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A Case of Cystinuria with Multiple Renal Stones in an 8-month-old Girl (8개월 여아에서 다발성 결석으로 진단된 시스틴뇨증 1례)

  • Kang, Eun Gu;Lee, Joo Hoon;Lee, Beom Hee;Kim, Gu-Hwan;Park, Young Seo
    • Childhood Kidney Diseases
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    • v.17 no.2
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    • pp.122-126
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    • 2013
  • Cystinuria is an autosomal recessive disease characterized by impaired transport of cystine and dibasic amino acids in the proximal renal tubule, resulting in the formation of cystine stones. It is believed to account for about 1% of all kidney stones and up to 10% of pediatric stones. Here we report a case of cystinuria with multiple renal stones confirmed by genetic mutational analysis. An 8-month-old girl was admitted to AMC with persistent fever and multiple renal stones. A renal sonogram showed multiple stones at the right renal pelvis, right distal ureter, and left renal medullary portion. An approximately 1 cm renal stone was extracted spontaneously, and stone analysis revealed it to be composed entirely of cystine. Cystinuria was confirmed by increased urine dibasic amino acid levels, including cysteine, and genetic mutational analysis showed the patient to be a homozygote for the pathogenic c. 1820del (p.L607fs) of SLC3A1. Despite treatment with oral hydration and urinary alkalinization, and restricted intake of animal protein, the stones increased in size and number. The patient has since been treated with tiopronin.

Environmental Effects on the Benthic Polychaete Communities Around the Power Plant Areas in the East Sea of Korea (동해 발전소 주변의 저서다모류 군집에 영향을 미치는 환경 요인)

  • KWON, SOON HYUN;LEE, JAE HAC;YU, OK HWAN
    • The Sea:JOURNAL OF THE KOREAN SOCIETY OF OCEANOGRAPHY
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    • v.22 no.1
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    • pp.18-30
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    • 2017
  • The East Sea is almost entirely composed of sandy facies, and the facies type is the major factor influencing benthic polychaete communities. There have been few studies of the effects of environmental factors on benthic polychaetes in thermal discharge areas consisting of different sediment types. This study identified the spatial distribution patterns based on the species composition and distribution of benthic polychaete communities, and the environmental factors influencing benthic polychaetes near power plants were investigated. The polychaete communities in the Uljin, Hupo, and Gori coastal areas near the power plants in the East Sea were seasonally investigated from August 2006 to February 2013. As a result, 283 species were collected. The dominant species were Spiophanes bombyx, Magelona japonica, Lumbrineris longifolia, and Sternaspis scutata. Spiophanes bombyx was the dominant species at Uljin and Hupo, but M. japonica was the dominant species at Gori. Two dominant species from the coastal waters of the East Sea, Lumbrineris longifolia and M. japonica, were rare in the power plant water intake or drainage areas. Cluster analysis was performed to divide the study area into groups by the total organic carbon content, sediment grain size and facies patterns. This study suggests that the community structures of polychaetes are more affected by the total organic carbon, grain size of sediment and facies than by disturbance from thermal discharge, which only affected the polychaete community near power plant drainage areas.

Effects of Dietary Supplementation of Fermented Microbial Complex (Eco-Farm$^{(R)/}$) on Performance of Finishing Pigs and Air Quality in Finishing Building (복합미생물 발효제(Eco-Farm$^{(R)}$ ) 급여가 비육돈 생산성과 돈사 환경개선에 미치는 영향)

  • 김두환;정치섭
    • Journal of Animal Environmental Science
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    • v.9 no.1
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    • pp.27-34
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    • 2003
  • This experiment was carried out to investigate the effect of dietary supplementation of fermented microbial complex(Eco-Farm$^{(R)}$) on performance of finishing pigs and indoor air quality in finishing building. A total 135 crossbred [(Landrace ${\times}$ Yorkshire) ${\times}$ Duroc] pigs were randomly arranged into nine groups and assigned to three treatments. Pigs were fed a basal diet supplemented with 0, 0.5 and 1% level of fermented microbial complex(Eco-Farm$^{(R)}$) until the market weight for 40 days of the experimental period. Average daily feed intake and feed conversion ratio were significantly improved (p<0.05) with dietary supplementation of 0.5% fermented microbial complex (Eco-Farm$^{(R)}$): however, average daily gain was not affected by dietary supplementation of fermented microbial complex(Eco-Farm$^{(R)}$). Indoor ammonia and hydrogen sulfide concentrations in the finishing building were significantly(p<0.05) decreased by dietary supplementation of fermented microbial complex(Eco-Farm$^{(R)}$) compared with those of control, however, indoor carbon dioxide concentration was not affected by dietary supplementation of fermented microbial complex(Eco-Farm$^{(R)}$). In conclusion, the results obtained from this experiment suggest that the dietary supplementation of fermented microbial complex(Eco-Farm$^{(R)}$) for finishing pigs improved performance and indoor air quality in the finishing building.hing building.

