• Title/Summary/Keyword: 보충치

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Serum 25-Hydroxy Vitamin $D_3$ Analysis of Korean People (한국인 일반인의 혈청 25-Hydroxy Vitamin $D_3$의 분석)

  • Kim, Bo-Kyung;Jung, Hyun-Mi;Kim, Yun-Kyung;Kim, So-Young;Kim, Jee-Hyun
    • The Korean Journal of Nuclear Medicine Technology
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    • v.14 no.1
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    • pp.133-137
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    • 2010
  • Purpose: The main function of vitamin D is the mineralization of the brain by increase of calcium and phosphorus, in case it is insufficient in children, lime deposition on cartilage cannot occur so it leads to rachitis, and in adults, it leads to osteomalacia or osteoporosis. It is also strongly believed in the academic world that vitamin D can restrict the growth of cancer cells and prevent heart diseases, which is also somewhat proven in epidemiological researches. While the right density of vitamin D is still being studied, 20-32 ng/mL is believed to be the most ideal density. Therefore, I wanted analyze how much density of 25-Hydroxyvitamin D3 that Koreans possess. Materials and Methods: From February 20th, 2008 to April 21st, 2009, the collection of 2800 serums, from medical examination treated subjects by Neodin Medical Institute, have been tested. The targets were tested by 25-Hydroxyvitamin D (125I Kit: Diasorin, USA), and were analyzed by dividing into many different categories (gender, age, season, region). Results: The average density of male were 20 ng/mL, female 17.08 ng/mL. Per age groups, the density of males were as follows: 10~20-18 ng/mL, 21~30-17 ng/mL, 31~40-19 ng/mL, 41~50-21 ng/mL, 51~60-22 ng/mL, 61~70-22 ng/mL, 71~80-22 ng/mL and 81~90-19.9 ng/mL. Average density of females per age groups, were as follows: 10~20-16 ng/mL, 20~30-15.26 ng/mL, 30~40-16 ng/mL, 40~50-17 ng/mL, 50~60-19 ng/mL, 60~70-19 ng/mL, 70~80-19 ng/mL, and 80~90-17 ng/mL. Per seasons, From December to May, the subjects showed the density of 15.97 ng/mL, while from June to November, it showed 21.60 ng/mL. On density of males from January to April regionally, Seoul+Gyeonggi-Do-15.52 ng/mL, Gangwon-Do-15.33 ng/mL, Choongchung-Do-18.03 ng/mL, Jeonla-Do-18.68 ng/mL, Gyungsang-Do-18.76 ng/mL and Cheju Do-21.23 ng/mL. Conclusions: The vitamin D of Koreans is has been insufficient compared to the suggested amount. Ultraviolet rays, which is the main source of vitamin D is critical, therefore it is suggested that more outdoor activities can definitely help.

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Study on the Long-term Forecasting of Brown Planthopper Outbreaks (벼멸구 발생의 장기예찰을 위한 기초적 연구)

