• 제목/요약/키워드: EE2

검색결과 936건 처리시간 0.029초

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth

  • Heo, Ju Sun;Choi, Ka Young;Sohn, Se Hyoung;Kim, Curie;Kim, Yoon Joo;Shin, Seung Han;Lee, Jae Myung;Lee, Juyoung;Sohn, Jin A;Lim, Byung Chan;Lee, Jin A;Choi, Chang Won;Kim, Ee-Kyung;Kim, Han-Suk;Kim, Beyong Il;Choi, Jung-Hwan
    • Clinical and Experimental Pediatrics
    • /
    • 제55권11호
    • /
    • pp.438-444
    • /
    • 2012
  • Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

Effect of Different Source of Energy on Urea Molasses Mineral Block Intake, Nutrient Utilization, Rumen Fermentation Pattern and Blood Profile in Murrah Buffaloes (Bubalus bubalis)

  • Hosamani, S.V.;Mehra, U.R.;Dass, R.S.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제16권6호
    • /
    • pp.818-822
    • /
    • 2003
  • In order to investigate the effect of different sources of energy on intake and nutrient utilization from urea molasses mineral block (UMMB), rumen fermentation pattern and blood biochemical constituents, 18 intact and 9 rumen fistulated male Murrah buffaloes aged about 3 years and average weight 310.8 kg were randomly allocated into three groups of 9 animals in each, thus each group having 6 intact and three rumen fistulated buffaloes. All animals were fed individually for 90 days. All buffaloes were offered wheat straw as basal roughage and urea molasses mineral block for free choice of licking. Three different energy sources viz., barley grain, (group I), maize grain (group II) and jowar green (group III) were offered to meet their nutrient requirement as per Kearl (1982). At the end of feeding trial, a metabolism trial of 7 days duration was carried out on intact animals to determine the digestibility of nutrients. Rumen fermentation studies were carried out on rumen fistulated animals. After the metabolism trial blood was collected from intact animals to estimate the nitrogen constituents in blood serum of animals fed on different sources of energy. Results revealed no significant difference in the intake of UMMB in three groups. Similarly, the intake of DM (kg), DCP (g) and TDN (kg) per day was similar in three groups statistically. The apparent digestibility of dry matter (DM), organic matter (OM), ether extract (EE) and nitrogen free extract (NFE) was significantly (p<0.05) more in group II than group III, whereas the digestibility of DM, OM and NFE was similar in group I and II. The digestibility of crude fiber (CF) and all the fiber fractions i.e. NDF, ADF, cellulose and hemicellulose was alike in 3 groups. Nitrogen balance (g/d) was significantly (p<0.05) more in group III as compared to group I and II, which were alike statistically, though the N intake (g/d) was similar in 3 groups but N balance (g/d) was significantly (p<0.05) less in group III as compared to other 2 groups. Significantly (p<0.05) higher concentration of total volatile fatty acids (TVFA), total nitrogen (TN) and its fractions were observed in group I and II as compared to group III. There was no effect on rumen pH, rumen volume and digesta flow rate due to different sources of energy in 3 groups. Similarly the blood serum biochemical parameters (NH3-N, urea-N and total protein) were statistically identical in 3 groups.

레가시 시스템의 컴포넌트화 방법론 개발을 위한 프로그램 분석 활동 (Program Analysis Activities for Development of Componentization Methodology for of Legacy System)

