• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• Korean Journal of Animal Reproduction
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• v.23 no.2
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• pp.175-180
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• 1999

Blood chemical values and sex steroid hormone concentration of repeat breeder and reproductive disorder in Hanwoo, which were bred in Chunchon areas, were examine to establish the basic physiological maker. The concentrations of albumin, blood urea nitrogen(BUN), calcium(Ca), cholesterol, creatinine, glucose, phosphorous, total protein and triglycerides in repeat breeder and reproductive disorder were 3.28$\pm$0.05 and 3.27$\pm$0.04 g/㎗, 11.07$\pm$0.61 and 12.69$\pm$0.88 mg/㎗, 9.98$\pm$0.10 and 9.56$\pm$0.11mg/㎗, 105.75$\pm$3.57 and 126.78$\pm$5.66mg/㎗, 1.62$\pm$0.05 and 1.65$\pm$0.09 mg/㎗, 67.46$\pm$9.07 and 76.97$\pm$3.76mg/㎗, 6.13$\pm$0.36 and 6.11$\pm$0.26mg/㎗, 5.82$\pm$0.08 and 6.18$\pm$0.08 g/㎗ , 12.82$\pm$1.46 and 15.19$\pm$1.61mg/㎗, respectively. Among the blood chemical values, Ca, cholesterol and inorganic phosphorous levels in repeat breeder and reproductive disorder were slightly higher than those in normal cows. The progesterone levels of pregnancy(0.18~6.56 ng/$m\ell$) and non-pregnancy(0.15~5.46ng/$m\ell$) cows at estrus cycle were showed similar trends from 0 day to 18 days. Those of pregnancy cows were increased gradually from 18 days to 180 days(5.90~7.51 ng/$m\ell$), while those of non-pregnancy cows were dramatically drop at 21 days (0.18$\pm$0.03mg/㎗). The concentrations of estrogen and progesterone in repeat breeder and reproductive disorder were 57.42$\pm$5.03, 56.76$\pm$8.73 pg/$m\ell$ and 6.26$\pm$0.83, 9.41$\pm$1.85 ng/$m\ell$ . respectively. These results indicate that blood chemical values in repeat breeder and reproductive disorder were not greatly difference in normal cows except for Ca, cholesterol, BUN and inorganic phosphorous. Since the progesterone levels in repeat breeder and reproductive disorder was maintained to the luteal phase levels of normal cows, it may cause of failing of conception and next estrus cycle.

• Korean Journal of Food Science and Technology
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• v.29 no.2
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• pp.230-239
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• 1997

In order to investigate the composition and the actual condition of extractive nitrogenous constituents in Tohajeot (a salted and fermented freshwater shrimp, Caridina denticulata denticulata) and in seasoned Tohajeot which were sold in the markets, the extract was analyzed separately into extractive nitrogen, free amino acids, oligopeptides, nucleotides and related compounds, quaternary ammonium bases, and guanidino compounds, using specimens collected at the fish markets of Yosu and Naju cities in 1994 and 1995. The salinity of Tohajeot was very high $(23.6{\sim}25.1)%$, but seasoned Tohajeot was relatively low $(8.4{\sim}11.4%)$. The extractive nitrogen in the extracts of Tohajeot and seasoned Tohajeot was $311{\sim}531\;mg\;and\;256{\sim}429\;mg$, and the total of free amino acids in them were $1,159{\sim}2,584\;mg\;and\;1,012{\sim}1,672\;mg$ respectively. Glutamic acid, leucine, lysine, histidine, alanine, ornithine, and tyrosine were the major amino acids in Tohajeot extract, and glutamic acid, lysine, arginine, aspartic acid, histidine, leucine and alanine were the main amino acids in seasoned Tohajeot. As for nucleotides and related compounds in them were $2.64{\sim}4.82\;{\mu}mol\;and\;1.08{\sim}1.93\;{\mu}mol$ respectively. Homarine, trigonelline, glycinebetaine and ${\beta}-alaninebetaine$ were detected in them. Homarine was the most abundant, ranging from 18 mg to 86 mg, but the others were very low. The content of major nitrogenous constituents in Tohajeot extract, such as extractive nitrogen, free amino acids, oligopeptides, nucleotides and related compounds, and betaines, was more abundant than that in seasoned Tohajeot extract. But the nitrogenous constituents of Tohajeot extract were poorer than those of anchovy sauce which was sold in the markets. Possibly, the extractive nitrogenous components, which consisted of total betains, total free amino acids, and phenylalanine might be recommended as the quality indices of standardizing Tohajeot and seasoned Tohajeot.

