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Myofibrillar myopathies (MFMs) are a genetically or clinically heterogeneous group of diseases that are characterized by focal myofibrillar dissolution associated with accumulation of myofibrillar degradation products and ectopic expression of multiple proteins. Since MFMs show morphologically distinct features but consist of genetically and clinically heterogeneous diseases, muscle biopsy is important for the diagnosis. A 20-year-old man complained of progressive weakness and atrophy of both legs for two years. He had a dysmorphic face and short stature. The light microscopic examination of his muscle biopsy showed mixed myopathic and neurogenic changes. Many myofibers with multiple clusters of blue red rod-like structures and cytoplasmic inclusions were noted. Immunohistochemistry showed a focal positive reaction in sarcoplasm to desmin and myotilin antibodies. An electron microscope study revealed variable abnormalities of myofibrillar structures. To the best of our knowledge, this is the first reported case of MFM with pathology findings in Korea.
The effects of radiation and Cis dichlorodiammineplatinum(Ⅱ) (Cis-DDP) were assessed in rectum of rats by histopathological changes. Rats were exposed to single doses of X ray(6~10 Gy) without or with Cis-DDP(2.5㎎/㎏). In combined group, Cis-DDP was given 30 minntes before or immediately after irradiation. Cis-DDP alone showed inflammatory cell infiltration and increased goblet cells in the mucosa and edema and fibrosis of submucosa with vascular sclerosis. With increased radiation dosage. such changes were aggravated. Necrosis of muscle layer developed 8 Gy irradiation. In groups with combination treatment of X-ray and Cis-DDP. changes of mucosa and submucosa were not significantly different from radiation alone group, but necrosis of the muscle layer was developed in 6 Gy combination group and degree was more severe in 8 and 10 Gy combination group compared to radiation alone group. There was no difference according to the timing of Cis DDP administration before or after irradiation. This result suggests Cis DDP enhance the radiation effect on the rectum of rats and enhancement ratio was 1.3 as the endpoint was necrosis of the muscle layer.
Profiles of 7-kDa Stress Protein, βAPP, Proliferating Cell Nuclear Antigen, and Glial Fibrillary Acidic Protein in the Gerbil with Focal Infarction and Combined Focal Infarction and Global Ischemia : Relation with Ischemic Tolerance
목적 : 본 연구는 성숙 gerbil 뇌에서 대퇴정맥을 통한 자성체 주입과 두개골에 부착된 자석을 이용하여 유발한 국소 경색에서 70-kDa stress protein(hsp70), βAPP, proliferating cell nuclear antigen(PCNA), glial fibrillary acidic protein(GFAP)의 발현을 관찰하여 경색의 시간에 따른 조직 변화와의 관계를 보고 또한 이런 단백이 반복 허혈손상에 의한 보호 작용과 관련이 있는지를 보기 위하여 시행하였다. 방법 : 국소 경색이 6시간, 1일, 2일, 3일, 7일 병변은 성숙 gerbil에서 측두부의 두개골 표면에 자석을 접착고정하고 산화철 입자의 생리식염수 현탁액을 대퇴정맥에 주입하여 유발한 구소 경색에서 관찰하였고, 8일, 10일, 14일 병변은 국소 허혈을 일으킨 후 1일, 3일, 7일이 지난 다음에 양측 경동맥을 동시에 결합하여 5분간 혈류를 차단시킨 다음 7일 후에 검사를 시행한 gerbil에서 관찰하였다. H-E염색에 의한 조직 소견과 면역조직화학 염색법에 의한 각 단백의 발현을 평가하였다. 결과 : 국소 허혈을 일으킨 후 1일에 병변의 중심부에 있는 괴사된 신경세포와 경계영역에 있는 변성되는 신경세포는 hsp70과 βAPP에 중등도의 양성 반응을 보이기 시작했다. 경색의 중심부에서 2일에 나타나기 시작하여 3일에서 8일까지 많은 수가 관찰된 hsp70과 βAPP에 강한 양성 반응을 보이는 탐식세포는 10일에는 수가 감소하였다. PCNA 양성인 세포는 6기간에 보이기 시작하여 경색의 주변부에서 1~3일에 많이 증가하였고 탐식세포와 별세포도 양성 반응을 보였다. GFAP 양성인 별세포는 1일에 경색의 주변에서 보였고 시간이 지날수록 점점 증가하였다. 결론 : 국소 경색 병변에서 hsp70과 βAPP, GFAP가 1일부터 7일까지 지속적으로 증가된 소견은 같은 연구방법에 의해 시행한 이전의 실험에서 1일과 3일에 관찰되었던 반복 허혈손상에 의한 보호 작용의 시간에 따른 변화와 일치하지 않으므로 이런 보호 작용에 다른 요소가 함께 관여하는 것을 알 수 있었다.