  • 차정은;김철홍;양영종
    • 한국정보처리학회:학술대회논문집
    • /
    • 한국정보처리학회 2002년도 추계학술발표논문집 (하)
    • /
    • pp.2119-2122
    • /
    • 2002
  • 기업의 비즈니스 프로세스가 복잡, 다양해짐에 따라, 현재 운영 시스템에 대한 급격한 기술적 변화를 수용하고 이를 조직적 측면의 기업 프로세스로 적용하기 위해 레가시 시스템의 현대화가 요구된다. 따라서, 현재의 기업들은 다양한 사용자들이 각자 그들의 관점에서 필요한 비즈니스 요구들을 웹 상에서 처리시킬 수 있도록 J2EE, .NET 등으로 대표되는 컴포넌트 및 웹 서비스 기술을 적용한 새로운 e-business 환경을 수용해야만 한다. 하지만, 기업 조직의 중요한 지식과 프로세스들을 처리하는 시스템들은 대부분 과거(Legacy)의 기술에 의해 개발되어졌으며, 이러한 시스템들을 새로운 비즈니스 환경에 적용하기에는 웹 환경을 위한 분산 아키텍쳐의 결여와 개방성과 표준화 미흡으로 시스템의 유지보수에 많은 어려움을 가진다. 또한, 방법론 차원에서 재공학의 절차와 기법을 체계적으로 정의하고 지원하기 위한 노력이 매우 부족한 실정이다. 본 논문에서는 레거시 시스템을 새로운 시스템 환경으로의 변환 및 통합을 위한 재공학 방법론 개발을 목적으로 프로그램 분석 활동을 설명한다. 본 논문에서 개발하고자 하는 방법론은 다양한 추상화 수준에서 역공학 정보를 복구하고, 컴포넌트화 단계를 통해 새로운 시스템으로 진화할 수 있는 절차 및 기법들을 제공한다. 레거시 시스템 컴포넌트화 방법론은 변환계획 단계, 역공학 단계, 컴포넌트화 단계, 인도 단계의 4 단계로 구성되어 있으며, 본 논문에서는 전체 단계 중 가장 기초가 되고 중요한 단계인 역공학 단계에 초점을 두고 프로그램 분석을 위한 절차 및 과정의 주요 지침들을 제시한다.0보다 유의적으로 우수하였으며, 맛, 다즙성 및 전체적인 기호도는 TMR-0 및 TMR-1 사이에 유의성이 없었다(p<0.05).能性)을 알아보고자 본(本) 실험(實驗)을 실시(實施)한 결과(結果), 유의성(有意性) 있는 결과(結果)를 얻었기에 보고(報告)하는 바이다.또한 이들 상피세포(上皮細胞)들을 투과전자현미경적(透過電子顯微鏡的)으로 관찰(觀察)하였을 때 초미세구조(超微細構造)가 잘 보존(保存)되었으나 Langer-hans cell내(內)의 Birbeck granule은 유리전(遊離前) 피부상피조직내(皮膚上皮組織內)의 Langerhans cell내(內)의 Birbeck granule에 비(比)해 수적(數的)으로 현저히 감소(減少)되어 있었다. 그러나 Thy-1 양성(陽性) dendritic cell에서 볼 수 있는 dense-core 과립(顆粒)은 별변화(別變化)없이 쉽게 관찰(觀察)될 수 있었다. 조직배양(組織培養)을 한 견(犬)의 keratinocyte에 대(對)해 사람 pemphigus vulgaris의 항체(抗體)로 반응(反應)시킨 후 protein-A gold(15 nm)로 표식(標識)시킨 바 제일 바깥 상층(上層)의 keratinocyte에 있어서 세포막표면(細胞膜表面)을 따라 표식(標識)되어 세포막항원(細胞膜抗元)을 나타내었으며, 이와 같은 소견(所見)으로 미루어 정상피부(正常皮膚) 중층편평상피세포(重層扁平上皮細胞)에서도 동일(同一)한 소견(所見)을 관찰(觀察)할 수 있다고 본다.al remnants, Resorption of fetus로 관찰된 것이다. Fetal death는 수정후

  • PDF

백두산 빙하지형의 존재 가능성과 제4기 화산활동과의 관계 (Some Evidences for Glacial Landforms on the Baekdusan and Its Implications to Quaternary Volcanic Eruptions)

