• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.2
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• pp.63-68
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• 2017

Gaucher disease (GD) is caused by the deficiency of glucocerebrosidase. In pediatric patients with GD, especially Type I GD, enzyme replacement therapy (ERT) can reduce the hepatosplenomegaly and improve the hematologic finding and growth velocity. Herein, we report a 2-year-old girl with Type I GD presented with hepatosplenomegaly, bone pain and growth retardation. A 2 year-old-girl was referred to our hospital due to severe hepatosplenomegaly and growth retardation. She suffered from both leg pain and chronic fatigue. Simple x-ray showed widened distal long bones like that of an 'Erlenmeyer flask' which is associated with GD. The laboratory test showed anemia and thrombocytopenia. The enzyme activity was markedly reduced and the direct sequencing of the GBA gene showed the compound heterozygous mutations, p.G46E and p.L444P. As the G46E have been considered as the protective gene against neuronopathic genotype, we could assess the Type I GD in this patient. After one year of ERT, the growth velocity became 11 cm per year. Bone pain and fatigue disappeared. The volume of liver and spleen was reduced from $683cm^3$ and $703cm^3$ to $590cm^3$ and $235cm^3$, respectively. Although GD is an extremely rare disease in Korea, growth retardation and bone pain in children are the important signs which lead to early detection of GD and a simple radiologic finding is helpful to assess the GD at outpatient clinic. We highlight that the early diagnosis and early ERT is important for good growth and outcome for pediatric patients with GD.

• Journal of Life Science
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• v.19 no.3
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• pp.317-321
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• 2009

Down syndrome (DS) results from overexpressed genes on an extra copy of human chromosome 21. Among various phenotypes seen in DS patients, mental retardation, such as learning and memory deficits, is a major factor that prevents DS individuals from leading fully independent lives. The Dyrk1A gene that plays a critical role in neurodevelopment has been isolated from chromosome 21, and transgenic mice with over-expression of Dyrk1A show severe hippocampal dependent learning and memory defects. In the present study, as an initial step to test the treatment of Dyrk1A dependent mental retardation phenotypes in model animals, we isolated human Dyrk1A specific lentiviral short hairpin RNA (shRNA) that inhibits the exogenous human Dyrk1A expression, but not the endogenous mouse expression in transgenic mice with human Dyrk1A overexpression. This limited and specific repression of exogenous human Dyrk1A will prove to be valuable information, if Dyrk1A dependent learning and memory defects in DS patients could be treated or at least ameliorated in vivo.

• Clinical and Experimental Pediatrics
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• v.61 no.11
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• pp.348-354
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• 2018

Purpose: The purpose of this study was to identify the causes, symptoms, and complications of hypoproteinemia to prevent hypoproteinemia and provide appropriate treatment to children with atopic dermatitis. Methods: Children diagnosed with atopic dermatitis with hypoproteinemia and/or hypoalbuminemia were retrospectively reviewed. The patients' medical records, including family history, weight, symptoms, treatment, complications, and laboratory test results for allergies and skin cultures, were examined. Results: Twenty-six patients (24 boys) were enrolled. Seven cases had growth retardation; 7, keratoconjunctivitis; 6, aural discharges; 5, eczema herpeticum; 4, gastrointestinal tract symptoms; and 2, developmental delays. In 21 cases, topical steroids were not used. According to the blood test results, the median values of each parameter were elevated: total IgE, 1,864 U/mL; egg white-specific IgE, $76.5kU_A/L$; milk IgE, $20.5kU_A/L$; peanut IgE, $30kU_A/L$; eosinophil count, $5,810/{\mu}L$; eosinophil cationic protein, $93.45{\mu}g/L$; and platelet count, $666.5{\times}10^3/{\mu}L$. Serum albumin and total protein levels decreased to 2.7 g/dL and 4.25 g/dL, respectively. Regarding electrolyte abnormality, 10 patients had hyponatremia, and 12, hyperkalemia. Systemic antibiotics were used to treat all cases, and an antiviral agent was used in 12 patients. Electrolyte correction was performed in 8 patients. Conclusion: Hypoproteinemia accompanying atopic dermatitis is common in infants younger than 1 year and may occur because of topical steroid treatment continuously being declined or because of eczema herpeticum. It may be accompanied by growth retardation, keratoconjunctivitis, aural discharge, and eczema herpeticum and can be managed through skin care and topical steroid application without intravenous albumin infusion.

• Korean Journal of Food Science and Technology
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• v.15 no.3
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• pp.295-301
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• 1983

The mechanical models representing the theological property of traditional Korean noodles; i.e. wheat flour noodle and wheat-sweet potato starch noddle, were investigated from the data obtained by creep and creep recovery test using a tensile tester. The rheological behavior of the noodle products could be expressed by the 6-elements Voigt model. The instantaneous elasticity, retarded elasticity, retardation time, retarded viscosity and Newtonian viscosity of the noodle products were evaluated. With the increasing cooking time, 4-elements Burger's model was applicable to represent the mechanical behavior of wheat-sweet potato starch noodle.

• Journal of the Korean Society for Precision Engineering
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• v.9 no.3
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• pp.140-148
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• 1992

The effect of mechanical, properties in the plastic zone near the crack-tip was investigated, under various controlled loading conditions, i.e., ${\Delta}K$ increasing, ${\Delta}K$ decreasing, and single overload test. For both ${\Delta}K$ decreasing test and ${\Delta}K$ increasing test with constant stress ratio, it is found that the ratio of material constant m'( ${\Delta}K$ decreasing test) to material constant m( ${\Delta}K$ increasing test) is larger than 1 for n<0.1, and it is equal to 1 for 0.10.2. A modified crack growth rate equation based on Forman's equation which applied stable region of fatigue crack propagation in ${\Delta}K$ decreasing test is proposed. Within the limit of this single overload test, an empirical relation between among the retardation ratio (Nd/ $N^{*}$), the strain hardening exponent (n) and the percent peak load (%PL) has been established.established.

