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      • 경한 임상 경과를 보인 신생아 시기의 프로피온산혈증 1례

        김경란,김진섭,허림,박형두,조성윤,진동규,Kim, Kyung-Ran,Kim, Jinsup,Huh, Rim,Park, Hyung-Doo,Cho, Sung Yoon,Jin, Dong-Kyu 대한유전성대사질환학회 2016 대한유전성대사질환학회지 Vol.16 No.1

        프로피온산혈증은 상염색체 열성유전에 의해 발생하는 질환으로, 혈중에 암모니아, 독성 물질 등이 축적되어 경한 증상에서부터 사망에까지 이르는 질환이다. 유병률은 50,000-100,000명당 1명이다. PCCA 또는 PCCB 유전자의 돌연변이로 발생하며, 이것을 규명하는 것이 가장 확실한 진단 방법이다. 두 유형에 따른 임상경과 차이는 뚜렷하게 밝혀져 있지 않다. 발병 시기에 따라 신생아기형과 후기형으로 나눌 수 있다. 증상 발현의 원인은 이화작용을 강화시키는 감염, 스트레스, 변비, 단백질의 과도한 섭취 등이며, 운동실조, 이상행동, 식욕부진, 주기적 구토, 성장장애, 신경발달이상 등의 광범위한 임상 경과를 보인다. 정확한 진단과 조속한 초기 치료가 환자의 생존율 및 신경 발달 장애 여부에 중요한 요소이다. 본 증례는 고암모니아혈증 및 대사산증이 심하지 않았으나 조기에 대사성 질환을 염두에 두고 PCCA와 PCCB 유전자의 돌연변이를 분석하여 프로피온산혈증을 진단하고 적극적인 금식 및 수액 치료와 진단을 통해 신체 발달 및 운동 및 치명적인 신경학적 장애 없이 성장한 고무적인 사례로 이를 보고하는 바이다. Propionic acidemia (PA) is an autosomal recessively inherited disorder of the organic acid metabolism. It is caused by a deficiency of propionyl-CoA carboxylase (PCC). PCC is a heteropolymeric enzyme composed of ${\alpha}$- and ${\beta}$-subunits. The clinical symptoms of PA are heterogeneous and present vomiting, dehydration, hypotonia, and lethargy, and it can result in death. The typical presentations of neonatal onset PA are life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of neonatal onset PA with mild clinical presentations. She was born to a healthy mother without complications. No significant illness was observed until nine days after birth. She started exhibiting poor oral feeding, vomiting, lethargy, and hypotonia at ten days old. Her laboratory results showed mild hyperammonemia and acidosis. The initial diagnosis was neonatal sepsis and she was treated with antibiotics. However, her clinical symptoms didn't improve. So we considered a metabolic disease. She was given nothing by mouth and intravenous hydration and nutrition support was performed. Propionylglycine and 3-hydroxypropionic acid were showed high concentrations in urine by gas chromatograph mass spectrometry (GC-MS). C3 level of acylcarnitine analysis elevated 10.4 uM/L (range, 0.200-5.00) in plasma. We took gene analysis for PA to be based on the symptoms and laboratory results. We detected PCCB gene mutation and diagnosed PA. She survived without severe neurologic defects and complications and was hospitalized only three times with upper respiratory tract infections for 7 years. We report a case of a ten days old neonate with PA presenting without severe metabolic acidosis and hyperammonemia who was effectively treated with early aggressive care and conventional methods.

      • KCI등재

        양돈 농가의 안전사고 발생현황 및 안전보건 관리수준

        김경란,김인수,김효철,이경숙,채혜선,Kim, Kyung-Ran,Kim, Insoo,Kim, Hyo-Cher,Lee, Kyung-Suk,Chae, Hye-Seon 한국환경보건학회 2014 한국환경보건학회지 Vol.40 No.5

