• Title/Summary/Keyword: 프로피온산 분해

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Inherited metabolic diseases in the urine organic acid analysis of complex febrile seizure patients (복합 열성경련 환자의 소변 유기산 분석에서 나타난 유전대사질환)

  • Cheong, Hee Jeong;Kim, Hye Rim;Lee, Seong Soo;Bae, Eun Joo;Park, Won Il;Lee, Hong Jin;Choi, Hui Chul
    • Clinical and Experimental Pediatrics
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    • v.52 no.2
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    • pp.199-204
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    • 2009
  • Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278 patients referred for complex febrile seizure. Results : Of 278 patients, 132 had no abnormal laboratory findings, and 146 patients had at least one of the following abnormal laboratory findings: acidosis (n=58), hyperammonemia (n=55), hypoglycemia (n=21), ketosis (n=12). Twenty-six (19.7 %) of the 132 patients with no abnormal findings and 104 (71.2%) of the 146 patients with statistically significant abnormalities showed abnormalities on the organic acid analysis (P<0.05). Mitochondrial respiratory chain disorders (n=23) were the most common diseases found in the normal routine laboratory group, followed by PDH deficiency (n=2) and ketolytic defect (n=1). In the abnormal routine laboratory group, mitochondrial respiratory chain disorder (n=29) was the most common disease, followed by ketolytic defects (n=27), PDH deficiency (n=9), glutaric aciduria type II (n=9), 3-methylglutaconic aciduria type III (n=6), biotinidase deficiency (n=5), propionic acidemia (n=4), methylmalonic acidemia (n=2), 3-hydroxyisobutyric aciduria (n=2), orotic aciduria (n=2), fatty acid oxidation disorders (n=2), 2-methylbranched chain acyl CoA dehydrogenase deficiency (n=2), 3-methylglutaconic aciduria type I (n=1), maple syrup urine disease (n=1), isovaleric acidemia (n=1), HMG-CoA lyase deficiency (n=1), L-2-hydroxyglutaric aciduria (n=1), and pyruvate carboxylase deficiency (n=1). Conclusion : These findings suggest that urine organic acid analysis should be performed in all patients with complex febrile seizure and other risk factors for early detection of inherited metabolic diseases.

Microbiological and Chemical Changes of Complete Feed during Spoilage (배합사료의 부패 동안 발생하는 미생물학적 및 영양학적 변화)

  • Yi, Kwonjung;Yeon, Jae-Sung;Kim, Juhyeon;Kim, Sam Churl;Moon, Hyung-In;Jeon, Che Ok;Lee, Sang Suk;Kim, Dong-Woon;Kim, Soo-Ki
    • Journal of Life Science
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    • v.25 no.10
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    • pp.1148-1155
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    • 2015
  • Commercial complete feeds contain enough nutrients to support animal growth and it is easy to be spoiled under proper temperature and humid conditions. The aim of this study was to investigate microbiological and chemical changes on complete feed for milking cow under open-air exposure with moisture 33% at 30℃ during 15 days. pH decreased 6.29 to 4.66 and water activity decreased gradually 0.99 to 0.95. Bacteria increased 6.2×106~1.6×107 to 2.1×109 CFU/g at 5 days and showed 108 CFU/g until 15 days. Fungi increased 103 CFU/g to 8.0×104 CFU/g. During the processing of spoilage, bacteria such as Acinetobacter oleivorans, Pediococcus acidilactici, Acinetobacter oleivorans, Weissella cibaria, and Methylobacterium komagatae were identified and fungi such as Fusarium sp. and Mucor sp. were also identified. Moisture content increased until 10 days (p<0.01). Crude protein was not changed so much whereas crude fat decreased 6.0% to 5.5% (p<0.01). Crude fiber and crude ash changed 2.0~ 3.0% and 4.5~ 4.8% levels with no significance, respectively. Gross energy was not almost changed at 4,400 kcal/g. During spoilage, lactate and propionate increased whereas acetate was not detected. Protease and lipase activities increased significantly during spoilage (p<0.01). Zearalenone content increased 59.2 μg/kg to 623.8 μg/kg, showing 10.5 times more production. During feed spoilage, pH decreased with microbial growth and various chemical changes were occurred.

Studies on the Feed Value of Yukmijihwangtang Meal (육미지황탕박의 사료가치에 관한 연구)

