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The Science-Related Attitudes from Adults' Experiences during Science Cultural Activities: Focusing on the Case of Science Fiction Discussions (성인들의 과학문화 활동 경험에서 나타난 과학 관련 태도 -과학소설 독서토론 활동 사례를 중심으로-)

  • Eunji Kang;Chaeyeon Shin;Jinwoong Song
    • Journal of The Korean Association For Science Education
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    • v.43 no.2
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    • pp.139-150
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    • 2023
  • This study started with the awareness of the need to explore various aspects of science education and was conducted according to the necessity of practical research on science cultural activities targeting adults. Accordingly, adults' book discussions of science fiction were selected as research cases, and science-related attitudes in science cultural activities were explored. There are four participants in the study, all of whom have engaged in a book club and have not majored or are working in science disciplines. Three science fictions were selected after establishing specific standards for the selection discussed with participants. For four months, a total of three unstructured book discussions of science fiction, post-interviews for each discussion, and in-depth individual interviews after the end of the entire activity were conducted. Various data such as recorded and transcribed reading discussion discourse, post- and in-depth individual interviews, researchers' observation records, and participants' book journals were collected and analyzed using a continuous comparison method. As a result of the study, as scientific thinking is illustrated in SF, the participants also demonstrated scientific attitudes during their discussions. In addition, the textual feature(storytelling) of science fiction was found to lessen cognitive overload and the burden of understanding science by providing scientific knowledge with context. Finally they demonstrated a shift in attitude toward science, valuing science cultural activities in themselves, rather than simply viewing science as a subject of understanding and learning. The conclusions and meanings of this study based on the above results are presented to enhance a positive attitude toward science for adults even after school education.

Interior Settings of a Chamber and a Temporary Place of Enshrinement at Yeonghuijeon and Features of the Five Peak Screens for the Hall (영희전 감실 및 이안소의 공간 구성과 오봉산병풍의 특징)

  • SON Myenghee
    • Korean Journal of Heritage: History & Science
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    • v.56 no.2
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    • pp.100-121
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    • 2023
  • This paper examines the interior settings of a chamber and of a temporary place of enshrinement at Yeonghuijeon (永禧殿, Hall of Eternal Happiness), the representative official portrait hall in which portraits of early and late Joseon kings were enshrined. Also, it discusses the features of the Five Peak screens used therein. The physical environment of a chamber at Yeonghuijeon mainly consisted of a four-panel folding screen with a painting of Five Peaks and a large wooden platform, which was adorned with dragon and lion patterns and attached to lotus-leaf column balustrades. The Five Peak screen was installed on a large platform in the shape of ⊓, spreading across the second and third panels on the back and folding out on the first and fourth panels on the right and left sides. When a portrait was enshrined in a temporary place, a simpler and smaller platform with railings was used. A four-panel folding screen of the Five Peak painting was installed in the same way as in a chamber, but was unfolded around a smaller platform behind it. A royal portrait was displayed in each chamber, whereas a case in which a portrait was rolled up was put on the smaller platform in a temporary place. The Five Peak screens for a chamber and a temporary place were all large four-panel folding screens with two wide panels in the middle and two narrow panels on each side, and only strips of silk were mounted on the four edges of the screens without additional wide lower-side mountings. While screens for the chamber used patterned silk for mounting and white paper for backing on screen frames, screens for the temporary place used plain silk and recycled failed test papers for mounting and backing, respectively. By examining records in the literature on the Five Peak screens for Yeonghuijeon, this paper highlights two Five Peak screens, both of which lost their provenance from the hall. The structures of the two screens reflected the way they were to be installed at the hall. Furthermore, this paper assumes that a Five Peak screen, which had been unfolded on the throne in the main hall of Changdeokgung Palace after the 1960s, was produced in 1858 for the purpose of temporarily enshrining King Sunjo's portrait due to the fact that failed test papers of the 1840s were laid taut over the frame.