• Transactions of the Korean Society of Automotive Engineers
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• v.9 no.3
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• pp.181-189
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• 2001

For the Tailor Welded Blank sheet used fur automobile body panel, the characteristics of fatigue crack propagation behavior were studied. The thickness of specimen was machined to be same (0.9+0.9mm) and different (0.9+2.0mm). As a base test, mechanical properties around welding zone were examined. The results indicated that there were no significant decreases in mechanical properties, but hardness around welding bead is 2.3 times greater than base material. The crack propagation rate was noticeably decreased around welding line and rapidly increased as it passed by welding line. Reviewing the shape of the crack propagation, crack width around welding line was wide around the welding zone due to retardation of crack growth, but it became narrow passing welding line due to decreased toughness. Elasto-Plastic analysis was performed by finite element analysis fur explaining the test results.

• Journal of the Korean Society of Food Science and Nutrition
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• v.23 no.2
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• pp.298-302
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• 1994

This study is carried out in order to investigate to the physical property of Castella. The material is 19 kinds of Castella on the market in Kyushu area.As an result of the sensory evaluation for Castella, it could be classified into 3 types ; a) high grade, C) low grade, and B) midium grade.The results are summarized as follow. The water content of 3 type Castella was most 26-30% but Korea's was more lower . ΔE of A an dB type Castella was similiar , but A and C types was very different. in the Creep test, 3 types are all the 6-element Voigt model, consisting of Hookean body , Newtonian body and two sets of Voigt body. Eo of A type is 13-36% higher than other types. The parts of retardation strain of A type are 21-41% lower than B type, 8-13% higher than C type, respectively.

• Biomolecules & Therapeutics
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• v.10 no.4
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• pp.258-267
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• 2002

In this study the general pharmacological profiles of AS6 on the central nervous system, cardiovascular and the other organs were investigated. The dosages given were 0, 250, 500 and 1000 mg/kg and drugs were orally administered. The animals used for this study were mice, rats and guinea pigs. Significant increases (p<0.01) in the charcoal transport capacity were observed at the high dose of 1000 mg/kg and significant increases in retardation of pain threshold were observed in the test using acetic acid in all dosed animals. However, AS6 showed no noticeable effects on general behavior, motor coordination, spontaneous locomotor activity, hexobarbital-induced sleep time, body temperature, analgesic activity in the test using hot plate method and anticonvulsant activity. Furthermore no noticeable effects were observed in cardiovascular functions in the isolated rat heart, contraction and relaxation of the smooth muscle in the isolated guinea ileum, gastric secretion and renal function.

• Journal of Korean Society of Environmental Engineers
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• v.22 no.1
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• pp.33-42
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• 2000

Adsorption onto the surfaces of solid particles is a well known phenomenon that causes the retardation effect of heavy metals in soils. For adequate remediation of soil and groundwater contamination, it is important to investigate the mobility of heavy metals that largely depends on pH conditions in the soil water since adsorption of heavy metals is pH-dependent. In this study, we investigated the transport of Zn ion under various pH conditions in a sandy soil by conducting batch and column tests. The batch test was performed using the standard procedure of equilibrating fine fractions collected from the soil with eleven different initial $ZnCl_2$ concentrations, and analysis of Zn ion in the equilibrated solutions using ICP-AES. The column test consisted of monitoring the concentrations of soil solutions exiting the soil column with time known as a breakthrough curve (BTC). We injected respectively $ZnCl_2$ and KCl solutions with the concentration of 10 g/L as a tracer in a square pulse type under three different pH conditions (7.7, 5.8, 4.1) and monitored the flux concentration at the exit boundary using an EC meter and ICP-AES. The resident concentration was also monitored at the 10cm-depth by Time Domain Reflectometry (TDR). The results of batch test showed that ion exchange process between Zn and other cations (Ca, Mg) was predominant. The retardation coefficients obtained from adsorption isotherms (Linear, Freundlich, Langmuir) resulted in the various values ranging from 1.2 to 614.1. No retardation effect but ion exchange was found for the BTCs under all pH conditions. This can be explained by the absence of other cations to desorb Zn ion from soil exchange sites under the conditions of ETC experiment imposing blank water as leachate in steady-state flow. As pH decreased, the peak concentration of Zn increased due to the competition of Zn with hydrogen ions ($H^+$) and the concentrations of other cations decreased. The peak concentration of Zn was increased by 12.7 times as pH decreased from 7.7 to 4.1.

• Journal of the Korean Graphic Arts Communication Society
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• v.23 no.2
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• pp.1-14
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• 2005

Generally, printing inks are composed of pigment, vehicle and additive. Among others, the vehicle transfers the pigment to substrate and then binds it on the surface. So, rheological properties of the vehicle are an important factor which has influence on printability. Thus, in this study, rheology of the vehicle was investigated by using rotational rheometer according to molecular weight of resin. Also, emlusion rheology of water in oil type and its microstructure were examined with increasing the shear rate. Consequently, the following results were obtained: (1) By viscometric flow test, zero shear viscosity and shear thinning index of vehicle increased with increasing the molecular weight of resin. (2) By relaxation and creep test, relaxation time and retardation time of vehicle increased with increasing the molecular weight of resin. (3) By frequency sweep test, crossover point of vehicle increased with increasing the molecular weight of resin. (4) G' and G" of emlusions increased with increasing the molecular weight by amplitude sweep test. (5) The shape of water drop in emlusions was changed to the capillary tube.