  • 이성이;성영배;강희철;최광희
    • 대한지리학회지
    • /
    • 제47권2호
    • /
    • pp.159-178
    • /
    • 2012
  • 백두산의 현재 지형은 반복적인 화산활동과 함께 신생대 제4기 기후변화로 추동된 빙하 활동의 결과물이다. 그러나 여전히 백두산 빙하지형의 존재에 대한 의문들은 해소되지 못해왔다. 이에, 본 연구에서는 야외조사를 통하여, 빙하지형에 관련된 지형들을 제시하고, 이의 연대측정을 실시하였으며, 제4기 화산 활동과의 관련성을 살펴보았다. 가속기 질량 분석기를 이용하여 빙하의 후퇴 이후 지표면에 노출되었을 백두산 천지 서사면의 빙하 기반암 지형 면에 대하여, 우주선 유발 동위원소 $^{36}Cl$의 농도를 측정하여 46~26ka의 표면노출연대를 얻었으며, 이는 적어도 천지 서쪽 사면은 지난 최종빙기 이후의 화산활동에 영향을 받지 않은 것을 지시하며, 특히 대략 천 년 전에 있었다고 보고되는 폭발성 분화에도 영향을 거의 받지 않았을 가능성이 매우 높다. 또한, 화산 쇄설물의 비대칭적 퇴적을 고려했을 때, 계절적으로 이 분화는 북서풍이 탁월한 겨울철에 발생한 것으로 사료된다. 빙하지형과 이의 연대 결과를 바탕으로 지형 발달과정을 복원해보면, 백두산의 빙하 지형은 지난 빙기까지 곡빙하(valley glacier)들이 연합한 빙모(ice cap) 형태에서 고립된 곡빙하를 거쳐 화산체 내부의 소규모 권곡(cirque) 형태로 변화한 것으로 추론된다.

  • PDF

열산화 처리된 Ti-Ag 합금의 세포적합성 (CYTOCOMPATIBILITY OF THERMALLY OXIDIZED TI-AG ALLOYS)

  • 김호중;오근택;이지환;김경남;한동후
    • 대한치과보철학회지
    • /
    • 제42권4호
    • /
    • pp.333-343
    • /
    • 2004
  • Statement of problem: In its preceding work, change in surface characteristics were investigated in consideration that both microtopograpy and macroscopic configuration of implants surface are two of the most important factors, in that they can construct agreeable environment by raising surface energy, to affect osseointegration and biocompatibility explained by cell proliferation. Purpose: This study focused on examining cytocompatibility of dental implants materials Ti-Ag alloys, of which mechanical and electrochemical superiority to cp-Ti or Ti6Al4V were verified, in comparison with that of cp-Ti, and Ti6Al4V. Materials and methods: In this regard, MTT tests for L-929, the fibroblast connective tissues and cell proliferation tests for osteoprogenitor cells, MC3T3-E1 were performed on cp-Ti, Ti6Al4V, and Ti-Ag alloys following thermal oxidation according to appropriate heat treatment temperature(untreated, 400, 600, $800^{\circ}C$) and heat treatment duration(untreated, 0.5, 1, 4 hr). Results: The MTT tests on fibroblasts L-929 resulted in cell viability of over 90% in all experimental group entities, where, especially, the 100% of the viability for Ti-Ag alloys specimens accounted for the slightest adverse effect of ions release from those alloys on the cell. In MC3T3-E1 proliferation tests, the population of cells in the experimental group was roughly increased as experimentation proceeded, after two to four days. Proliferation showed highest viability for most of specimens, including Ti2.0Ag, treated at $600^{\circ}C$. Conclusion: In conclusion, it is the heat treatment temperature, not the duration that has considerable effects on thermal oxidation of specimens. Ti-Ag alloys treated at $600^{\circ}C$ proved to have the best surface morphology as well as cytocompatibility when compared with Ti or Ti6Al4V for short-term biocompatibility tests.