Kimura’s disease is a rare chronic inflammatory disorder mimicking a malignant tumor. It usually occurs in the head and neck regions, but occasionally shows up in the groin. A gray-scale groin sonograph showed a lobulated hypoechoic mass. Color Doppler sonography revealed the paucity of blood vessels within the mass.
24 hour's urines were collected from the 66 women medical students and vari?? components were analyzed and results were as follows; total volume 1,269.7ml, pecific gravity 1.0195, pH6.3, titratable acidity 223, Creatinine 1.48 gm, Urinary Nitrogen 12.7gm, Uric acid 0.59gm, Chloride(as Nacl)14.5gm, Sodium 6.62gm, Potassium 1.89gm, Calcium 0.18gm, Phosphorus 272mg. The results were compared with those of others.
Background : This study was done to see if there were correlations between anatomic and molecular parameters such as microvessel density (MVD), lymphatic vessel density (LVD), and vascular endothelial growth factor receptor (VEGFR)-3 expression and various clinical parameters for papillary thyroid carcinomas of size > 1.0 cm (PTCs) and size ≤ 1.0 cm (papillary thyroid microcarcinomas, PTMCs). PTMCs were divided into two subgroups (0-5 mm and 6-10 mm). Methods : We analyzed 197 thyroid carcinomas including 113 PTCs and 84 PTMCs. Tissue samples form 30 patients from each group matched for clinical characteristics were selected for immunostaining. Results : Although PTCs and PTMCs showed significant differences in clinical characteristics, they did not show significant difference in MVD, LVD, or VEGFR-3 expression. There was a significantly higher LVD in the PTMC subgroup with the larger tumors but no difference in clinical characteristics. LVD was higher in patients > 45 years old (more apparent in the PTC group) and LVD had suggestive correlations with multicentricity and extrathyroidal extension depending on analytic conditions. Conclusions : Since LVD showed variable correlations with clinical variables for papillary carcinoma of the thyroid depending on analytic conditions, the individually planned treatments based on overall clinicopathological factors are advised.
Background : This study was done to obtain comprehensive data on changes in the structural components of the enteric nervous system in pediatric patients with intestinal pseudo-obstruction (IPO). We evaluated routinely processed, in formalin-fixed tissues by quantitative morphometric analysis. In addition, we used formalin-fixed tissue to explore the possibility of using previously proposed diagnostic criteria to evaluate frozen serial sections for intestinal neuronal dysplasia (IND) type B and hypoganglionosis. Methods : We analyzed data for 19 IPO cases. Morphometric analysis for quantification of ganglia and ganglion cells (GCs) was done for the myentric and the submucous plexus. In addition, we determined the presence of immature GCs and the distribution of nerve fibers and interstitial cells of Cajal (ICC). Results : Nine patients showed combined hypoganglionosis, IND, and decreased ICC; others showed various combinations of these. Several morphometric factors were significantly different between patient groups as well as being different than the control group. Conclusions : Our pediatric IPO cases showed extensive overlapping of pathological findings. And the findings suggest the utility of using previously proposed morphometrically measured factors in multiple frozen sections as diagnostic criteria for IND type B and hypoganglionosis in formalin-fixed tissue.