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Hereditary Tyrosinemia Type I (Hereditary Tyrosinemia Type I 환아의 NTBC 치료 경험)

  • Kang, Hyun-Young;Kim, Sook Za;Song, Wung Joo;Chang, Mi-Young
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.4 no.1
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    • pp.13-17
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    • 2004
  • Hereditary tyrosinemia type I (fiunarylacetoacetate hydrolase deficiency) is an autosomal recessive inborn error of tyrosine metabolism that results in liver failure in infancy or chronic liver disease with cirrhosis, frequently complicated by hepatocellular carcinoma in childhood or early adolescence. Early detection of this condition is very important to early intervention for better prognosis of patients. Neonatal screening test using tandem mass spectrometry (MS-MS) is performed, and this method facilitates detection of the inborn error of tyrosine. For early treatment of tyrosinemia type I, phenylalanine and tyrosine restricted diet and NTBC (2-nitro-4-trifluoromethylbenzoyl-1,3-cyclohexanedione) for inhibition of succinylacetone production are recommended. We studied a 10-month-old Korean boy with tyrosinemia type I whose condition was not discovered earlier through conventional neonatal screening testing available in Korea. The patient presented hyperbilirubinemia, liver failure, bleeding tendency, colicky pain and skin melanin pigmentation in neonatal period. MS-MS made it possible to detect tyrosinemia type I and allowed immediate treatment of the patient. This was the first successful NTBC trial on tyrosinemia type I patient in Korea.

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Collagen Scaffolds in Cartilage Tissue Engineering and Relevant Approaches for Future Development

  • Irawan, Vincent;Sung, Tzu-Cheng;Higuchi, Akon;Ikoma, Toshiyuki
    • Tissue Engineering and Regenerative Medicine
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    • v.15 no.6
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    • pp.673-697
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    • 2018
  • BACKGROUND: Cartilage tissue engineering (CTE) aims to obtain a structure mimicking native cartilage tissue through the combination of relevant cells, three-dimensional scaffolds, and extraneous signals. Implantation of 'matured' constructs is thus expected to provide solution for treating large injury of articular cartilage. Type I collagen is widely used as scaffolds for CTE products undergoing clinical trial, owing to its ubiquitous biocompatibility and vast clinical approval. However, the long-term performance of pure type I collagen scaffolds would suffer from its limited chondrogenic capacity and inferior mechanical properties. This paper aims to provide insights necessary for advancing type I collagen scaffolds in the CTE applications. METHODS: Initially, the interactions of type I/II collagen with CTE-relevant cells [i.e., articular chondrocytes (ACs) and mesenchymal stem cells (MSCs)] are discussed. Next, the physical features and chemical composition of the scaffolds crucial to support chondrogenic activities of AC and MSC are highlighted. Attempts to optimize the collagen scaffolds by blending with natural/synthetic polymers are described. Hybrid strategy in which collagen and structural polymers are combined in non-blending manner is detailed. RESULTS: Type I collagen is sufficient to support cellular activities of ACs and MSCs; however it shows limited chondrogenic performance than type II collagen. Nonetheless, type I collagen is the clinically feasible option since type II collagen shows arthritogenic potency. Physical features of scaffolds such as internal structure, pore size, stiffness, etc. are shown to be crucial in influencing the differentiation fate and secreting extracellular matrixes from ACs and MSCs. Collagen can be blended with native or synthetic polymer to improve the mechanical and bioactivities of final composites. However, the versatility of blending strategy is limited due to denaturation of type I collagen at harsh processing condition. Hybrid strategy is successful in maximizing bioactivity of collagen scaffolds and mechanical robustness of structural polymer. CONCLUSION: Considering the previous improvements of physical and compositional properties of collagen scaffolds and recent manufacturing developments of structural polymer, it is concluded that hybrid strategy is a promising approach to advance further collagen-based scaffolds in CTE.

원전 I&C FSE 분류기준과 이에 따른 상용 소프트웨어의 원전 사용 승인기준

  • 김장열;권기춘
    • Proceedings of the Korean Nuclear Society Conference
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    • 1995.05a
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    • pp.1041-1046
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    • 1995
  • 본 논문에서는 원전 I&C Function System Equipment (FSE)의 분류기준을 제시하기 위하여 IEEE 730.1, IEEE 828, IEEE 1012 및 IEC 1226의 관련 표준들을 비교 분석하여 I&C FSE를 근간으로 계측제어 소프트웨어를 원전 I&C 계통의 기능에 따라 Type I, Type II, Type III 및 Type IV로 분류할 수 있는 분류기준, 분류절차 및 예를 제시하였다. 또한, 본 논문의 분류기준을 토대로 하여 최근 이슈가 되고 있는 상용 소프트웨어 (Commercial Off The Shelf Software)의 원전 사용 승인기준을 제시하였다.

