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Late Onset Glutaric Acidemia Type II Manifested as Afebrile Seizure (경련 발작으로 발현된 지발형 제II형 글루타르산혈증)

  • Nam, Sang Jeong;Lee, Gun Joon;Park, Won Il;Bae, Eun Joo;Lee, Kyung Hwa;Lee, Hong Jin
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.5 no.1
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    • pp.1-8
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    • 2005
  • Glutaric acidemia (GA) type II is a very rare inherited disorder that have no accruate figure on its icidende. People with Glutaric acidemia type II have an enzyme that does not work properly. Two specific enzymes are associated with Glutaric acidemia type II:1. Electron transfer flavoprotein (ETF), 2. ETF-ubiquinone oxidoreductase (ETF-QO). Both of these enzymes have similar functions in the body, and children with Glutaric acidemia type II may lack one or the other of these enzymes. They play an important role in breaking down fats and proteins, and help the body to produce energy. GA II clinically manifested as (1) neonatal onset with congenital anomalies (2) neonatal onset without anomalies, and (3) mild and/or later onset. The first two groups are sometimes said to have multiple acyl CoA dehydrogenation deficiency-severe and the third to have multiple acyl CoA dehydrogenation deficiency-mild. The course and age at presentation of later-onset glutaric acidemia type II is extremely variable, therefore it is difficult to diagnosis. We experienced one case of late onset form glutaric acidemia type II with afebrile status epilepticus-like convulsion.

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Three-Dimensional Approaches in Histopathological Tissue Clearing System (조직투명화 기술을 통한 3차원적 접근)

  • Lee, Tae Bok;Lee, Jaewang;Jun, Jin Hyun
    • Korean Journal of Clinical Laboratory Science
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    • v.52 no.1
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    • pp.1-17
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    • 2020
  • Three-dimensional microscopic approaches in histopathology display multiplex properties that present puzzling questions for specimens as related to their comprehensive volumetric information. This information includes spatial distribution of molecules, three-dimensional co-localization, structural formation and whole data set that cannot be determined by two-dimensional section slides due to the inevitable loss of spatial information. Advancement of optical instruments such as two-photon microscopy and high performance objectives with motorized correction collars have narrowed the gap between optical theories and the actual reality of deep tissue imaging. However, the benefits gained by a prolonged working distance, two-photon laser and optimized beam alignment are inevitably diminished because of the light scattering phenomenon that is deeply related to the refractive index mismatch between each cellular component and the surrounding medium. From the first approaches with simple crude refractive index matching techniques to the recent cutting-edge integrated tissue clearing methods, an achievement of transparency without morphological denaturation and eradication of natural and fixation-induced nonspecific autofluorescence out of real signal are key factors to determine the perfection of tissue clearing and the immunofluorescent staining for high contrast images. When performing integrated laboratory workflow of tissue for processing frozen and formalin-fixed tissues, clear lipid-exchanged acrylamide-hybridized rigid imaging/immunostaining/in situ hybridization-compatible tissue hydrogel (CLARITY), an equipment-based tissue clearing method, is compatible with routine procedures in a histopathology laboratory.

Antibiotic Resistance Patterns of Enterococcus spp. Isolated From Commercial Frozen Foods (시판 냉동식품에서 분리한 장구균의 항생제 내성 양상)