A Study on the Interactive Narrative - Focusing on the analysis of VR animation <Wolves in the Walls> (인터랙티브 내러티브에 관한 연구 - VR 애니메이션 <Wolves in the Walls>의 분석을 중심으로)

  • Zhuang Sheng
    • Trans-
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    • v.15
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    • pp.25-56
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    • 2023
  • VR is a dynamic image simulation technology with very high information density. Among them, spatial depth, temporality, and realism bring an unprecedented sense of immersion to the experience. However, due to its high information density, the information contained in it is very easy to be manipulated, creating an illusion of objectivity. Users need guidance to help them interpret the high density of dynamic image information. Just like setting up navigation interfaces and interactivity in games, interactivity in virtual reality is a way to interpret virtual content. At present, domestic research on VR content is mainly focused on technology exploration and visual aesthetic experience. However, there is still a lack of research on interactive storytelling design, which is an important part of VR content creation. In order to explore a better interactive storytelling model in virtual reality content, this paper analyzes the interactive storytelling features of the VR animated version of <Wolves in the walls> through the methods of literature review and case study. We find that the following rules can be followed when creating VR content: 1. the VR environment should fully utilize the advantages of free movement for users, and users should not be viewed as mere observers. The user's sense of presence should be fully considered when designing interaction modules. Break down the "fourth wall" to encourage audience interaction in the virtual reality environment, and make the hot media of VR "cool". 2.Provide developer-driven narrative in the early stages of the work so that users are not confused about the ambiguous world situation when they first enter a virtual environment with a high degree of freedom. 1.Unlike some games that guide users through text, you can guide them through a more natural interactive approach that adds natural dialog between the user and story characters (NPC). Also, since gaze guidance is an important part of story progression, you should set up spatial scene user gaze guidance elements within it. For example, you can provide eye-following cues, motion cues, language cues, and more. By analyzing the interactive storytelling features and innovations of the VR animation <Wolves in the walls>, I hope to summarize the main elements of interactive storytelling from its content. Based on this, I hope to explore how to better showcase interactive storytelling in virtual reality content and provide thoughts on future VR content creation.

Comparison of Factors Associated With Agitation Among Youngest-Old, Middle-Old, and Oldest-Old Hospitalized Patients Referred to the Psychiatric Department (정신건강의학과에 협진의뢰된 연소노인, 고령노인, 초고령노인 입원환자의 초조의 연관요인 비교)

  • Min-Suk Jang;Seo-Hyun Choi;Se-ri Maeng;Yang-Sik Kim;Jae-Nam Bae;Jeong-Seop Lee;Won-Hyoung Kim
    • Korean Journal of Psychosomatic Medicine
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    • v.31 no.2
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    • pp.89-99
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    • 2023
  • Objectives : In this study, elderly hospitalized patients aged 65 years or older who were admitted to a university hospital and referred to the department of psychiatry were classified into youngest-old, middle-old, and oldest-old. It was conducted to find out what factors contribute to the agitation pattern by age group, whether there is a difference in the factors, and whether there is a difference in the severity of the agitation pattern. Methods : From July 1, 2021 to December 31, 2021, the medical records of patients aged 65 years or older who were referred to the department of psychiatry were retrospectively reviewed. Age, gender, route of hospitalization, department of referral, reason for referral, treatment method, presence of internal and surgical diseases, and hematological test data were investigated. Results : There was a significant correlation with the RASS score in cases of surgery referral and high CRP levels for youngest-old aged 65 to 74 years, in cases of surgery referral, dementia and hyponatremia for middle-old aged 75 to 84 years, in case of dementia for oldest-old aged 85 years old or older. In addition, there were differences in the severity of agitation patterns between age groups. Conclusions : As the age group increases, the agitation of delirium patients becomes more severe, and various factors contributing to the agitation pattern also differ by age group. Therefore, when treating elderly patients with delirium, attention should be paid to factors that may affect agitation depending on age.