        Objectives: The purpose of this study was to seek measures for improvement and management of farm work safety and health by conducting questionnaire surveys and on-site investigations to ascertain the present state of occurrence of safety accidents and safety and health management levels among swine farmers. In particular, the purpose of this study is to provide basic data for the establishment of measures for the management of safety and health suitable to the characteristics of related working environments. Methods: Questionnaire surveys were conducted among 223 farmers engaged in swine farming in 14 regions, and 10 farms were visited in order to implement multilateral methods, including in-depth interviews, along with field surveys. Results: The surveys indicated that 26.2% of all respondents experienced farm-work related safety accidents and body-reaction related accidents showed a high ratio at 31.1% of all respondents. With regard to cause materials of safety accidents, work other than that directly related to swine raising showed high ratios of safety accidents, with pigsty facility related accidents at 26.6%. Although most workers recognized the dangers latent in the working environments, their behavior and responses to the prevention of safety accidents were still insufficient due to a lack of understanding of safety and health management. In the survey on the present state of personal hygiene and wearing of protective equipment, workers were found to have been exposed to dangerous and harmful environments both inside and outside pigsties, but the actual states of their wearing protective safety equipment were very poor. Conclusion: Given the results of this study, swine farmers well recognize problems in their control of safety accidents and management of safety and health, but their knowledge about safety and health education and management guidelines was insufficient. Therefore, safety and health education, public relations, and customized personal protective equipment suitable for swine raising work should be developed in order to address the foregoing problem.

      • KCI등재

        댄스스포츠 참가에 따른 부부갈등해소와 결혼만족과의 관계

        이경일(Kyung Il Lee),정명수(Myung Soo Jung),김경란(Kyung Ran Kim) 한국사회체육학회 2001 한국사회체육학회지 Vol.16 No.-

        The purpose of this study is to investigate the relationship between house wife`s participation on dance sports, marital conflict resolution, and marriage satisfaction. Specifically, it aims to examine: The influence of the dance sports involvement on marital conflict resolution, and marriage satisfaction. The subjects of this study was selected by cluster random sampling of involvement(participation 561, non-participation 177) in dance program facility of Seoul, Kyung-ki, Daejun, and Kwang-ju. The questionnaire for the marital conflict resolution had been developed by Hyun(1994). And also the marriage satisfaction had been developed by Kwon & Chei(1998) The statistical methods such as factor analysis and multiple regression analysis. Marital conflict resolution of dance sport participant house wife are partially influence marriage satisfaction. Among marital conflict resolution of sub-factor, higher dissatisfaction, attack behavior, sex trouble, common time conflict is higher of control and evasion lower of adjustment.

      • KCI등재

        댄스스포츠 참여가 의사소통 , 갈등해소 및 결혼만족에 미치는 영향

        정명수(Myung Soo Jung),이경일(Kyung Il Lee),김경란(Kyung Ran Kim) 한국사회체육학회 2001 한국사회체육학회지 Vol.16 No.-

        The purpose of this study is to investigate the relationship between house wife`s participation on dance sports, marital communication, marital conflict resolution, and marriage satisfaction. Specifically, it aims to examine: The difference of marital communication, marital conflict resolution, and marriage satisfaction by characteristics of dance sports participation-non participation. The subjects of this study was selected by cluster random sampling of involvement(participation 561, non-participation 177) in dance program facility of Seoul, Kyung-ki, Daejun, and Kwang-ju. The survey questionnaires were used to collect the data The questionnaire for the formalization factor marital communication, had been developed by Lee(1991). The questionnaire for the marital conflict resolution had been developed by Hyun(1994). And also the marriage satisfaction had been developed by Kwon & Chei(199h). The statistical methods such as factor analysis, ANCOVA, multiple classification analysis. Path analysis were used to analyze the collected data From the analyses of the data, the study reached the following conclusions: Marital communication, marital conflict Resolution, and marriage satisfaction are partially different by dance sport participation-non participation. That is, dance sports participant is higher reason attitude, conciliatory attitude among sub-factor of marital communication, dance sports participant is higher compromise among sub-factor of marital conflict resolution, but marriage satisfaction is not different.