  • Paik Woon Hah;Paik Hyun Joon
    • Korean journal of applied entomology
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    • v.16 no.3 s.32
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    • pp.171-179
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    • 1977
  • Since the outbreak of the brown planthopper (Nilaparvata lugens) in 1915 caused tremendous losses in rice production, one of the more effective method of prevention of such a disaster could be the establishment of longterm forecasting system, In 1916 the author indicated there was a correlation between sunspot activities and brown planthopper and the white back planthopper outbreaks. However, the examples seem to be too small size to state a definite correlation. The purpose of the present study IS to revi~w the history of the brown planthopper outbreaks, and to establish a more effective forcasting system. The present forcasting methods are based on light trap catches of adults which already migrate into this country from mainland China. The regular cycle of 11.2 years of sunspot activity began in 1710, and was continued to present. To gather more records of brown planthopper, the author checked 'Joseon Wangjo Silrok' and analized the so-called 'Hwang' 'Hwang-chung' and 'Chung' which have multiple meanings, together with 'Samguk Sagi' 'Goryo Sa' and 'Munheon Bigo.' The results obtained by the about from review of these old literature citations revealed that ten species of insect and unknown species were involved: i. e., pine moth (Dendrolimus spectabilis), army worm (Mythimna separata), brown planthopper (Nilarvata lugens), white-back planthopper (Sogatella furcifera), migratory locust (Locutsa migratoria), rice stem borer (Chilo suppressalis,), mole cricket (Gryllotalpa africana), rice-plant weevil (Echinocnemus squameus), cut worm (Euxoa segetum), and mulberry pyralid Margaronia pyloalis) The suspected incidence of planthopper in old records expressed by 'Hwang' or 'Chung' revealed a total or 25 out of 37 in 'Samguk sagi,' 21 out of 49 in 'Goryo sa,' 9 of 73 in 'Wanjo-silrog,' and none of 8 in 'Munheon bigo' were planthoppers. Therefore, a total of 36 out of 167 records of insect incidence in the old literature can be possibly attributed to planthoppers. The brown planthopper and white-back planthopper migrate together to Korea every year from mainland China, However, the number of each species are differ by year. In 1975 outbreak the brown planthopper was dominant; and the white-back planthopper prevailed in 1946 and 1977 outbreaks, During the course of this study, the author was able to add a new record of outbreak of planthop per. In 1916 the white-back planthopper outbreak caused serious losses in Chungcheong-namdo and Jeonla-namdo, with losses estimated as high as 160 and 190 thousand seok (23.2 and 27.5 thousand M/T), in Naju and Secheon county, respectively. Since 1912, major outbreaks of brown planthopper or white-back planthopper have been recored 5 times. These occurrences coincide and well matched the period of minimum number of sunspots, With these authenticated records of planthoppers, the author believes there is a close correlation between brown planthopper and white-back planthopper outbreaks in Korea and sunspot activities. Therefore, in years of low number of sunspots, we should watch for and expect outbreaks of these. insects. At this time, it will be necessary to provide all possible prevention measures.

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Development and Application of the Slope Management Program in Urban Area (대도시 사면관리프로그램 개발 및 적용)

  • Kim, Kyeong-Su;Chae, Byung-Gon;Cho, Yong-Chan;Lee, Choon-Oh;Song, Young-Suk
    • The Journal of Engineering Geology
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    • v.17 no.1 s.50
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    • pp.15-25
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    • 2007
  • In general, the life and asset casualties that occur due to landslide or slope failure in urban areas are larger than that in rural areas. In order to reduce the casualties, a slope management program is necessary to categorize slopes based on properties and to manage them systematically. The slope management system is the establishment of the data base for the geological and geotechnical factor according to slope stability, and the utilization of the data base to manage slopes. The suitable system must develop to slopes in urban area through the survey, analysis and evaluation process. Based on the above necessity, the slope management program which is applicable to slope management in an urban area has been developed at Hwangryung Mt. in Busan as a target area. The developed slope management program has various functions such as slope ID number of each slope or sub-region of a mountain, making a slope data sheet, analysis and grouping of slope stability, and establishment of a data base. The slope management program is constructed by use of GIS, and the survey, test and analysis data according to all slopes can be input and edited into the program. The program can also be utilized practically by end users due to the convenient input, edition printing, management and operation of slope data. Therefore, the slope management system has been established on the application of the developed program in Busan which is located in slope area. As the system is widely applied to other cities, the slope in urban area can be managed systematically and the slope hazards can be minimized.

Usefulness of auditory brainstem response as early predictor of kernicterus in early breast-feeding jaundice (조기 모유 황달에서 핵황달의 조기 예측도구로서의 청성 뇌간유발 반응 검사의 유용성)