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The Environment-Friendly High Quality Pork Production by feeding the Fermentation Products of Natural Clay Mineral (천연의 점토광물질 발효산물 급여에 의한 친환경 고품질 원료돈육 생산)

  • Cho, Jin Kook;Yang, Seung Hak;Hwang, Seong Gu
    • Journal of Animal Environmental Science
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    • v.18 no.sup
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    • pp.55-64
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    • 2012
  • We investigated the effect of the clay mineral fortified with complex trace elements by specific fermentation microbes on growth performance and a quality of pork in this study. For the declared experimental animal, 80 heads of crossbred with Large White and Yorkshire were randomly splitted into a control group and a test group. Each group were assigned with 4 replicates and 10 heads of each replicate. When 0.3% of the fermentation product of the clay mineral (FCM) was added into feed stuff and fed for 9 weeks of experimental feeding period, the results of the feed intake, weight gains, pH of carcass, lightness of meat color, and brightness of the muscle semimembranosus were similar to those of the control group. However, dressing percentage, reddishness of carcass, and water holding capacity were improved, respectively, as much as 2.7%, 12%, and 10%, and reduced by 6% in cooking loss. In order to investigate the immune modulatory effect of fermented clay mineral, pheripheral blood mononuclear cells (PBMC) were isolated and Immune modulatory parameters were measured. The proliferation activity of PBMC from pigs fed the fermented clay mineral were significantly increased compared to control group pigs, and also those results were more clearly observed as activated with lipopolysaccharide and concanavaline A. The secretion of TNF-${\alpha}$ of the FCM group pigs showed an increasing tendency. Therefore it was suggested that the feeding of FCM which was high in cation metathesis and the value of infrared ray, activated the immune responses, and thus the production of the environment-friendly high quality pork without the use of antibiotics would be possible.

Analysis of Heat Environment in Nursery Pig Behavior (자돈의 행동에 미치는 열환경 분석)

  • Sang, J.I.;Choi, H.L.;Jeon, J.H.;Jeon, B.S.;Kang, H.S.;Lee, E.S.;Park, K.H.
    • Journal of Animal Environmental Science
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    • v.15 no.2
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    • pp.131-138
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    • 2009
  • This study was conducted to find ways to control environment with the difference between body temperature and background temperature based on swine activity, and to apply to the environment control system of swine barns based on the findings. Following are the results. 1. Swine activity related to background temperature was achieved as color images and swine activity status was categorized into cold, comfortable, and hot periods with visualization system (thermal image system). 2. Thermal image system consisted of an infrared CCD camera, an image processing board - DIF (TH3100), an main computer (400Hz, 128M, 586 Pentium model) with C++ program installed. 3. Thermal image system categorizing temperatures into cold, comfortable, and hot was applicable to the environment control system of swine barns 4. Feed intake was higher in cold temperature, and finishing weight and weight gain per day in cold temperature were lower than others (p<0.05).

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Investigation of Porcine Melanocortin-4 Receptor (MC4R) Polymorphism on Economic Traits (돼지 melanocortin-4 receptor (MC4R) 유전자의 경제형질과의 연관성에 관한 연구)

  • Kim Kwan-Suk;Shin Hee Young;Lee Joong-Jae;Hong Sung-Kwang;Choi Bong-Hwan;Kim Tae-Hun;Lee Hak-Kyo;Cho Byung-Wook
    • Journal of Life Science
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    • v.15 no.6 s.73
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    • pp.968-971
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    • 2005
  • The Melanocortin-4 receptor gene (MC4R) regulates the energy balance and the genetic basis of obesity. A polymorphism in the porcine melanocortin-4 receptor has previously shown to be associated with growth, fat deposition and feed intake. In this study, the polymorphism of the gene was studied in several pig breeds of Duroc, Landrace, Berkshire, and Yorkshire. The results showed that the frequencies of MC4R genotype varied among those breeds. Association analyses were also performed between the MC4R polymorphism and average daily gain, feed conversion ratio, backfat thickness and lean percentage phenotypes. The results strongly support that the MC4R polymorphism can be used DNA marker selection indicator for economically important traits for pig breeding program in Korea.

Nutritional quality of leaf proteins prepared from crops containing phenolic compounds and polyphenolase (Phenolic compound와 polyphenolase 함유 작물로부터 조제한 녹엽단백질의 영양가)

  • 조영수;차재영
    • Journal of Life Science
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    • v.7 no.3
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    • pp.192-197
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    • 1997
  • Italian ryegrass, red clover, sorghum, and alfalfa were used for leaf protein preparation. Fresh leaves were pulped in the presence or absence of a reducing agent(sodium ascorbate or NaHSO$_{4}$) and green juice was heated and washed with acetone. The biological evaluation of leaf proteins was carried out by the growth method with male rats weighing about 45g. Italian ryegrass, red clover, and sorghum were brown when leaves were pulped in the absence of a reducing agent. On the other hand, alfalfa had neither o-diphenolics nor polyphenolase, and hence the alfalfa leaf protein did not brown during pulping ever in the absence of a reducing agent. The brown leaf protein from Italian ryegrass hd lower digestibility than the leaf protein protected from browning, although there were no difference in growth-promoting effect and protein efficiency ratio(PER)between the two leaf protein. The feeding of brown leaf protein from red clover resulted in the lowering of weight gain, digestibility, and PER, and all the measurement including diet intake were lowered by feeding the brown leaf protein from sorghum. In the case of alfalfa leaf protein, there were no difference in nutritional quality between the two leaf protein made with and without an attempt to prevent browning. The results mentioned above indicate that the occurrence of phenolics and polyphemolase in a crop in responsible for the browning of leaf protein and that the browning of leaf protein caused its nutritional impairment.

