• Title/Summary/Keyword: 지방증

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Parosteal Lipoma of the Femur with Hyperostosis: A Case Report (대퇴골의 과골증을 동반한 방골성 지방종: 증례 보고)

  • Kim, Do-Yeon;Jeon, Ho-Seung;Jeon, Seung-Ju;Noh, Haeng-Kee;Kang, Seo-Goo;Song, Ji-Ung;Park, Byeong-Moon
    • The Journal of the Korean bone and joint tumor society
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    • v.18 no.2
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    • pp.104-108
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    • 2012
  • Parosteal lipoma is a rare kind of lipoma that occurring adipous tissue around the periosteum. It has been reported most commonly in the femur, the radius, the tibia and the fibular. Treatment consists of resecting the lipomatous tumor with further exeresis of the bone and periosteal excrescence in cases with hyperostosis. The authors report a rare case of parosteal lipoma occurring at the medial portion of the femur shaft with a review of the relevant literatures.

Characterization of a Mucolipidosis Type II Mouse Model and Therapeutic Implication of Lysosomal Enzyme Enriched Fraction Derived from Placenta (뮤코지방증 2형 마우스 모델의 특징과 태반에서 추출한 리소좀 효소 투여의 결과)

  • Cho, Sung Yoon;Kim, Ki-Yong;Kim, Su Jin;Sohn, Young Bae;Maeng, Se Hyun;Kim, Chi Hwa;Ko, Ah-Ra;Song, Junghan;Yeau, Sung-Hee;Kim, Kyung-Hyo;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.5-13
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    • 2012
  • I-cell disease (mucolipidosis type II; MIM 252500) and pseudo-Hurler polydystrophy (mucolipidosis type III; MIM 252600) are disorders caused by abnormal lysosomal transport in cells. The presence of numerous inclusion bodies in the cytoplasm of fibroblasts, a lack of mucopolysacchariduria, increased lysosomal enzyme activity in serum, and decreased GlcNAc-phosphotransferase activity are hallmark. Here, we attempted to investigate phenotypical and biochemical characteristics of the knockoutmouse of GlcNAc-phosphotransferase ${\alpha}/{\beta}$ subunits; in addition, we also attempted to determine whether the lysosome enriched fraction derived from placenta can be beneficial to phenotype and biochemistry of the knockout mouse.We found that the knockout mouse failed to thrive and had low bone density, as is the case in human. In addition, skin fibroblasts from the animal had the same biochemical characteristics, including increased lysosomal enzyme activity in the culture media, in contrast to the relatively low enzyme activity within the cells. Intravenous injection of the lysosome rich fraction derived from placenta into the tail vein of the animal resulted in a gain of weight, while saline injected animals didn't.In conclusion, our study demonstrated the phenotypical and biochemical similarities of the knockout mouse to a mucolipidosis type II patient and showed the therapeutic potential of the lysosome enriched fraction. We admit that a larger scale animal study will be needed; however, the disease model and the therapeutic potential of the lysosome enriched fraction will highlight the hope for a novel treatment approach to mucopolipidosis type II, for which no therapeutic modality is available.

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RI동정

  • Korea Radioisotope Association
    • 동위원소뉴스
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    • no.4 s.64
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    • pp.5-8
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    • 2002
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The Lipid Accumulation Suppressive Effect of Monostroma nitidum In kidney of Murine with hyperlipidemia induced by Triton WR-1339 (Triton WR-1339 주사에 의한 고지혈증 유발 생쥐 신장에 미치는 참홑파래 추출물의 지방축적 억제 영향)

  • Park, In-Sick;Ahn, Sang-Hyun;Choi, Nan-Hee;Kim, Jin-Tack
    • The Journal of Dong Guk Oriental Medicine
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    • v.6 no.2
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    • pp.87-98
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    • 1998
  • Renal tissues of ICR mouse were intraperitoneally injeced with Monostroma nitidum extract(ME) after Triton WR-1339(TX) injection were observed to investigate the lipid accumulation suppressive effect of ME. The renal tissues were obtained at hour 24, 48, and 72 after TX injection that were fixed in fromol-calcium solution and were cryocut. The tissue stained by H&E for general morphology, sudan black B for lipid and perchloric acid-naphthoquinone method for cholesterol. The parietal layer of Bowman's capsule is swatted as cuboidal type at hour 48 after TX injection and the lipid blot and cholesterol particle were noticeably increased in glomerulus than control group and these appeared in convoluted tubule, collecting tubule, and Helen's loop. But the parietal layer of Bowman's capsule was shaped as squamous type that was looked like as normal morphology in ME treated group after TX injection and the lipid blot and cholesterol particle were considerably decreased in glomerulus than TX group. As results indicated that the accumulation of lipid including cholesterol caused by TX injection were mitigated in renal tissues by the antihyperlipidermic effect of ME.