  • Jang, Jae Won;Lee, Gil Sang;Song, Dae Keun;Kim, Sung Hee;Kim, Won Duck;Lee, Sang Geel
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.848-854
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    • 2007
  • Purpose : The present study examined the etiology and risk factors of the early breast-feeding jaundice and the usefulness of auditory brainstem response test as early predictor of kernicterus. Methods : Medical records of neonatal jaundice in newborn admitted to Daegu Fatima Hospital between September 2005 and May 2006 were analyzed prospectively. Infants were grouped according to feeding method : breast feeding group (breast feeding only, n=23), mixed feeding group (breast feeding mainly plus addition of fomula feeding, n=13). Results : There were no significant differences in gestational age, birth weight, sex, duration of phototherapy, serum bilirubin and hemolytic evidence between the two study groups. First visiting day of life at out patient department was significantly delayed in breast feeding group ($8.7{\pm}3.6day$) compared to mixed feeding group ($6.0{\pm}1.9$) (P=0.009). Weight loss was significantly severe in breast feeding group compared to mixed feeding group (P<0.05). In auditory brainstem response test, loss of Wave V in 3 cases was observed and recoverd after blood exchange transfusion in follow up test.Wave III latency had significant correlation to serum bilirubin in auditory brainstem response test (70 dB) (P=0.002). Conclusion : Our study suggest that further education about breast feeding and follow up within the first postnatal week would be necessary for early detection and prevention of early breast-feeding jaundice. Test of serum bilirubin and auditory brainstem response would be helpful in determination of blood exchange transfusion.

Development of Protein-rich Food Mixtures for Infants and Growing Children in Korea -(4) Nutritional and Biochemical Evaluation of Formulas F-P-5, F-P-6, F-P-7 and storage stability of F-P-4- (유유아(乳幼兒) 및 성장기아동을 위한 영양식품 개발에 관한 연구 -(4) F-P-5, F-P-6 및 F-P-7의 영양학적, 생화학적 검토 및 그 저장성-)

  • Kwon, Tai-Wan;Cheigh, Hong-Sik;Kim, Sook-He;Lee, Hyun-Keum
    • Journal of Nutrition and Health
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    • v.3 no.3
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    • pp.129-135
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    • 1970
  • From the previous studies, F-P-4 formula was found to be comparable to full fat dry milk in its nutritive value and feeding performance. However, an attempt was made in order to make sure whether or not any possibility might exist, by which further improvement of nutritive quality and simultaneous reduction of product costs may be achieved. Using F-P-4 as a control, modifications were made in new formulas, F-P-5, F-P-6 and F-P-7 by reducing FPC, eliminating yeast from the mixture, and by enriching with methionine as needed. In particular, F-P-7 is completely free of FPC, hydrogenated oil and yeast. Yet, levels of total protein and fat were kept equal to those of F-P-4 in all formulas. An animal feeding test for all formulas using 10 female rats per group for 8 weeks and an infant feeding trial for F-P-5 and F-P-6 with 5 of each female infants under age of one for one month were conducted along with F-P-4 as a control. Almost the same results were obtained with F-P-4, 5 and 6, but F-P-7 showed the lowest body weight gain. FER of F-P-5 and 6 was 0.20 as was with F-P-4, while that of F-P-7 was 0.16. Acceptability to infants was excellent; growth, appearance and biochemical data were normal. As an example F-P-4 packed in 0.04mm polyethylene bags was used for storage study at $25^{\circ}C$ and relative humidity of $65{\sim}85%$ for 8 months. Although viable bacterial counts and vitamin C contents were reduced, peroxide and TBA values were increased gradually during such storage. Since there are also significant changes in color and organoleptic quality, the expected shelf life under the given conditions is considered to be about 2 months and thus further works are needed both on the product and packaging in order to improve the storage stability. Either elimination of yeast form F-P-4, that is F-P-5, or partial replacement of FPC with methionine, that is F-P-6 may well reduce material costs about 10%. Considering blending process of ingredients, F-P-5 is thus found to be the best formula developed. While F-P-7 free of FPC is inferior in its nutritive quality than that of others, but significantly superior than of rice. Furthermore, the material cost of the product can be reduced about 20% from that of F-P-4. And thus this vegetable blend is considered to be useful as a low cost supplementary food mixture for growing children.