  • Cha, Sang-Woo;Choi, Hyun-Soo;Kim, E-Soo;Jung, Kang-Seok;Jang, Won-Seop;Shin, Yong-Joon;Lee, Soo-Kee
    • Korean Journal of Agricultural Science
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    • v.36 no.1
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    • pp.63-71
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    • 2009
  • Two experiments were conducted to investigate feed value of Yukmijihwangtang (herbal medicine) meal. In experiment 1, the chemical composition of Yukmijihwangtang ingredients was determined before and after extraction, and investigated the effects of partial substitution of dried Yukmijihwangtang meal on TMR for fattening cattle. In experiment 2, nine Korean native male goats were employed to investigate the palatability and ruminal parameters of Yukmijihwangtang meal silages. In experiment 1, the crude protein content after water extraction was higher in Alisma orientale Juzepczuk (Teaksa), and lower in Pachyma hoelen Rumph (Bokryeong). the crude fat content after water extraction was higher in Cornus officinalis Sieb. et Zucc (Sansuyu), and lower in other ingredients. ADF content after water extraction of Cornus officinalis Sieb. et Zucc (Sansuyu) was higher compared to those of Pachyma hoelen Rumph (Bokryeong) and Paeonia suffruticosa Andrews (Mokdanpi). Dioscorea japonica Thunberg (Sanyak) was the lowest in ADF content. Dry matter disappearance was increased by adding Yukmijihwangtang meal to TMR. In experiment 2, feed intakes of Yukmijihwangtang silage containing molasses (0.5 and 1%) for 30 min and 6 hr were significantly higher than that of control (0%) in Korean native goats. In various ruminl parameters, molasses addition to silage materials trended to increase total VFA, propionate and valerate contents, but trended to decrease iso-butylate, butylate and iso-valerate contents in Korean native goats. In conclusion, Yukmijihwangtang meal could be used as partial substitution of low level in TMR for fattening cattle. Molasses supplementation to silage materials contained with herbal medicine meal could improve its nutritional quality and palatability for Korean native goats. One percent level of molasses addition could give a useful effect in ensiling.

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Effects of Molasses Addition on the Feeding Value of Socheongryongtang(Herbal Medicine) Meal Silage (당밀 첨가가 소청룡탕박 사일리지의 품질에 미치는 영향)

  • Moon, Kye Bong;Cho, Sung Kyung;Park, Duk Sub;Kim, Sung Bok;Lee, Bong Duk;Jo, Cheorun;Lim, Jae Sam;Lee, Soo Kee
    • Journal of Animal Science and Technology
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    • v.50 no.3
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    • pp.381-390
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    • 2008
  • Two experiments were conducted to investigate effects of molasses addition to silage materials in ensiling Socheongryongtang meal on the nutritive quality of silage, palatability and various ruminal parameters in Korean native goats. In Experiment 1, Socheongryongtang meal silage was produced by the addition of 0, 0.5, or 1.0% molasses and stored for 40 days at room temperature. There were three replicates per treatment. Lactic acid contents of Socheongryongtang meal silage containing molasses(0.5 and 1.0%) were significantly(p<0.05) higher than that of the control(0%). However, the pH and butyric acid contents of Socheongryongtang meal silage containing molasses(0.5 and 1.0%) were lower than those of the control(0%). In addition, molasses(0.5 and 1.0%) increased the number of lactobacillus, but decreased the number of fungi in Socheongryongtang meal silage. In vitro dry matter disappearance tended to increase by molasses addition. In Experiment 2, three 1.5-yr-old Korean native female goats were employed in cross-over design to measure the palatability and various ruminal parameters of Socheongryongtang meal silage. Feed intake of Socheongryongtang meal silage containing molasses(0.5 and 1.0%) for 30 min significantly higher than that of control(0%). Silmilar trend was found when the feed intake was measured for 6 hr. In various ruminal parameters, molasses addition to silage materials increased propionic acid(P) and total volatile fatty acid contents, but decreased pH, acetic acid (A), and butyric acid. The A/P ratio was the lowest in 1.0% molasses treatment group. It is concluded that molasses addition in ensiling Socheongryongtang meal could enhance its nutritional quality and lengthen storage period. Also the palatability of silage was improved by the addition of molasses.

The Effect of the Addition of Carbohydrate Sources on the Concentration of Odorous Compounds for Recycling of Pig Slurry to Grassland (발효탄수화물 첨가 비육돈사료가 초지환원용 슬러리의 악취물질 농도에 미치는 영향)

  • Cho, Sung Back;Hwang, Ok Hwa;Park, Kyu Hyeun;Choi, Dong Yun;Yang, Seung Bong;Kim, Do Hwan;Park, Sung Kwon
    • Journal of The Korean Society of Grassland and Forage Science
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    • v.33 no.4
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    • pp.257-262
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    • 2013
  • This study investigates the effect of fermentable carbohydrate on the concentration of odorous compounds in pig slurry. Four types of pig diet were studied: control, peanut hull (crude fiber 29.87, NDF 44.02%), golden fiber (crude fiber 48.77, NDF 65.88%), and almond hull (crude fiber 44.30, NDF 64.44%). Pigs (average BW 37.0 kg) were fed diets that met the Korean Feeding Standard (2012) and their excreta samples were collected from the slurry pits. Levels of volatile organic compounds (phenols and indoles) and volatile fatty acids were analyzed by gas chromatography. Phenol level was the lowest (p<0.05) in golden fiber (33.26 ppm) group and the highest in control (97.29 ppm). The concentration of indoles in the peanut hull (1.27 ppm), almond hull (1.20 ppm), and golden fiber (1.02 ppm) groups was lower (p < 0.05) than that of control (1.79 ppm). Levels of short chain fatty acid (SCFA) were lower (p < 0.05) in golden fiber (1,319 ppm) and almond hull (1,433 ppm) groups than in control (1,893 ppm). Concentration of branched chain fatty acid (BCFA) in the golden fiber group (74 ppm) was lower (p < 0.05) than that of control (98 ppm). Taken together, the concentration levels of phenols, indoles, and VFAs decreased on addition of peanut hull, golden fiber or almond hull to the diet, suggesting that fermentable carbohydrate may contribute to reducing odorous compounds in pig slurry.