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Effect of Feeding a By-product Feeds-based Silage (Biosilage®) on Behavior Pattern of Growing Hanwoo Steers (부산물사료 주원료 사일리지(Biosilage®) 급여가 거세 육성 한우의 행동양식에 미치는 영향)

  • Kim, Young Il;Lee, Sang Moo;Park, Keun Kyu;Kwak, Wan Sup
    • Journal of The Korean Society of Grassland and Forage Science
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    • v.33 no.4
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    • pp.290-297
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    • 2013
  • This study was carried out to investigate the effects of a by-product feed-based silage (BF silage) feeding on behavior patterns of growing Hanwoo steers. A total of 10 Hanwoo steers (11 months old, 302kg of body weight) were assigned to 2 dietary treatments: the control (concentrate mix + free access to rice straw), and the treatment (concentrate mix + free access to BF silage). The behavior patterns were observed for 48 hours. The intakes of dry matter (DM) and neutral detergent fiber (NDF) of the treatment group were higher than those of the control group. Eating time, ruminating time and resting time were not different between the control and treatment. But, the intake time per kg DM was higher for the control than treatment. The number of bolus, total chewing frequency, number of ruminating per bolus and number of bolus per minute were not different between the control and treatment. But the chewing frequency per bolus was higher in the treatment than control (p<0.05), and feed value index was lower in treatment than control (p<0.05). Frequencies of drinking and defecating were not different between the two groups, but the frequency of urinating was higher for the treatment (p<0.05) than control. Eating rate, ruminating efficiency and chewing efficiency were much higher in the treatment group than control (p<0.05). These results indicate that the replacement of conventional rice straws with the BF silage (physically effective NDF, about 25%) did not affect the ruminating behaviors of Hanwoo steers significantly.

Effect of Insoluble Dietary Fiber Extracted from Salicornia herbacea L. on Large Intestinal Function in Rats (불용성 함초 식이섬유의 섭취가 흰쥐 장기능에 미치는 효과)

  • Kim, Soo-Hwan;Kim, Sook-Ja;Lee, Hyeong-Seon
    • Korean Journal of Food Science and Technology
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    • v.46 no.5
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    • pp.648-654
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    • 2014
  • This study investigated the effect of insoluble dietary fibers extracted from Salicornia herbacea L. (S. herbacea) on the improvement of intestinal function in rats. Sprague-Dawley rats were fed on diet containing 5% and 10% S. herbacea dietary fiber (SHDF) for four weeks. Rats receiving the SHDF diet showed a significant decrease in their triglyceride levels and an increase in HDL-cholesterol levels. In addition, compared with the control group, the SHDF group showed a significant increase in the total quantity of the feces and its moisture content. The intestinal transit time of the feces was also shorter in this group. The pH of the feces decreased in all the other experimental groups. Particularly, the bile acid content of the feces and the thicknesses of the mucus layers showed significant recovery on SHDF intake. These results suggest that dietary fiber isolated from S. herbacea has a marked effect on the improvement of bowel function in rats with loperamide (2 mg/kg)-induced constipation.

Hazardous Heavy Metal Contents of Mushrooms from Retail Markets in Seoul (서울 지역에서 유통되는 버섯류의 유해중금속 함량 조사에 관한 연구)

  • Cha, Hyun-A;Kang, Soon-Hwa;Choe, Su-Bin;Kim, Hyung-Kook;Kang, Sung-Tae
    • Korean Journal of Food Science and Technology
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    • v.46 no.3
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    • pp.283-287
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    • 2014
  • This study determined the hazardous heavy metal content of mushrooms from markets in Seoul, Korea. One hundred and fifty mushroom samples were digested by the microwave method then the hazardous heavy metal (Pb, Cd, As, Cr, Ni and Hg) content was determined by the inductively coupled plasma-mass spectrometry (ICP-MS) and mercury analyzer. The average values of heavy metals in the samples were as follows: (mean [minimum-maximum], ${\mu}g/kg$); Pb 7.8 (1.9-30.0), Cd 45.3 (0.7-292.4), As 54.6 (24.2-229.3), Cr 42.6 (8.4-334.3), Ni 22.4 (up to 180.4), and Hg 8.9 (2.0-25.0). The contents of hazardous heavy metal in mushrooms were lower than those reported previously and also lower than the Provisional Tolerable Weekly Intake (PTWI) limit prescribed by the FAO/WHO. Thus, it could be suggested that the hazardous heavy metals levels in the mushrooms of retail market were adequately safe for consumption.