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우리나라 어린이 건강 및 영양상태의 현황과 문제점

  • Yun, Deok-Jin
    • Journal of Nutrition and Health
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    • v.12 no.2
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    • pp.1-13
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    • 1979
  • 성장발육하는 시기에 영양과 성장하는 아동들의 체위와는 밀접한 관계가 있고 또한 영양부족과 그로 말미암아 자라나는 이학적 소견과도 불가분의 관계가 있기 때문에 그동안 우리나라에서 부분적, 단편적으로 연구 발표된 문헌을 중심으로 그를 검토 고찰하여 보았다. 그 결과를 요약하면 다음과 같다. 우리나라 아동들이 출생후 6개월까지는 주로 모유에 의존하고, 약간이 인공영양에 의존하고 있는데, 이 시기에는 다른 선진제국의 어린이들의 성장발육과의 별 차이가 없었다. 그러나 이유기인 $6{\sim}30$개월 사이에는 우리나라 어머니들의 이유에 대한 지식이 빈약하고 이들 음식물을 그대로 아기들에게 적용하기 힘든 이유등으로 아이들에게 보급해서 주는 음식물이 빈약함으로 말미암아 다른 선진제국 아이들에 비해서 이 시기에 체위가 많이 떨어지고 빈혈현상을 나타내는 것을 볼 수 있다. 이때 고양군에서 농민의 아동들에 대해서 시행한 영양조사 내용을 보면, 전체 칼로리도 부족되고 특히 단백질, 철분, 칼슘, 비타민 A, B복합제가 전반적으로 부족되는 것을 알았다. 이는 어머니들의 육아와 이유식에 대한 지식의 결집도 중요한 역할을 했겠거니와 더욱더 중요한것은 우리나라 어른들의 식습관이, 민족적 전통적으로 짜고, 매웁고, 딱딱해서 이를 아기들에게 그대로 적응해서 먹이기가 힘든 것도 더 중요한 이유가 된다. 어른들을 위한 음식물에도 맵지 않고, 짜지 않으며, 아기들도 즐겨 먹을 수 있는 유동식 혹은 반유동식으로 된 영양가치가 좋은 음식물을 발전시켜 애용하도록 하는 것이 민족적인 긴급한 과제라고 하겠다. 그렇지 않으면 선진제국 모양으로 이유기에 있는 아이들을 위해서 아이들이 즐겨 먹을 수 있는 이유식을 크게 발전시켜서 이유기의 아이들에게 저렴하게 공급할 수 있는 생산공장과 제도의 마련이 긴급하다고 하겠다. 우리 민족의 체위를 향상 시키는데 영양학자와 영양사들에게 기대 하는 바가 크다. 육아하는 어머니의 교육정도와경제적인 면이 아기 체위에 주는 영향에 대한 연구에서는 교육정도가 높고, 경제적으로 좋은 위치에 있는 엄마가, 교육정도가 낮고 경제적으로 가난한 위치에 있는 어머니보다 아이들 체위에 훨씬 좋게 기를 수 있다는 것을 알았다. 특히 이유기에 들어간 6개월 이후에 체위 격차가 심한 것을 볼 때 보충해 주는 음식물의 적절한 보급이 얼마나 필요한가를 말해 주는 것이며 이에는 어머니의 교양과 열의와 경제적 뒷받침이 얼마나 필요하다는 것을 말해주는 것이다. 지역별로 서울에서 부유층 가정과 학교 아동, 농촌 지역 가정과 학교아동, 강원도 산간지역 가정과 학교 아등으로 나누어서 자라나는 아동들의 체위와 이학적 소친을 연구 조사한 바로는 서울 부유층이 제일 좋고 그 다음이 서울 빈한층, 강원도 산간지역 아동의 순으로 좋았으며 농촌지역 아종은 체위가 가장 떨어졌다. 구각증, 구내염등은 강원도 산간지역 아동에 제일 많고, 다음이 농촌지역, 서울 판자촌지역의 순으로 많으며 서울부유충지역이 가장 적어서 이는 산간일수록 또 는 빈한할수록 등물성단백질이 부족되는 것을 말해 주는 것이다. 지역별 혈색소치는 109gm/100ml 이하인 경우가 서울 빈한층가정 아동에 제일 많아서 51.7%나 되며 다음이 산간지역가정, 농촌지역가정의 순으로 많으며 서울부유층가정 아동은 2.2%에 지나지 않는다. 한국아등의 체위와 일본아동의 체위를 비교해 본 바로는 출생 후 약 12개월까지는 별차가 없지만 그후로는 점차로 그 격차가 심해져서 $12{\sim}15$세 사이에 평균체중면에서 5kg정도, 신장면에서 5cm정도의 큰 격차를 나타냈다. 그러나 그후로 성인이 되어감에 따라서 그 격차는 좁아졌다. 이는 일본국이 경제적으로 우리보다 우위에 있음에 따라서 아이들 성장에 필요한 영양분을 적기에 적당하게 공급해 주었기 때문이며 우리는 많은아이들이 가만하기 때문에 그 나이에 맞는 영양분 보급을 적당히 하지 못한 결과로 생각할 수 밖에 없다. 한국 아동의 발육표준치를 1965년초에 측정한 것과 1975년도에 측정한 것을 비교해 본 결과 1975년 치는 1965년도치에 비해 체중과 신장 면에서 상당히 증가하였다. 이는 우리라라 경제상태가 좋아지면서 굶는 아이들이 적어지거나 없어졌을 뿐만 아니라 식품 내용이 개선된 때문으로 해석된다. 따라서 민족의 체위가 개선되고 향상되려면 제일 먼저 해결되어야 할 문제는 국가의 경제력이 향상될뿐만 아니라 빈부의 격차가 적어져서 우리나라에 생을 받은 사람은 누구나 굶는 자가 없이 균형 있는 영양분을 충분히 섭취 할 수 있는기회를 갖도록 되어야 하겠다. 영양학자와 식품관리자들은 우러나라 아이들이 타고난 체위의 잠재성을 충분히 키우기 위해서 어떠한 음식물이 얼마나 필요한지 계산하여 이를 작물로 충분히 충당할 수 있도륵 계획해서 농수산물을 생산하도록 해야겠다. 영양학자들은 우리 민족의 음식물을 개선해서 아기들이 즐겨 먹을 수 있는 어른들의 음식물, 영양식을 발전시켜 이유기에 받는 우리 어린아기들의 수난을 조속히 극복하는 것이 바람직하다. 또한 영양에 대한 교육을 국민학교, 중학교 교과과정에 많이 넣어서 교육하고 영양에 대한 교육과 보급을 조직적으로, 신문, 라디오, 텔레비젼등을 통해서 성인교육을 강화해서 모든 국민들이 적은 돈을 가지고도 보다 나은 영양물을 구해서 섭취할 수 있는 지혜를 가지도록 해야겠다. 국민학교 재학중 및 졸업시기를 전후딘서 체위가 많이 떨어지며 그 영향이 일생동안 간다는 것을 감안할 때 국민학교에서 점심 한끼라도 영양식을 보급해서 우리 국민체위 향상을 위해서 나라에서 직접적으로 공헌하는 바가 있었으면 한다.