  • Park, Sun-Hee;Kim, Kyung-Sig;Yoo, Young-A;Lee, Jae-Kyoo;Jung, Sung-Kook;Han, Ki-Young;Kim, Moo-Sang
    • Journal of Food Hygiene and Safety
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    • v.25 no.2
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    • pp.122-128
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    • 2010
  • From April to December in 2009, microbial investigation is accomplished for 100 frozen foods asked to microbial control team that corresponds with total aerobic viable bacteria, coliform group, Escherichia coli, Enterococcus spp. and antibiotic resistance patterns of Enterococcus spp. isolates are investigated. Average of total erobic viable bacteria numbers is $4.3{\times}10^4CFU/g$. Average of coliform group numbers is $4.3{\times}10^3CFU/g$. Average f Enterococcus spp. numbers is $1.8{\times}10^3CFU/g$. Escherichia coli from 100 frozen foods is not detected and detection ate is 0.0%. 22 Enterococcus spp. are isolated from 100 frozen foods. 12 of 22 Enterococcus spp. strains are identified as E. faecium. 7 of 22 Enterococcus spp. strains are identified as E. faecalis. 2 of 22 Enterococcus spp. trains are identified as E. gallinarum. 1 of 22 Enterococcus spp. strains is identified as E. hirae. Enterococcus spp. solates show a high resistance to erythromycin, rifampin, tetracycline, ciprofloxacin, chlorampenicol, penicillin and susceptibility to vancomycin, ampicillin, gentamicin, strepomycin, linezolid. 15 of 22 Enterococcus spp. strains are multi-resistant and the most frequent multi-resistant pattern is erythromycin-rifampin for 6 Enterococcus spp. strains.

The Content and Risk Assessment of Heavy Metals in Herbal Medicines used for Food and Drug (식약공용 농·임산물의 유해중금속 분석 및 위해도 평가)

  • Cho, In-Soon;Kim, Su-Jin;Park, Ae-Suk;Kim, Jin-Ah;Jang, Jung-Im;Lee, Sung-Deuk;Yu, In-Sil;Shin, Yong-Seung
    • Journal of Food Hygiene and Safety
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    • v.35 no.4
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    • pp.354-364
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    • 2020
  • For this paper we analyzed heavy metals including lead, cadmium, arsenic and mercury, and conducted risk assessment on 171 types of herbal medicines used as foods and drugs distributed in Seoul's Yangnyeongsi and Kyungdong (Gyeongdong) herbal medicine markets. The concentrations of the heavy metals were measured by the ICP-MS and a mercury analyzer. The detection ranges of the lead, cadmium, arsenic and mercury were found to be ND-4.719 mg/kg, ND-1.019 mg/kg, 0.002-8.696 mg/kg and ND-0.058 mg/kg, respectively. Artemisiae Capillaris Herba exceeded the standards of the MFDS (Ministry of Food and Drug Safety) for cadmium items, and Actinidiae Ramulus et Fulium et Fructus Vermicultus exceeded the standards for arsenic items. The non-carcinogenic risk assessment showed that there were no items exceeding 1 and that they were safe. The risk of carcinogenicity to lead was below 10-6 in all items and was evaluated as safe. The risk of carcinogenicity of arsenic was almost all in the range of 10-4 to 10-6. All %PTWI are considered to be safe below 100.

A Case Report of Three Patients with Nontuberculous Mycobacterial Pulmonary Disease Caused by Mycobacterium kansasii (Mycobacterium kansasii에 의한 비결핵성 마이코박테리아 폐질환 3례)

  • Koh, Won Jung;Kwon, O Jung;Suh, Gee Young;Chung, Man Pyo;Kim, Hojoong;Lee, Nam Yong;Kim, Tae Sung;Lee, Kyung Soo;Park, Eun Mi;Park, Young Kil;Bai, Gill Han
    • Tuberculosis and Respiratory Diseases
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    • v.54 no.4
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    • pp.459-466
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    • 2003
  • Mycobacterium kansasii is the second most common cause of nontuberculous mycobacterial pulmonary disease in Western countries and Japan. The clinical and radiological features of pulmonary disease caused by M. kansasii usually resemble those of pulmonary tuberculosis including cavitary infiltrates with an upper lobe predilection. It is also now apparent that patients with M. kansasii pulmonary disease can present with noncavitary nodular bronchiectatic infiltrates similar to lung diseases of M. avium complex. With rifampin-containing regimens, treatment success rates are almost 100%. Timely diagnosis before the development of extensive disease and effective overall treatment strategies are very important to ensure that patients receive the appropriate medications for a sufficiently long period of time. To our knowledge, there has been no Korean case report of M. kansasii pulmonary disease in the immunocompetent patient until now. We report three cases of M. kansasii pulmonary disease in immunocompetent adult patients.