• Korean Journal of Food Science and Technology
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• v.29 no.2
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• pp.211-217
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• 1997

The physical and chemical stability of anthocyanins from a Korean pigmented rice variety was investigated at various conditions of pH, temperature, metal ion, sugar, organic acid and light. The anthocyanin pigments were relatively stable with half-lives of 36 days (pH 2.0) and 17 days (pH 3.0), while they were decomposed in a day at neutral and basic pH of 7.0 and 9.0 at $25^{\circ}C$. The anthocyanins also showed high thermal stability at pH 3.0; the half-lives were 7.4 hr, 23.6 hr and 96.3 hr at $95^{\circ}C,\;75^{\circ}C\;and\;50^{\circ}$, respectively. Addition of di- and tri-valent metal ions at pH 3.0 resulted in the increase of color intensity and stability throughout 21 days of storage periods at $25^{\circ}C$. Most sugars added accelerated the degradation of anthocyanin pigments, so that fructose showed the greatest degradation effect on the pigments. Addition of citric acid at pH 3.0 increased stability of anthocyanins, while tartaric acid decreased stability. The anthocyanins were very sensitive on light irradiation with a degradation half-life of 14 hr under 20,000 lux-light dosage at pH 3.0.

• Korean Journal of Poultry Science
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• v.12 no.1
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• pp.17-24
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• 1985

In order to investigate an effect of non-protein nitrogen on the biological utilization of protein, hatched single comb White Leghorn male chicks were fed for the first 8 days with a commercial chicks mash, next 6 days with protein-free diet and subsequent 6 days with protein-free diets and protein diets containing 10.59% of crude protein supplemented with 0, 0.5, 1.0 and 1.5%, respectively. During experimental feeding period, chicks fed protein-free diets had intaked gradually lower feed and had shown a similar body weight loss though urea contents were increased. When birds fed protein diets, body weight gain and feed intake were not different among birds fed the graded levels of urea although feed conversions were shown a highering tendency along with increasing urea contents. According as supplemented urea were increased, protein efficiency ratio f (PER) and net protein ratio (NPR) were increased in chicks fed protein-free diets, which were shown a lowering trend in chicks fed protein diets. Effect of supplemented urea on the urinary excretion of uric acid were not found in birds fed protein-free diets, while which were increased in birds fed protein diets with the increase of urea contents. Urea addition did not affect the excretion of total creatine in birds fed protein-free or protein diets. Excretion of ammonia was jogjered in order to increasing level of urea in birds fed protein-free diets, but which were not found any particular effect in birds fed protein diets. Also urea excretion were gradually increased with the increasing contents of urea in protein-free and protein diets. Nitrogen balance of birds fed protein-free diets were minus values, which were increased with increasing urea contents in diets. When birds fed protein diets, nitrogen balance and urinary nitrogen excretion was highered and fecal nitrogen excretion were not altered as urea levels of diets increased. Digestibility of urea nitrogen supplemented in protein-free diets were lowered along with increasing contents of urea, but biological value(BV) and net protein utilization(NPU) was found a highering tendency in birds fed protein-free diet containing 1.5% of urea. When birds fed with protein diets, digestibility, BV and NPU of protein were found a highering trend in birds fed protein diets added with 0.5% of urea.

