregnancy. Punicalagin treatment at the tested doses significantly reduced diastolic, systolic, and mean arterial blood pressure in L-NAME treated rats from day 14. Punicalagin also restored angiogenic balance by increasing the expression of vascular endothelial growth factor and downregulating vascular endothelial growth factor receptor-1/fms-like tyrosine kinase-1. Punicalagin, significantly increased the placental nitric oxide levels as compared to PE group. The increased levels of oxidative stress in rats with PE were markedly decreased by treatment with punicalagin. Punicalagin at the tested doses markedly (p<0.05) enhanced the placental antioxidant capacity in L-NAME-treated rats. The raised catalase activity observed following L-NAME induction was significantly (p<0.05) and restored to normal activity levels in punicalagin treatment. Further, 100 mg dose of punicalagin exhibited higher protective effects as compared to lower doses of 25 and 50 mg. This study shows that supplementation with punicalagin decreased blood pressure and oxidative stress and restored angiogenic balance in pregnant rats with induced PE.