Differentiation of Korean isolates of Entamoeba histolytica from Entamoeba dispar (우리 나라에서 분리한 이질아메바(Entamoeba histoItica)와 동형아메바(Entamoebn dispar)의 감별)

  • 최성준;이미정
    • Parasites, Hosts and Diseases
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    • v.34 no.1
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    • pp.15-20
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    • 1996
  • Cysts of Entamoebn histoIMtica are still found from humans in Korea, but notall of the cysts are known as pathogenic. The non-pathogenic strain is regarded as a differenL species, E. nispnr. In this study, Korean isolates of conventional E. histolvticn were subjected for the differentiation by polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) analysis. Human stools were screened by routine microscopic examination, and cyst or trophozoite positive stools were inoculated into Robinson media. The cultivated trophozoites were prepared for DNA extraction, and the DNAs were used for PCR with common primers of Pl gene. The PCR products were divested with 3 restriction enzymes and RFLP was observed. Also anti-sense primers containing the cleavage site of each restriction eWe were designed for differentiation only by PCR. The PCR products of Korean isolates 59,512, YS-6, and YS-27 were spliced by Taq I and Xmnl but not byAccl, and the isolates S1, S3, S11, S15, S16, S17, S20, YS- l7, and YS-44 were spliced by Acc I but not by Taq I and Xmn I. These RFLP pattern correlated well with PCR products by the species specific primers. The findings confirm that the Korean isolates 59,512, YS-6, and YS-27 are E. histolwtico and others are E. dispar. In Korea, most of the asymptomatic cyst carriers are infected by E. dispar, not by E. histolytica. Key words: Entcmoebc histolytica, Entcmoebn dispar Korean isolates, polymerase chain reaction (PCR), restriction fragment length polymorphism (RFLP)

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Implementation of an Efficient Microbial Medical Image Retrieval System Applying Knowledge Databases (지식 데이타베이스를 적용한 효율적인 세균 의료영상 검색 시스템의 구현)

  • Shin Yong Won;Koo Bong Oh
    • Journal of the Korea Society of Computer and Information
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    • v.10 no.1 s.33
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    • pp.93-100
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    • 2005
  • This study is to desist and implement an efficient microbial medical image retrieval system based on knowledge and content of them which can make use of more accurate decision on colony as doll as efficient education for new techicians. For this. re first address overall inference to set up flexible search path using rule-base in order U redure time required original microbial identification by searching the fastest path of microbial identification phase based on heuristics knowledge. Next, we propose a color ffature gfraction mtU, which is able to extract color feature vectors of visual contents from a inn microbial image based on especially bacteria image using HSV color model. In addition, for better retrieval performance based on large microbial databases, we present an integrated indexing technique that combines with B+-tree for indexing simple attributes, inverted file structure for text medical keywords list, and scan-based filtering method for high dimensional color feature vectors. Finally. the implemented system shows the possibility to manage and retrieve the complex microbial images using knowledge and visual contents itself effectively. We expect to decrease rapidly Loaming time for elementary technicians by tell organizing knowledge of clinical fields through proposed system.

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Analytical Methods on the Determination of Active Ingredients for Hair Nourisher Products (HPLC를 이용한 양모제 유효성분의 동시분석법)