배양온도 변화에 대한 전이성 암세포의 반응 (Response of Metastatic Cancer Cells to Thermal Changes in vitro)

  • 안상길;권영이;최호순;권중균;유진영;김종룡;김원규
    • Applied Microscopy
    • /
    • 제37권4호
    • /
    • pp.239-248
    • /
    • 2007
  • 암세포가 있는 장소의 온도를 변화시키는 것은 하나의 암 치료 방법이 될 수 있다. 명확한 기전은 아직 잘 밝혀져 있지 않지만, 고온은 미토콘드리아로 신호를 전달해서 cytochrome c를 분비시키는 세포자멸사로의 길로 유도하는 것으로 알려져 있다. 저온은 $30^{\circ}C$ 미만에서 세포자멸사를 유도하지만 심하지 않은 저온에서는($35{\sim}33^{\circ}C$ 혹은 $31{\sim}29^{\circ}C$)오히려 세포자멸사를 막는 것으로 알려져 있다. CC-t6와 GB-d1세포 주는 림프절로 전이된 사람의 담관암과 담낭암에서 확립한 것으로, 이와 같은 전이성 암세포가 온도 변화에 어떻게 반응을 하는지를 연구하기 위해 고온노출($37{\rightarrow}43^{\circ}C$)과 저온노출($37{\rightarrow}17.4^{\circ}C$)을 시행하였다. 세포의 종류나 온도 변화를 통한 스트레스의 방법과 관계없이 죽는 세포가 관찰되었으며, 고온노출이 가장 심한 영향을 주었다. 이런 죽어가는 세포는 세포자멸사가 아닌 세포괴사의 경로를 거치고 있었다. 투과전자현미경을 이용한 관찰에서 세포자멸사적인 모습은 보이지 않았고, caspase-3, -9, cytochrome c, Bax 같은 세포자멸사와 관련된 단백질의 변화도 관찰되지 않았고, 열충격단백질 70과 27도 증가하였다. 결국 CC-t6와 GB-d1 세포는 온도변화를 통한 스트레스를 주었을 경우 세포괴사로 죽음을 알 수 있었다. 온도변화를 통한 스트레스는 열충격단백질의 증가와 함께 세포괴사를 일으켰다. GB-d1과 CC-t6 세포에서 고온은 가장 심각하게 세포괴사를 일으켰으며, 저온은 초기에는 세포괴사를 유발하였으나 12시간 경과후에는 세포분열이 더욱 활발하게 일어나 세포의 생명력을 연장시켜주었다. 결국 이 실험에서는 전이성 암세포를 제거하는 방법으로는 고은이 가장 효과적이며 유용함을 알 수 있었다.

Determination and Prediction of the Amino Acid Digestibility of Sunflower Seed Meals in Growing Pigs

  • Liu, J.D.;Li, Q.Y.;Zeng, Z.K.;Li, P.;Xu, X.;Wang, H.L.;Zhang, S.;Piao, X.S.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제28권1호
    • /
    • pp.86-94
    • /
    • 2015
  • This experiment was conducted to evaluate the chemical composition and amino acid (AA) digestibility of sunflower seed meal (SFSM) and to use this data to develop prediction equations for estimating AA digestibility for growing pigs. Ten SFSM were collected from five provinces in China. Twelve barrows ($38.8{\pm}4.6kg$), fitted with ileal T-cannula were allotted into two $6{\times}6$ Latin square designs. Each of six experimental periods comprised a 5-d adaption period followed by a 2-d collection of ileal digesta. The ten test diets contained 50% SFSM as the sole source of AA. Another nitrogen-free diet was used to measure the basal endogenous losses of crude protein (CP) and AA. Chromic oxide (0.3%) was used as an inert marker in each diet. There was considerable variation (CV>10%) among the ten SFSM in chemical composition (dry matter [DM]). The concentration of CP and ether extract (EE) ranged from 29.33% to 39.09% and 0.88% to 11.33%, respectively. Crude fibre (CF), neutral detergent fibre and acid detergent fibre ranged from 21.46% to 36.42%, 38.15% to 55.40%, and 24.59% to 37.34%, respectively. There was variation among the ten SFSM in apparent ileal digestibility (AID) and standardized ileal digestibility (SID) for lysine and threonine, which ranged from 63.16 to 79.21 and 55.19% to 72.04% for AID and 67.03% to 82.07% and 61.97% to 77.01% for SID, respectively. The variation in CP and methionine ranged from 60.13% to 74.72% and 74.79% to 88.60% for AID and 66.70% to 79.31% and 77.16% to 90.27% for SID, respectively. Methionine was a good indicator to predict AA digestibility. These results indicate that conventional chemical composition of SFSM was variable (CV>10%) among the ten SFSM (DM). The results of AID, SID and prediction equations could be used to evaluate the digestibility of SFSM in growing pigs.