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Effects of Dietary Protein and Energy on the Growth and Body Composition of Growing Rats (단백질과 에너지 수준이 흰쥐의 성장 및 체조성에 미치는 영향)

  • Chang, Y.K.;Han, I.K.
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.11 no.1
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    • pp.57-68
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    • 1982
  • In order to investigate the effect of dietary protein and energy on growing female and male rats, Sprague-Dawley 90 female rats and 54 male rats of 3 weeks old weighing approximately 70-80g and 65-75g, respectively, were subjected to feeding trials for 8 weeks and then subsquently to metabolic trials for 2 weeks. Three dietary energy levels (3200, 3600, 4000 kcal ME/kg) were employed and each energy level contained three protein levels (15, 25, 35% of 3600 kcal ME/kg) and three fat levels (10, 20, 40% of 3600 kcal ME/kg) by addition of an appropriate amount of carbohydrate and the following results were obtained. The body weight gain of female rats was highest for LPHE ration but that of male rats was highest for LPME ration. The weight gains both of female and male rats were not affected by the level of protein. Food efficiencies both of female and male rats was affected by the level of protein, whereas that of male rats was not. Protein efficiencies of female and male rats were highest at low protein level and tended to decrease as the level of protein increased, but that of female rats was highest at high energy level, while that of male rats was highest at medium energy level. The analysis of the body composition after feeding trials for 8 weeks has shown that the contents of body water and protein were not affected by protein level both in female and male rats. The content of body fat increased remarkably as the protein and energy levels increased in case of female rats, but it was not affected by the protein and energy levels in case of male rats. From the above-mentioned experimental results it may be con eluded that the best formula of diet of growing female rats may be composed of low protein (13%) and high energy levels (4000 kcal/kg) whereas that for male rats may be composed of low protein (13%) and medium energy levels (3600 kcal/kg), since all the efficiencies of food, protein and energy have shown to be best at these levels.