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Girder Section of Continuous Bridges Spliced by Partial Post-Tensioning (부분 포스트텐션닝 방법에 의해 연속화된 교량의 주형단면)

  • 이환우;곽효경;송영용
    • Journal of the Korea Concrete Institute
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    • v.12 no.6
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    • pp.43-50
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    • 2000
  • In this paper, a new splicing method was applied to design the girder section of bridges with the span length of 25m, 30m, 35m, 40m and 45m. A U-type precast prestressed section was also determined for each bridge. Additionally, the sectional area, beam depth and Guyon's efficiency factor of the spliced U-type sections in each span were analyzed in comparison with the present I-type PSC bridges. As a result, in spite of an increase of 31%∼50% in the sectional areas compared with the I-type precast girders, the spliced U-type the beam depth of the spliced U-type girder was designed as 2,050 mm compared with the I-type precast girder of 2,600mm in a 40m span bridge. The sectional efficiency factors of the spliced U-type sections were analyzed as 0.76∼0.99. It shows that the spliced U-type sections ar of a superior structural efficiency in contrast to the average sectional efficiency factor of 0.66 value in the I-type girders.

Epithelioid sarcoma associated with neurofibromatosis type I

  • Hwang, Sung Oh;Lee, Soo Hyang;Lee, Han Byul
    • Archives of Craniofacial Surgery
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    • v.21 no.1
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    • pp.41-44
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    • 2020
  • In general, patients with neurofibromatosis type I have a higher risk than those with other types of neurofibromatosis of developing soft-tissue sarcomas related to the nervous system. We here present a 42-year-old man with neurofibromatosis type I who developed a protruding mass over only 2 weeks. The histopathological diagnosis was epithelioid sarcoma. Epithelioid sarcomas are rare and, to the best of our knowledge, no epithelioid sarcomas have been reported in patients with neurofibromatosis type I. Radical excision of the primary lesion was performed and postoperative radiotherapy and chemotherapy administered, as is recommended for epithelioid sarcoma. Our case emphasizes that patients with neurofibromatosis type I may develop malignant tumors.

Personality Type Test(MBTI) of Korean College Students with Symptoms of Temporomandibular Disorders (측두하악장애증상자의 성격유형검사(MBTI))

  • Park, Hye-Sook
    • Journal of Oral Medicine and Pain
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    • v.36 no.1
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    • pp.25-37
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    • 2011
  • The purpose of this study is to investigate the relationship between personality type and symptoms and contributing factors of temporomandibular disorders. 199 college students completed the MBTI(Myers-Briggs Type Indicator) and a questionnaire and collected data were analyzed by SAS 9.2 program. The obtained results were as follows : 1. The prevalence of symptoms of temporomandibular disorders and mean scales of positive answers of contributing factors appeared to be higher in I type, S type, T type, P type than in E type, N type, F type, J type. 2. ISTP and ISFP among 16 types of personality seemed to have higher prevalence of symptoms and contributing factors of temporomandibular disorders than other types of personality. 3. Symptom of TMJ pain during mouth opening seemed to occur more frequently in I type, S type, F type, J type than in E type, N type, T type, P type. 4. Contributing factors including clenching and stressful state occurred significantly more frequently in I type than E type. Gum chewing habit occurred significantly more frequently in E type than in I type. 5. Unilateral chewing habit occurred significantly more frequently in J type than in P type. 6. Nervous or sensitive persons had significantly higher mean scales of positive answers of subjective symptoms than relaxed or general persons. 7. General persons had significantly lower mean scales of positive answers of contributing factors than nervous, sensitive and relaxed persons. In conclusion, these results show that there is the relationship between personality and temporomandibular disorders and patient education and counselling considering personality type may contribute to treating patients with temporomandibular disorders.

Growth and Yield According to Various Bending Methods when Planting Seedlings Directly on Coir Substrate Slabs in Paprika Cultivation (파프리카 묘의 직접 정식 시 절곡 방법에 따른 생육 및 생산량)

  • Hur, Young Mun;Ko, Baul;Ku, Yang Gyu;Kim, Chul Min;Kim, Ho Cheol;Bae, Jong Hyang
    • Journal of Bio-Environment Control
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    • v.30 no.4
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    • pp.251-256
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    • 2021
  • This study aimed to compare the growth and production of paprika (Capsicum annuum) planted directly on a coir substrate slab according to the bending methods. The existing root direction was bent to 0° (I-type), 90° (L-type), and 180° (U-type), respectively. The weekly average growth such as stem length, diameter, and leaf area tended to be the highest in the I-type bending, but there was no statistical difference. Root weight at 46 weeks after planting was also about 1.3 to 1.7 times heavier in the I-type than the L- and U-type bending. As the yield produced by 330 plants by bending methods, the initial yield was the highest in the U-bending, but then the highest in the I-bending. Accordingly, the total yield was the highest in I-bending. Consequently, when planting on coir substrates directly in paprika cultivation, the I-type bending should be considered most suitable for securing root and plant vigor in the early stages of planting and for enhancing fruiting stability.