By-product of Tropical Vermicelli Waste as a Novel Alternative Feedstuff in Broiler Diets

  • Rungcharoen, P.;Therdthai, N.;Dhamvithee, P.;Attamangkune, S.;Ruangpanit, Y.;Ferket, P.R.;Amornthewaphat, N.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제26권12호
    • /
    • pp.1732-1741
    • /
    • 2013
  • Two experiments were conducted to determine physical and chemical properties of vermicelli waste (VW) and effect of VW inclusion levels on growth performance of broilers. In experiment 1, VW samples were randomly collected from vermicelli industry in Thailand to analyze nutritional composition. Vermicelli waste contained 9.96% moisture, 12.06% CP, 32.30% crude fiber (CF), and 0.57% ether extract (EE), as DM basis. The ratio of insoluble:soluble non-starch polysaccharide (NSP) was 43.4:8.9. A total of 120 chicks (6 pens per treatment and 10 chicks per pen) were fed a corn-soybean meal-based diet or 20% VW substituted diet to determine the apparent metabolizable energy corrected for nitrogen retention ($AME_n$) of VW. The $AME_n$ of VW was $1,844.7{\pm}130.71$ kcal/kg. In experiment 2, a total of 1,200 chicks were randomly allotted to 1 of 4 dietary treatments for 42-d growth assay. There were 300 chicks with 6 pens per treatment and 50 chicks per pen. The dietary treatments contained 0%, 5%, 10%, or 15% VW, respectively. All diets were formulated to be isocaloric and isonitrogenous. From 0 to 18 d of age chicks fed VW diets had higher (p<0.001) feed conversion ratio (FCR) compared with those fed the control diet. No difference was observed during grower and finisher phase (19 to 42 d). Chicks fed VW diets had lower relative weight of abdominal fat (p<0.001) but higher relative weight of gizzard (p<0.05) than those of chicks fed the control diet. Increasing VW inclusion levels increased ileal digesta viscosity (p<0.05) and intestinal villus height of chicks (p<0.001). For apparent total tract digestibility assay, there were 4 metabolic cages of 6 chicks that were fed experimental treatment diets (the same as in the growth assay) in a 10-d total excreta collection. Increasing VW inclusion levels linearly decreased (p<0.05) apparent total tract digestibility of DM and CF.

Effect of Feeding Urea and Acetic Acid Treated Wheat Straw on the Digestibility of Nutrients in Adult Male Murrah Buffaloes (Bubalus bubalis)