      • KCI등재

        조선후기 忠淸道 公州牧의 有力 姓氏와 향촌 지배세력의 추이 - 『鄕案』,『居接名錄』의 분석을 중심으로

        김경란(Kim, Kyung-ran) 호서사학회 2019 역사와 담론 Vol.0 No.92

        16세기 이래 在地士族의 향촌 지배의 주요한 수단이었던 鄕案은 18세기 중반을 전후하여 그 성격이 변질되거나 더 이상 작성되지 않았던 것으로 알려져 있다. 그 원인은 지역적인 차이가 있지만 기본적으로 향촌사회에 새롭게 성장한 新鄕과 기존의 지배세력인 舊鄕의 대립으로 인해 야기된 것이었다. 이에 따라 19세기 이후에는 대부분의 지역에서 향안이 작성되지 않았던 것으로 이해되고 있다. 이는 士族支配體制가 종식되었음을 의미하는 것이기도 하다. 그런데 忠淸道 公州牧에서는 19세기 후반까지 향안이 여전히 改修되고 있어 주목된다. 공주목의 주도적인 재지사족의 명단이었던 公州 鄕案은 17세기 전반~19세기 후반까지 약 300여 년 동안 지속적으로 작성되었다. 이를 통해 향안을 중심으로 하는 재지사족의 지배 네트워크가 외형상으로는 19세기 후반까지 여전히 유지되었을 가능성을 유추할 수 있다. 이러한 가능성은 『居接名錄』의 기록을 보면 더욱 확연해진다. 『거접명록』은 19세기 중반 공주목에서 결성된 學契의 명단이다. 여기에 기재된 인물들은 17세기 이후 향안을 주도했던 유력 성씨들이었다. 『거접명록』에는 향안에서 찾아 볼 수 없었던 새로운 성씨가 기재되어 신흥세력이 성장했을 것으로 추정되기도 한다. 하지만 여전히 향안을 주도했던 성씨들이 대다수를 차지하고 있었다. 이러한 사실들을 통해 곧바로 공주목의 향안 질서가 공고하게 유지되었다고 확언하기는 어렵다. 그러나 공주목이 다른 지역에 비해 외형적으로는 비교적 늦게까지 향안을 중심으로 한 재지사족들의 연대가 유지되고 있었을 가능성은 유추할 수 있다. 또한 향촌 질서를 주도했던 유력 성씨가 그 세력을 어느 정도 유지했을 가능성 역시 짐작할 수 있다. The purpose of this study is to analyze the trends of the ruling forces of rural villages in Gongju-mok, Chungcheong-do in terms of influential surnames. To this end, this study analyzed the influential surnames described in the family register and “Geojeob-myeonglog” of Gongju. The family register was no longer created in most regions in Korea around the mid-18th century, while the family register in Gongju continued to be created until the late 19th century. This suggests that the ruling network of the clans of aristocracy and gentility based on the family register still remained formally. The figures in the “Geojeob-myeonglog,” which is the list of learning association formed in Gongju-mok in the mid-19th century, had the surnames of influential families who led the family register since the 17th century. The “Geojeob-myeonglog” had the list of new surnames that could not be found in the family register, indicating the growth of new emerging forces. However, the surnames who led the family register still occupied the majority. These facts suggest that the rule by the clans of aristocracy and gentility based on the family register was maintained to some extent in Gongju-mok.