Type I Interferon Increases Inflammasomes Associated Pyroptosis in the Salivary Glands of Patients with Primary Sjögren's Syndrome

  • Seung-Min Hong;Jaeseon Lee;Se Gwang Jang;Jennifer Lee;Mi-La Cho;Seung-Ki Kwok;Sung-Hwan Park
    • IMMUNE NETWORK
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    • v.20 no.5
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    • pp.39.1-39.13
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    • 2020
  • Sjögren's syndrome (SS) is a chronic and systemic autoimmune disease characterized by lymphocytic infiltration in the exocrine glands. In SS, type I IFN has a pathogenic role, and recently, inflammasome activation has been observed in both immune and non-immune cells. However, the relationship between type I IFN and inflammasome-associated pyroptosis in SS has not been studied. We measured IL-18, caspase-1, and IFN-stimulated gene 15 (ISG15) in saliva and serum, and compared whether the expression levels of inflammasome and pyroptosis components, including absent in melanoma 2 (AIM2), NLR family pyrin domain containing 3 (NLRP3), apoptosis-associated speck-like protein (ASC), caspase-1, gasdermin D (GSDMD), and gasdermin E (GSDME), in minor salivary gland (MSG) are related to the expression levels of type I IFN signature genes. Expression of type I IFN signature genes was correlated with mRNA levels of caspase-1 and GSDMD in MSG. In confocal analysis, the expression of caspase-1 and GSDMD was higher in salivary gland epithelial cells (SGECs) from SS patients. In the type I IFN-treated human salivary gland epithelial cell line, the expression of caspase-1 and GSDMD was increased, and pyroptosis was accelerated in a caspase-dependent manner upon inflammasome activation. In conclusion, we demonstrate that type I IFN may contribute to inflammasome-associated pyroptosis of the SGECs of SS patients, suggesting another pathogenic role of type I IFN in SS in terms of target tissue -SGECs destruction.

A COMPARATIVE STUDY OF BOND STRENGTH OF RECYCLED BRACKETS (재생 브라켓의 전단접착강도에 관한 비교 연구)

  • Shur, Cheong-Hoon;Choi, Eun-Ah
    • The korean journal of orthodontics
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    • v.28 no.4 s.69
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    • pp.641-657
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    • 1998
  • This study was undertaken to compare the bond strength and the fracture site of new and recycled brackets according to the base design. 252 sound premolars extracted for orthodontic treatment were collected and Type I, Type II, Type III brackets were divided into four groups by recycling method Each bracket was then bonded to an extracted premolar. Instron Universal Testing Machine(model W) was used to measure the shear bond strength, and the surface of the recycled brackets were viewed in SEM For the analysis of the results, one way ANOVA and Scheffe's multiple range test was executed using the SPSSWIN program. 1. The shear bond strength showed statistically significant difference according to the bracket base design(p<0.001). Type III bracket(round indentation base, micro-etched) showed the highest bond strength, Type I bracket(foil-mesh base) was second, and Type II bracket(grooved integral base, micro-etched) was last. 2. The effect of recycling on the bond strength was different according to bracket type. The shear bond strength of Type I, Type II brackets showed the smallist reduction when treated for 1 minute in Big Jane(p<0.05), but the shear bond strength of Type III brackets showed no statistically significant difference according to recycling method(p>0.05). 3. In Type I, Type II brackets, frequent fracture site was bracket-resin interface, but in Type III brackets, about half of the resin was retained on the tooth surface frequently. 4. The shear bond strength was highest when about half of the resin was retained on the tooth surface(p<0.05). 5. The resin remnant on the bracket base after recycling had no effect on the shear bond strength.

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Effects of human collagen α-1 type I-derived proteins on collagen synthesis and elastin production in human dermal fibroblasts

  • Hwang, Su Jin;Kim, Su Hwan;Seo, Woo-Young;Jeong, Yelin;Shin, Min Cheol;Ryu, Dongryeol;Lee, Sang Bae;Choi, Young Jin;Kim, KyeongJin
    • BMB Reports
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    • v.54 no.6
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    • pp.329-334
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    • 2021
  • Collagen type I is the most abundant form of collagen in human tissues, and is composed of two identical α-1 type I chains and an α-2 type I chain organized in a triple helical structure. A previous study has shown that human collagen α-2 type I (hCOL1A2) promotes collagen synthesis, wound healing, and elastin production in normal human dermal fibroblasts (HDFs). However, the biological effects of human collagen α-1 type I (hCOL1A1) on various skin properties have not been investigated. Here, we isolate and identify the hCOL1A1-collagen effective domain (CED) which promotes collagen type I synthesis. Recombinant hCOL1A1-CED effectively induces cell proliferation and collagen biosynthesis in HDFs, as well as increased cell migration and elastin production. Based on these results, hCOL1A1-CED may be explored further for its potential use as a preventative agent against skin aging.