• Korean Journal of Poultry Science
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• v.36 no.2
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• pp.165-175
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• 2009

Fatty liver-hemorrhagic syndrome (FLHS) is a common nutritional disease in commercial layers and breeders. The most important clinical sign of FLHS is a sudden drop in egg production and increased mortality which causes significant economic loss in the poultry industry. However, the current diagnostic method for FLHS is based on the gross findings at necropsy which is not helpful to reduce the economic loss because of lateness of diagnosis. Therefore, we need early diagnosis and diagnostic methods before chickens were affected by FLHS. In this study we tried to evaluate the effectiveness of clinical pathology including blood chemistry as an early diagnostic method for FLHS in commercial chickens. Profiles of blood biochemistry were compared between two flocks selected in the same commercial layer farm based on the presence of FLHS clinical sings. A flock with clinical signs of FLHS was designated as FLHS and other flock without clinical signs of FLHS as Non-FLHS. Several parameters of blood biochemistry were selected and compared between FLHS and Non-FLHS to evaluate the possibility of early diagnosis. Average concentrations of serum cholesterol, serum calcium, aspartate aminotransferase (AST), lactate dehydrogenase (LDH) and creatine kinase (CK) were $139.4\;{\pm}\;87.2$ (mg/dL), $24.5\;{\pm}\;5.4$ (mg/dL), $153.6\;{\pm}\;23.1$ (IU/L), $1238.3\;{\pm}\;475.2$ (IU/L) and $1107.3\;{\pm}\;422.8$ (IU/L) in Non-FLHS flock, respectively, and $210.2\;{\pm}\;173.2$ (mg/dL), $25.2\;{\pm}\;4.1$ (mg/dL), $174.3\;{\pm}\;53.5$ (IU/L), $1694.9\;{\pm}\;691.3$ (IU/L) and $1104.9\;{\pm}\;472.9$ (IU/L) in FLHS flock, respectively. The activities of serum cholesterol, AST and LDH except CK, were significantly higher in FLHS than those in Non-FLHS flock (p<0.05). Some birds of FLHS flock showed 2~17 times greater than in Non-FLHS flock. For the definitive diagnosis of FLHS in the flocks tested for blood chemistry, we analyzed fat content and histological lesion score in the liver sampled from both FLHS and Non-FLHS flock. Average liver fat contents based on dry weight were $16.1\;{\pm}\;0.4$ (%) in Non-FLHS flock and were $21.6\;{\pm}\;16.0$ (%) in FLHS flock. These result confirmed that FLHS flock was definitely affected by FLHS. The above results suggest that selected parameters of blood biochemistry, particularly AST, could be useful to diagnose FLHS before significant liver damage occurred in commercial layers.

• The Journal of Korean Physical Therapy
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• v.15 no.2
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• pp.19-51
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• 2003

