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Background: The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is a quantitative measure of ALS-related physical deficit with established reliability and validity in prospective clinical research. This study aimed to test the reliability and validity of the Korean version of ALSFRS-R (K-ALSFRS-R). Methods: The subjects of the study were 51 patients with ALS diagnosed on the basis of El Escorial criteria. The patients were evaluated by one rater using K-ALSFRS-R, Norris scale, and Appel ALS rating scale on the same day. Subsequently, K-ALSFRS-R was measured by two other raters using a videotape design. Test-retest data were obtained within the time interval of 7 days. Results: Internal consistency and test-retest reliability were high. Inter-rater comparisons showed significant reliability with Kappa or Kendall’s tau-b value. Moreover, K-ALSFRS-R scores correlated significantly with clinical status as measured by Norris and Appel ALS rating scale. Conclusions: Our results showed that K-ALSFRS-R would be a reliable and useful instrument for the evaluation of functional status in patients with ALS.
This article attempts to understand the characteristics of the management of historical records and documents in the Joseon Period, compared with that of the Edo Period in Japan. Sillok(實錄), Seungjeongwon-ilki(承政院日記), Bibyonsa-deungnok(備邊司謄錄), and various Eugwe(儀軌) show the essence of the historical record and the document culture of the Joseon Period. We can suggest Dokugawa-zikki(德川實紀), Yuhitssho-ikki(右筆所日記), Soshabanikki(奏者番日記), and Soshaban-tedome(奏者番手留) as the records and documents of the Edo Period corresponding with those of the Joseon Period. There are some similarities between Joseon’s system of record and document management and that of the Edo Period, from the point of view that every office had its own system of recording and documenting. However, there was a difference in the system to make Sillok the highest level of records and documents by reporting and abridging from the records and documents that had been produced by every office, the original records and documents being discarded as hyuji (or disused papers for recycling) in the Joseon Period. In the Edo Period, by contrast, no record or document was ever discarded, except for special cases, but was preserved permanently, in other words, a permanent preservation system of records and documents. This point is the most important difference between two countries in their management of historical records and documents.
Red blood cell (RBC) alloimmunization varies across human populations and ethnic groups. We evaluated the characteristics of RBC alloimmunization and compared the risk of alloimmunization in Korean patients with myelodysplastic syndrome (MDS) and liver cirrhosis (LC), two representative diseases in which chronic transfusion is required. In total, 115 MDS patients and 202 LC patients transfused with RBCs between 2013 and 2015 were retrospectively included. Twenty patients (6.3%) were newly alloimmunized (five MDS patients, 4.3%; 15 LC patients, 7.4%). The median number of RBC units transfused in alloimmunized patients was nine (interquartile range, 4–15 units). As the number of transfused RBC units increased, the cumulative risk of alloimmunization was higher in LC than in MDS patients (P=0.001). The most common alloantibody detected in patients was anti-E (45%), followed by anti-c (17%), anti-e (10%), anti-C (7%), anti-Fyb (7%), and anti-Jka (7%). The present data indicate the need for matching of extended RBC antigens (Rh, Duffy, and Kidd systems) for chronically transfused patients with MDS and LC in Korea.
Chronic granulomatous disease (CGD) is a rare genetic disease, which is caused by defects in the NADPH oxidase complex (gp91phox, p22phox, p40phox, p47phox, and p67phox) of phagocytes. This defect results in impaired production of superoxide anions and other reactive oxygen species (ROS), which are necessary for killing bacterial and fungal microorganisms and leads to recurrent, life-threatening bacterial and fungal infections and granulomatous inflammation. The dihydrorhodamine (DHR) flow cytometry assay is a useful diagnostic tool for CGD that can detect absent or reduced NADPH oxidase activity in stimulated phagocytes. We report a patient with X-linked CGD carrying a novel mutation of the CYBB gene whose chimerism status following hematopoietic stem cell transplantation (HSCT) has been rapidly determined using the DHR assay. The level of DHR activity correlates well with short tandem repeat PCR analysis. Considering the advantages of this simple,rapid, and cost-effective procedure, serial measurement of DHR assay would facilitate the rapid determination of a patient’s engraftment status, as a supplementary monitoring tool of chimerism status following HSCT.
부당해고에 대한 형사처벌 조항인 「구 근로기준법」 제110조는 헌법재판소가 합헌결정을 하였음에도 이후 시행 4년째를 맞는 「근로기준법」 개정 법률을 비판적으로 검토할 수 있다는 점에서 동 조항의 위헌성 여부에 대하여 논의할 실익이 있다. 헌법재판소가 ‘준법정신을 가진 평균인’을 기준으로 명확성을 판단한 것은 적절하고, 무엇보다 이 사건 법률조항의 ‘정당한 이유’에 대하여는 오랜 기간 다수의 행정해석과 관련 판례, 학문적 연구가 집적되어 그 의미가 구체적으로 형성되어 있으므로, 사용자는 이를 바탕으로 해고에 관하여 자신의 행위를 결정해 나갈 수 있고, 집행자의 자의가 배제될 수 있다. 과잉금지원칙 위배 여부에 대하여 살펴보면, 부당해고에 대한 형사처벌 조항이 예방적 기능을 수행하며, 개정 법률이 도입한 이행강제금 등 민사법적 제재만으로는 해고예방의 목적을 달성하기 어렵다는 것을 보여준다. 따라서 부당해고에 대한 형사처벌 조항은 적합성원칙과 필요성원칙을 충족한다. 해고의 태양은 사회변동에 따라 변화하므로, 일반적 처벌규정을 통하여 가벌성이 크지만 개별적 처벌규정으로 규율되지 않는 새로운 부당해고행위 형태로부터 근로자를 보호할 필요가 있다. 또한, 법원은 「구 근로기준법」 제110조를 엄격하게 적용하고 있으므로 균형성원칙에도 반하지 않는다. 부당해고에 대한 형사처벌 조항이 헌법에 위반되지 아니한다는 헌법재판소의 결정이 타당함에도 「근로기준법」 개정 법률에서 이를 삭제한 것은, 이 사건 법률조항이 목적으로 하는 부당해고 예방과 이를 통한 근로자의 생존권 보호가 문제에 노출될 수 있음을 암시한다. An article 110 of old Labor Standards Act, the criminal punishment clause on unfair dismissal, was erased by amendment of the Act itself despite that Constitutional Court had decided it was Constitutional. It’s a proper decision regarding Constitutional Court’s ruling that Article 110 of old Labor Standards Act does not violate Constitution because it does not violate the principle of clarity and the principle of prohibition against excessive restrictions. Nevertheless, deletion of Article in the Amended Labor Standards Act suggests that prevention of unfair dismissal and the protection of the right to live of workers through it be exposed to problem. The fact that after the enforcement of Amended Labor Standards Act, the number of report of judgement cases like unfair dismissal at Seoul Labor Relations Commission increased 73.8% dramatically could be an empirical evidence. Restricting dismissal that menace the right to live of workers is the requirement of Constitution which guarantees human dignity and the right to labor. Also it is the task of whole society. Now that has it been 4th year since the enforcement of revised Labor Standards Act, we need to dwell on again the Constitutional Court’s decision whether criminal penalty Article regarding unfair dismissal does violate Constitution.