  • Mehra, Usha R.;Dass, R.S.;Verma, A.K.;Sahu, D.S.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제14권12호
    • /
    • pp.1690-1695
    • /
    • 2001
  • Wheat straw was treated on laboratory scale with 4% urea at a moisture level of 50% along with different amounts of acetic acid (AA) to fix various levels of ammonia nitrogen (15, 30, 45 and 60%) evolved from urea and stored for 4 weeks. Chemical composition of the treated samples revealed a significant (p<0.01) increase in N content of the samples where AA was added. The N content of the ammoniated straw was only 1.21% which increased to 2.58 with the addition of AA to trap 30% $NH_3-N$. The concentration of free $NH_3-N$ in the straw was significantly (p<0.01) less when more than 15% $NH_3-N$ was trapped with AA. There was significant increase (p<0.01) in N disappearance and depression in NDF and hemicellulose disappearance, when AA was used to trap 30% $NH_3-N$. Large scale treatment of wheat straw with 4% urea at a moisture level of 50% along with AA (to trap 30% $NH_3-N$) increased the N content, but not as much as in laboratory scale treatment. In vivo experiment conducted on nine adult male buffaloes divided into three groups revealed no difference in the intake of DM, OM, NDF, ADF, cellulose and hemicellulose among group I (ammoniated straw), group II (AA treated ammoniated straw) and group III (AA treated ammoniated straw +1 kg barley grain), but the intake was significantly (p<0.05) more in groups where AA treated straw was fed as compared to only ammoniated straw fed group. However EE digestibility was depressed in group II. The digestibility of cellulose and hemicellulose both depressed significantly (p<0.05) in group II and III as compared to group I. Animals in all the 3 groups showed positive nitrogen balance and it was significantly more in group II and III as compared to group I. DCP intake was significantly (p<0.05) more in group II and III as compared to group I, but there was no significant difference among the three groups in TDN intake. It can be concluded that AA (to trap 30% $NH_3-N$) is effective in capturing the excess ammonia released during urea ammoniation of straw and improving its nutritive value, as well as animal performance.

Influence of Dietary Supplementation of Condensed Tannins through Leaf Meal Mixture on Intake, Nutrient Utilization and Performance of Haemonchus contortus Infected Sheep

  • Pathak, A.K.;Dutta, Narayan;Banerjee, P.S.;Pattanaik, A.K.;Sharma, K.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제26권10호
    • /
    • pp.1446-1458
    • /
    • 2013
  • The study assessed the effect of dietary supplementation of leaf meal mixture (LMM) containing condensed tannins (CT) on feed intake, nutrient utilization and performance of sheep infected with Haemonchus contortus. Eighteen adult sheep of similar age and body weight ($25.03{\pm}1.52$) were included in this study and out of these, 12 sheep were infected with single dose of infective third stage larvae of H. contortus at 2,000 larvae per sheep. The experimental sheep were allocated in three different groups' i.e. negative control (NC; no infection), control (C; H. contortus infected) and treatment (T; H. contortus infected+CT at 1.5% of the DM through LMM) and the experiment was conducted for a period of 90 d. The intake of dry matter (DM), organic matter (OM) and digestibility of DM, OM, neutral detergent fibre (NDF) and acid detergent fibre (ADF) were comparable among three animal groups. However, digestibility of crude protein (CP) and ether extract (EE) were significantly (p<0.05) higher in NC group as compared to both C and T groups. Nitrogen (N) retention (g/d or % of N intake) was significantly (p = 0.038) lower in C group as compared to T and NC groups. Daily intake (g/kg $W^{0.75}$) of digestible crude protein (DCP), digestible organic matter (DOM) and total digestible nutrient (TDN) did not differ significantly (p<0.05) in the three groups. Haemoglobin (Hb) and packed cell volume (PCV) were significantly (p<0.001) higher in treatment group as compared to control. The level of Hb and PCV reduced (p<0.001) after 30 days of experimental feeding. CT significantly (p<0.001) reduced serum urea in T group as compared to NC and C groups. Serum proteins differed significantly (p<0.01) among the three groups. The activity of serum enzymes AST, ALT, ALP and LDH were also statistically non significant (p<0.05) among treatments. The weight of abomasal lymph nodes (ALN) in T group was higher (p<0.05) than in C group. Treatment group had lower (p<0.05) total worms and fecal egg count compared to control group. It may be concluded that dietary supplementation of CT through LMM significantly improved the N retention, and inhibited the different developmental stages of Haemonchus contortus in experimental sheep.