To demonstrate the effect of massages on the skeletal muscles of immobilization ddY mice models induced by right side sciatic nerve neurectomy, the cross sectional histological profiles of the muscularis (M) gastrocnemius, M. tibialis cranialis and M. tibialis caudalis were observed after 28 days of treatment of massages with the changes of body weight, thickness of hind limb and individual muscle weights. In addition, changes of serum aspartate amino- transferase (AST) and creatine phosphokinase (CK) levels were also demonstrated with diameter of individual muscle fiber and muscle fasciculata, and number of muscle fiber in each of three types of muscles located in the calf. The experimental groups were divided into five groups, 1) Sham-operated group (Sham), 2) Neurectomized but not massage control group (Control), 3) Neurectomized and massage at knee joint regions (T1), 4) Neurectomized and massage at calf regions (T2), and 5) Neurectomized and massages at achilles tendon regions (T3). The experimental animals were used 5 per groups. The results are as follow: 1. No significant changes of body weight were demonstrated in this groups during whole experimental periods compared to that of Sham group. In addition, no significant changes of body weight gains during experimental periods (Day 0 - Day 27: 28 days of treatment) were also observed. 2. The thickness of intact left side hind limb was not changes in all tested groups compared to that of Sham group. However, the thickness of right side hind limb were remarkedly decreased in Control, T1, T2 and T3 groups compared to that of Sham group. In addition, the differences of thickness of hind limb between right and left sides were also significantly increased in Control and massage groups (T1, T2 and T3) compared to that of Sham group. However, the thickness of right side of hind limb were significantly increased and the differences of thickness between both sides of hind limb were dramatically decreased in massage groups compared to that of Control group in followed order: T1 > T3 > T2. 3. Absolute and relative weight of M. gastrocnemius in the intact left side of hind limb was not changes in all tested groups compared to that of Sham group. However, the weight of right side M. gastrocnemius were remarkedly decreased in Control, T1, T2 and T3 groups compared to that of Sham group. In addition, the differences of weight of M. gastrocnemius between right and left sides were also significantly increased in Control and massage groups compared to that of Sham group. However, the weight of right side M. gastrocnemius were significantly increased and the differences of weight between both sides of M. gastrocnemius were dramatically decreased in massage groups compared to that of Control group in followed order: T3 > T1 >. 4. Absolute and relative weight of M. tibialis cranialis in the intact left side was not changes in all tested groups compared to that of Sham group. However, the weight of right side M. tibialis cranialis were remarkedly decreased in Control, T1, T2 and T3 groups compared to that of Sham group. In addition, the differences of weight of M. tibialis cranialis between right and left sides were also significantly increased in Control and massage groups compared to that of Sham group. However, the weight of right side M. tibialis cranialis were significantly increased and . the differences of weight between both sides of M. tibialis cranialis were dramatically decreased in massage groups compared to that of Control group in followed order: T3 > T1 > T2. 5. Absolute and relative weight of M. tibialis caudalis in the intact left side was not changes in all tested groups compared to that of Sham group. However, the weight of right side M. tibialis caudalis were remarkedly decreased in Control, T1, T2 and T3 groups compared to that of Sham group. In addition, the differences of weight of M. tibialis caudalis between right and left sides were also significantly increased in Control and massage groups compared to that of Sham group. However, the weight of right side M. tibialis caudalis were significantly increased and the differences of weight between both sides of M. tibialis caudalis were dramatically decreased in massage groups compared to that of Control group in followed order: T3 > T1 > T2. 6. A significant increase of serum AST levels were demonstrated in Control group. However, serum AST levels of massage groups were significantly decreased compared to that of Control group in followed order: T3 > T1 > T2. In case of 73 group, the values decreased into similar levels compared to that of Sham group. 7. A significant increase of serum CK levels were demonstrated in Control group. However, serum CK levels of massage groups were significantly decreased compared to that of Control group in followed order: T3 > T1 > T2.

• Investigative Magnetic Resonance Imaging
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• v.8 no.2
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• pp.94-99
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• 2004

Purpose : To investigate the signal enhancement ratio by NOE effect on in vivo $^{31}P$ MRS in human heart muscle and liver. we also evaluated the enhancement ratios of different phosphorus metabolites, which are important in 31P MRS for each organ. Materials and Methods : Ten normal subjects (M:F = 8:2, age range = 24-32 yrs) were included for in vivo $^{31}P$ MRS measurements on a 1.5 T whole-body MRI/MRS system using $^1H-^{31}P$ dual tuned surface coil. Two-dimensional Chemical Shift Imaging (2D CSI) pulse sequence for $^{31}P$ MRS was employed in all $^{31}P$ MRS measurements. First, $^{31}P$ MRS performed without NOE effect and then the same 2D CSI data acquisitions were repeated with NOE effect. After postprocessing the MRS raw data in the time domain, the signal enhancements in percent were estimated from the major metabolites. Results : The calculated NOE enhancement for liver $^{31}P$ MRS were $\alpha-ATP\;(7\%),\;\beta-ATP\;(9\%),\;\gamma-ATP\;(17\%),\;Pi\;(1\%),\;PDE\;(19\%)$ and $PME\;(31\%)$. Because there is no creatine kinase activity in liver, PCr signal is absent. For cardiac $^{31}P$ MRS, whole body coil gave better scout images and thus better localization than surface coil. In $^{31}P$cardiac multi-voxel spectra, DPG signal increased from left to right according to the amount of blood included. The calculated enhancement for cardiac $^{31}P$ MRS were : $\alpha-ATP\;(12\%),\;\beta-ATP\;(19\%),\;\gamma-ATP\;(30\%),\;PCr\;(34\%),\;Pi\;(20\%),\;(PDE)\;(51\%),\;and\;DPG\;(72\%)$. Conclusion : Our results revealed that the NOE effect was more pronounced in heart muscle than in liver with different coupling to 1H spin system and thus different heteronuclear cross-relaxation.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.882-890
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• 2007