      • KCI등재

        조선후기 대구부 同姓마을의 형성시기에 대한 검토

        김경란(Kim Kyung-ran) 한국사학회 2016 史學硏究 Vol.0 No.123

        이 글은『慶尙道大丘府戶籍大帳』의 기록을 토대로 同姓마을이 형성되는 시기와 그 과정에 주목한 연구이다. 同姓마을의 형성시기와 과정에 대한 분석 작업은 性理學的이념에 입각한 父系的친족질서가 조선사회에 확산되는 시점 및 그 과정을 확인할 수 있는 단서가 된다는 점에서 매우 주요한 문제이다. 이러한 이유로 경상도 대구부 지역의 동성마을 형성시기와 그 과정에 대한 분석 작업을 진행하였고, 그 결과를 요약하면 다음과 같다. 첫째, 지역 내 同姓同本집단의 거주가 시간이 경과될수록 동성마을로 집중되는 양상이 나타났다. 둘째, 지속적인 集居를 통해 동성마을을 형성하였던 姓貫이 있었던 것에 비해 집거를 지속적으로 유지하지 못하고 마을에서 사라지는 성관들도 발견된다. 셋째, 한 마을에 2개 이상의 성관이 공존하면서 동성마을을 형성한 사례도 확인할 수 있다. 넷째, 20세기 전반 무렵 대구지역에 분포했던 동성마을 중 적지 않은 수가 19세기 후반 내지 20세기에 형성된 것이었고, 그 이전인 18세기 후반 또는 19세기에 형성되었던 동성마을은 대부분 양반마을이었으나 일부 평민 동성마을도 형성되기 시작했음을 발견할 수 있었다. 이상의 사실을 종합해 볼 때, 대구부의 동성마을은 주로 18세기 후반~19세기 중반에 본격적으로 형성되기 시작했으며, 19세기 후반 내지 20세기에 형성된 마을도 적지 않았을 것으로 보인다. 또한 일부 평민 동성마을도 형성되기 시작했다는 점은 상당히 흥미로운 사실이다. 왜냐하면 동성마을이 조선후기 부계적 친족질서의 최종적 귀결점이라는 일반적 이해에 비추어 볼 때, 대구부의 사례는 우리 사회에 성리학에 입각한 가부장적 부계질서가 뿌리내린 시점이 지역에 따라서는 일반적 이해보다 더 늦었을 가능성을 시사해 준다. 대구부 지역의 경우에는 동성마을이 급증하고, 평민층도 동성마을을 형성했던 19세기 후반 내지 20세기 전반기에도 여전히 부계적 친족질서가 강화되고 있었던 것으로 여겨진다. 근대에 들어와서도 조선후기적 가치인 가부장적 친족질서가 약화되지 않고 오히려 강화되는 양상이 나타났던 것이다. An analysis on the time and process of formation of clan villages is very meaningful in that it is a clue to identifying the time and process of spread of patrilineal kindred order based on Neo-Confucianism in Joseon society. In this sense, this study conducted an analysis on the time and process of formation of clan villages in Daegu prefecture, Gyeongsang Province. The analysis result shows that a lot of clan villages in Daegu prefecture were formed from the late 18th to the mid-19th century and that not a few clan villages were formed from the late 19th to the 20th century. It is very interesting that commoners’ clan villages also began to be formed. In the light of general understanding that a clan village is the ultimate endpoint of patrilineal kindred order in the late Joseon period, this case of Daegu prefecture suggests that patrilineal order based on Neo-Confucianism is likely to have been rooted in the overall society later than generally understood. It seems that, in Daegu prefecture, the late 19th to the early 20th century when clan villages increased rapidly and also commoners began to form clan villages was a time when patrilineal kindred order was most strengthened.

      • KCI등재

        일반논문 : 18세기 경상도(慶尙道) 단성현(丹城縣)의 개관실태(改貫實態)와 특징

        김경란 ( Kyung Ran Kim ) 수선사학회 2013 史林 Vol.0 No.46

        The 18th century witnessed a major change in the family clan system in Korea; namely, there occurred a rapid trend for having a family name with the 18th century as a starting point. In the process, in which numerous classes without their own family name got to have a new family name, the family clan system of the Joseon Dynasty came to be faced with changes from various aspects. One of the major changes is the very progression of the family clan change. ‘Gaegwan’ means changing one`s own family clan to other one with the family name left as it is. As a result of taking note of the issue about how the family clan change was in progress around the former half of the 18th century, this study could confirm that the family clan change was made led by those who belonged to the lower-middle classes hierarchically. Such an aspect slightly differed from the tendency for the family clan change made before the 18th century. In other words, the trend for a family clan change in the former half of the 18th century took an different aspect from the trend for a family clan until the early days of the Joseon Dynasty when mainly the yangban (the gentry) changed a family clan in order to get a superior one. However, there is also the same tendency in that even the lower-middle classes who made a family clan change in the 18th century tended to reject a rare family clan, i.e. scarcity family clan. There came up a phenomenon of some family clans` transformation into an illustrious family clan after the 18th century at Danseong-hyeon; hereupon, this study thinks that the tendency to evade the scarcity family clan in the process of a family clan change led by the lower-middle classes at that period might serve as a contributor to the formation of an illustrious family clan.

      • KCI등재후보

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