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GLCM Algorithm Image Analysis of Nonalcoholic Fatty Liver and Focal Fat Sparing Zone in the Ultrasonography (초음파검사에서 비알콜성 지방간과 국소지방회피영역에 대한 GLCM Algorithm 영상분석)

  • Cho, Jin-Young;Ye, Soo-Young
    • Journal of radiological science and technology
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    • v.40 no.2
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    • pp.205-211
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    • 2017
  • There is a need for aggressive diagnosis and treatment in middle-aged and high-risk individuals who are more likely to progress from nonalcoholic fatty liver to hepatitis. In this study, nonalcoholic fatty liver was divided into severe, moderate, and severe, and classified by quantitative method using computer analysis of GLCM algorithm. The purpose of this study was to evaluate the characteristics of ultrasound images in the local fat avoidance region. Normal, mild, moderate, severe fatty liver, and focal fat sparing area, 80 cases, respectively. Among the parameters of the GLCM algorithm, the values of the Autocorrelation, Square of the deviation, Sum of averages and Sum of variances with high recognition rate of the liver ultrasound image were calculated. The average recognition rate of the GLCM algorithm was 97.5%. The result of local fat avoidance image analysis showed the most similar value to the normal parenchyma. Ultrasonography can be easily accessed by primary screening, but there may be differences in the accuracy of the test method or the correspondence of results depending on proficiency. GLCM algorithm was applied to quantitatively classify the degree of fatty liver. Local fat avoidance region was similar to normal parenchyma, so it could be predicted to be homogeneous liver parenchyma without fat deposition. We believe that GLCM computer image analysis will provide important information for differentiating not only fatty liver but also other lesions.

Nonalcoholic Fatty Liver Disease Progressing to Cirrhosis in an Obese Boy with Hypopituitarism (청소년기에 간경화증으로 진행된 비알콜성 지방간질환 1예)

  • Park, Ji-Yong;Ko, Jae-Sung;Seo, Jeong-Kee;Lee, Ran;Shin, Choong-Ho;Kang, Gyeong-Hoon;Kim, Woo-Sun
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.204-209
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    • 2008
  • Non-alcoholic fatty liver disease (NAFLD) is typically associated with obesity and insulin resistance. Non-alcoholic steatohepatitis (NASH) is a more serious form of NAFLD. Although fibrosis is common in pediatric NASH, cirrhosis has been rarely reported. Patients with hypothalamic or pituitary dysfunction are at risk for obesity and insulin resistance with subsequent development of NAFLD. We report a case of NAFLD progressing to cirrhosis in an obese 16 year-old boy with hypopituitarism.

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Three Cases of Mucocutaneous Angiomyolipoma in the Head and Neck Region with Reference to the HMB-45 and Melan-A Immunohistochemistry (두경부에서 발생한 점막피부 혈관근육지방종 3예 : HMB-45와 Melan-A에 대한 면역화학검사 특징)

  • Kim, Na-Rae;Kim, Dong-Young;Cha, Heung-Eog;Ha, Seung-Yeon
    • Korean Journal of Head & Neck Oncology
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    • v.25 no.2
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    • pp.150-152
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    • 2009
  • 혈관근육지방종은 지방, 평활근, 두꺼운 벽의 혈관이 다양한 비율로 구성된 흔하지 않은 과오종이며, 이는 결정 경화증을 동반하거나 산발적으로 발생한다. 저자들은 혈관근육지방종이 드물게 발생하는 두경부에서 발생한 귓바퀴 1예, 구개점막 2예의 혈관근육지방종을 보고하고자 한다. 병리소견상, 3예 모두에서 성숙지방조직, 불규칙한 혈관, 그리고 HMB-45와 Melan-A에 음성을 보이는 평활근육세포로 이루어진 조직소견을 보였으며, 여러군데에서 림프구 침윤이 3예 모두에서 관찰되었다. 점막피부 혈관근육지방종으로 진단하였다. 세증례 모두 결절경화증은 동반되지 않았다. 점막피부 혈관근육지방종에서는 혈관주변세포가 HMB-45와 Melan-A에서 음성을 보였으며, 이는 간이나 신장의 혈관근육지방종에서의 특징적인 양성반응과는 다른 점이었다. 간이나 신장에서 생긴 혈관근육지방종과 다른 임상병리적 특징을 비교 기술하고자 두경부에서 발생한 점막피부 혈관근육지방종 3예를 보고한다.