Purpose : Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental sequale in perterm VLBW. Methods : Subjects were 26 preterm infants whose birth weight are less than 1,500 g and delivered under the intrauterine period 34 weeks. Autologous umbilical mononuclear cells (about $5.87{\times}10^7/kg$) were injected to neonate via the umbilical vein on the postnatal 24-48 hour. The therapeutic efficacy was assessed by numbers of nucleated RBC, urinary uric acid/creatinine ratio, concentration of neuron specific enolase (NSE), interleukin 6 (IL6), interleukin-$1{\beta}$ ($IL-1{\beta}$), and glial cell derived neurotrophic factor (GDNF) in serum and cerebrospinal fluid on day 1 and 7. Results : There were no significant differences in the numbers of the nucleated RBC, urinary uric acid/creatinine ratio, concentration of creatine kinase between the transplanted infants and controls. But the nucleated RBC is more likely to be rapidly discharged in the transplanted group. In the transplanted group, the concentrations of IL6, $IL-1{\beta}$, and GDNF were no significant difference between day 1 and 7, although GDNF seemed to be elevated. Serum NSE concentration was significantly elevated after transplantation, but not in CSF. Conclusion : It is suggested that autologous umbilical cord blood transplantation in preterm very low birth weight infant is safe to apply clinical practice. Long term follow up study should be needed to evaluate the potential therapeutic effect of umbilical cord blood transplantation for neuroprotection.

• Journal of radiological science and technology
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• v.26 no.2
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• pp.57-61
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• 2003

The purpose of this study is to compare both 1.5T and 4.7T in Praietal White matter material Phantom using the same methodology at both field strengths. Data at both field strengths are compared in terms of $T_2$ relaxation times, line widths and SNRs MR imaging and $^1H$ MR spectroscopy were performed on GE 1.5T SIGNA system and Broker Biospec 4.7T/30 MRI/MRS system. After phantom axial scan $^1H$ MRS was obtained from T2 weighted image by 3-dimensional localization technique(PRESS : Point RE solved spectroscopy Sequence) this phantom is composed of an aqueous solution 36.7 mmol/L of NAA, 25.0 mmol/L of Cr, 6.3 mmol/L of choline chloride, 30.0 mmol/L or Glu, and 22.5 mmol/L of MI(adjusted to a pH of 7,15 in a phosphate buffet). Data processed using software developed inhouse. At 1.5T, T2 relaxation times for Cho, Cr, and NAA were $0.41{\pm}0.07,\;0.26{\pm}0.04,\;0.46{\pm}0.07$ while at 4.7T they were $0.17{\pm}0.03,\;0.14{\pm}0.05,\;0.20{\pm}0.03$ respectively. At 1.5T, line widths for water, Cho, Cr and NAA were $2.9{\pm}0.7,\;1.6{\pm}0.7,\;1.7{\pm}0.8,\;2.2{\pm}0.02Hz$ while at 4.7T they were $5.2{\pm}1.1,\;4.6{\pm}1.9,\;4.01{\pm}1.8,\;4.8{\pm}1.9Hz$ respectively. It can be seen that $T_2$ relaxation times were significantly shorter at 4.7 compared to 1.5T and that the line widths were also broader. The average SNRs for NAA for subjects at short and long TEs were $23.5{\pm}11.3$ at TE=20 msec ; $15.4{\pm}7.7$ at TE=272 msec at 1.5T and $40{\pm}8.3$ and $17{\pm}3.5$ respectively at 4.7T higher field strength is superior because of improved sensitivity and chemical shift dispersion. However these improvements are partially offset by increased line widths and decrease $T_2$ relaxation times, which act to reduce both sensitivity and resolution. In our experiments with the equipment available to us, 4.7T proton spectra at short TEs exhibit moderately improved sensitivity compared to 1.5T.