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Methodologies for Enhancing Immersiveness in AR-based Product Design (증강현실 기반 제품 디자인의 몰입감 향상 기법)

  • Ha, Tae-Jin;Kim, Yeong-Mi;Ryu, Je-Ha;Woo, Woon-Tack
    • Journal of the Institute of Electronics Engineers of Korea CI
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    • v.44 no.2 s.314
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    • pp.37-46
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    • 2007
  • In this paper, we propose technologies for enhancing the immersive realization of virtual objects in AR-based product design. Generally, multimodal senses such as visual/auditory/tactile feedback are well known as a method for enhancing the immersion in case of interaction with virtual objects. By adapting tangible objects we can provide touch sensation to users. A 3D model of the same scale overlays the whole area of the tangible object so the marker area is invisible. This contributes to enhancing immersion. Also, the hand occlusion problem when the virtual objects overlay the user's hands is partially solved, providing more immersive and natural images to users. Finally, multimodal feedback also creates better immersion. In our work, both vibrotactile feedback through page motors, pneumatic tactile feedback, and sound feedback are considered. In our scenario, a game-phone model is selected, by way of proposed augmented vibrotactile feedback, hands occlusion-reduced visual effects and sound feedback are provided to users. These proposed methodologies will contribute to a better immersive realization of the conventional AR system.

Effect of Types of Egg Shell Calcium Salts and Egg Shell Membrane on Calcium Metabolism in Rats (난각 칼슘염의 종류와 난막의 존재유무가 흰쥐의 칼슘대사에 미치는 영향)

  • Noh, Kyung-Hee;Lee, Sang-Hyun;Ma, Jie;Zhou, Yusi;Kim, Jae-Cherl;Kim, Myo-Jeong;Song, Young-Sun
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.35 no.7
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    • pp.853-859
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    • 2006
  • This study was carried out to investigate the effect of egg shell calcium salt types and egg shell membrane on calcium metabolism in rats. Sprague-Dawley male rats, 4 weeks of age, were fed on free-calcium diets for 2 weeks after adjustment period. Rats weighing approximately $247{\pm}2.3g$ were divided into 6 groups and were fed on the experimental diets containing 0.2% calcium for 4 weeks. Experimental groups were as follows; {ES(M+)} (egg shell powder diet with egg shell membrane), {ES(M-)} (egg shell powder diet without egg shell membrane), {AC(M+)} (egg shell calcium acetate diet with egg shell membrance), {AC(M-)} (egg shell calcium acetate diet without eg shell membrane), {GC(M+)} (egg shell calcium glucuronate diet with egg shell membrane) and {GC(M-)} (egg shell calcium glucuronate diet without egg shell membrane). Bone length of femur was significantly different by the types (p<0.05) of egg shell calcium salts. Bone mineral density of femur showed the highest level in AC(M-) group. Calcium content of femur and calcium absorption rate were higher in egg shell calcium salt groups than in eg shell powder groups. Calcium absorption rate and retention were significantly different (p<0.05) among the types of eg shell calcium salts and were higher in the AC(M-) group than in the other groups. Alkaline phosphatase activity, parathyroid hormone and osteocalcin levels of serum showed no significant difference among the experimental groups. From the above results, it is concluded that bioavailability of calcium is higher in groups of egg shell calcium salts compared to those in egg shell powder, even though egg shell membrane has no effect on calcium metabolism. Thus, these findings suggest the possibility of using egg shell calcium salts as a functional food material related to calcium metabolism.

A Newborn Case of Maple Syrup Urine Disease Type 1B Presenting with Lethargy and Central Apnea (기면과 중추성 무호흡으로 나타난 단풍시럽뇨병 Type 1B 신생아 1례)

  • Kang, Youngtae;Choi, Sung Hwan;Ko, Jung Min;Shin, Seung Han;Kim, Ee-Kyung;Kim, Han-Suk
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.2
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    • pp.43-49
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    • 2018
  • Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.

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