  • Kwon, Kyoung-Jin;Kim, Do-Jung;Kim, Sang-Seop;Choi, Yu-Bin;Kim, Mi-Jeong;Choi, Myeong-Sin;Cho, Bo-Kyung;Kim, Dong-Sup
    • Journal of the Society of Cosmetic Scientists of Korea
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    • v.36 no.3
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    • pp.167-174
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    • 2010
  • The hair nourisher products are used for prevention of hair loss and classified as quasi-drug in Korea. As concerns about hair loss has been increased, the demand for hair nourisher products has been growing. It is difficult to analyze their main ingredients because they contain various ingredients including natural plant extracts, vitamins, preservatives and exfoliators. The purpose of this study was to develop and validate simultaneous analytical methods of active ingredients in hair nourisher products such as nicotinamide, tocopherol, salicylic acid, dexpanthenol and benzyl nicotinate by HPLC. The active ingredients were separated on a $C_{18}$ column by using acetonitrile/phosphate buffer as a mobile phase, and detected at UV 220, 270 and 300 nm. The calibration curve showed linearity in the range of $12.5{\sim}800\;{\mu}g/mL$ and the recoveries were 97.3 ~ 103.5 % (RSD 0.9 ~ 2.8 %) in liquid matrix and 101.9 ~ 115.9 % (RSD 0.7 ~ 7.7 %) in shampoo matrix. Validated method was applied to hair nourisher products obtained from distribution market. Fortunately, all samples met their criteria. This study might be expected to provide the method for determining active ingredients in hair nourisher products and lead to promote a rapid market entry.

Synthesis and Characterization of Porous Poly(ε-caprolactone)/Silica Nanocomposites (다공성 폴리(ε-카프로락톤)/실리카 복합체의 제조 및 특성평가)

  • Son, Siwon;Choi, Ji-Eun;Cho, Hun;Kang, DaeJun;Lee, Deuk Yong;Kim, Jin-Tae;Jang, Ju-Woong
    • Polymer(Korea)
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    • v.39 no.2
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    • pp.323-328
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    • 2015
  • Poly(${\varepsilon}$-caprolactone) (PCL) nanofibers and PCL/silica membranes were synthesized by sol-gel derived electrospinning and casting, respectively. Smooth PCL nanofibers were obtained from the precursor containing N,N-dimethylformamide (DMF). PCL/silica membranes were prepared by varying the tetraethyl orthosilicate (TEOS) contents from 0 to 40 vol% to investigate the effect of silica addition on mechanical properties and cytotoxicity of the membranes. Although the strength of the membranes decreased from 12 to 8 MPa with increasing the silica content, the strength remained almost constant 7 weeks after dipping in phosphate buffered saline solution (PBS). The strength reduction was attributed to the presence of a patterned surface pores and micro-pores present in the walls between pores. The crystal structure of the membranes was orthorhombic and the crystallite size decreased from 57 to 18 nm with increasing the silica content. From the agar overlay test, the PCL/silica membranes exhibited neither deformation and discoloration nor lysis of L-929 fibroblast cells.

Diagnostic effectiveness of fine needle aspiration cytology on pediatric cervical lymphadenopathy (소아 경부 림프절 종대의 세침 흡인 세포검사의 진단적 유용성)

  • Byun, Jun Chul;Choe, Byung Kyu;Hwang, Jin-Bok;Kim, Heung Sik;Lee, Sang Sook
    • Clinical and Experimental Pediatrics
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    • v.49 no.2
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    • pp.162-166
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    • 2006
  • Purpose : There are few reports in Korean literature on the diagnostic efficacy of fine needle aspiration cytology(FNAC) for pediatric cervical lymphadenopathy. This study examined the diagnostic value of FNAC on cervical lymphadenopathy in children. Methods : Data from 57 pediatric patients(aged 0.3 to 14 years) who underwent FNAC due to cervical lymphadenopathy between January 2001 and March 2005 was reviewed retrospectively. Results : Reactive cervical lymphadenitis was the most common result of the FNAC(52.5 percent). Malignant disease were revealed in 14 percent of all cases. The sensitivity, specificity was 86 percent and 96 percent, respectively. The positive and negative predictive values for malignant disease was 75 percent and 98 percent, respectively. There were two false-positive cases on FNAC; one case of Langerhans cell histiocytosis, and another with infectious mononucleosis. A false-negative case on FNAC was found to be acute lymphocytic leukemia. In seven cases(12 percent), the final diagnosis was confirmed by an open biopsy after the FNAC. There were four FNAC cases where the specimen was not satisfactory for making a diagnosis(7 percent). There were no serious complications of the FNAC procedure. Conclusion : FNAC had a high diagnostic efficacy for evaluating children with cervical lymphadenopathy.