Buddhist Sculptures from Seongbulsa Temple in Hwanghae-do Province as Seen through Gelatin Dry Plates and Archival Materials from the Collection of the National Museum of Korea (국립중앙박물관 소장 유리건판과 기록자료로 본 황해도 성불사(成佛寺)의 불교조각)

  • Heo Hyeonguk
    • Bangmulgwan gwa yeongu (The National Museum of Korea Journal)
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    • v.1
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    • pp.278-305
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    • 2024
  • Gelatin dry plate photographs dating to the Japanese colonial era and the official documents from the Japanese Government-General of Korea Museum in the collection of the National Museum of Korea are significant materials documenting cultural heritage in North Korea before it was severely damaged in 1950 during the Korean War. There has been an increase in recent years in studies of Buddhist sculptures in North Korea based on these photographs and documents. This paper presents some new comments on the Buddhist sculptures at Seongbulsa Temple in Hwangju, one of the most famous temples in Hwanghae-do Province, based on the related existing research outcomes. This paper aims to facilitate a more comprehensive understanding of the Buddhist sculptures at Seongbulsa Temple by chronicling its history based on historical records, examining its current status, and exploring in detail the production dates and backgrounds of the Buddhist sculptures featured on gelatin dry plates. Prior to Korea's liberation from Japan in 1945, Seongbulsa housed at least seven sculptural items: two Bodhisattva statues, four Buddha statues, and a triad. Two items are from the early Goryeo period, one is from the late Goryeo period, three are from the early Joseon period, and one is from the late Joseon period. Among them, two surviving items are noteworthy. One is the early Goryeo-era Stone Seated Bhaishajyaguru Buddha photographed in Eungjinjeon Hall at Seongbulsa Temple. A close examination of a schematic drawing of the sculpture's pedestal made at the time it was photographed reveals that its material accords with the materials used for the headless Stone Seated Bhaishajyaguru Buddha and pedestal currently found in the old Sangwonam Hermitage site in the Inner Geumgang Valley of Jeongbangsan Mountain. This accordance could mean that the statue is a new significant example of early Goryeo Buddhist sculpture in North Korea. The other notable sculpture is the Gilt-bronze Seated Amitabha Buddha Triad created in 1454 (the second year of the reign of King Danjong) and discovered in Geungnakjeon Hall at Seongbulsa. This statue is currently in the collection of the Sariwon History Museum in Hwanghae-do Province. It is an important example of a dated small gilt-bronze Buddhist statue from the early Joseon period found in North Korea. This paper is a case study of Buddhist sculptures in North Korea, focusing on Seongbulsa Temple. Further utilization of the National Museum of Korea's gelatin dry plates will contribute to developing the study of the history of Korean Buddhist sculpture.

A Study on the Excavated Sab(a funeral fan) from Lime-filled Tomb and Lime-layered Tomb during the Joseon Dynasty (조선시대 회격·회곽묘 출토 삽(翣)에 대한 고찰)

  • Yi, Seung Hae;An, Bo Yeon
    • Korean Journal of Heritage: History & Science
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    • v.41 no.2
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    • pp.43-59
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    • 2008
  • Sap(?, a funeral fan) is a funeral ceremonial object used in association with a Confucian ceremonial custom, which was crafted by making a wooden frame, attaching a white cloth or a thick paper onto it, drawing pictures on it, and making a holder for a handle. According to Liji(Records of Rites), Sap was used since the Zhou Dynasty, and these Chinese Sap examples are no big different than the Korean Sap examples, which were described in Joseon Wangjo Sillok(Annals of the Joseon Dynasty), Gukjo Oryeui(the Five Rites of the State), and Sarye Pyeollam(Handbook on Four Rituals). This study explored Sap excavated in lime-filled tombs and lime-layered tombs of aristocrats dating back to Joseon, as well as their historical records to examine Sap's characteristics according to their examples, manufacturing methods, and use time. The number and designs of Sap varied according to the deceased' social status aristocrats used mainly one pair of 亞-shaped Bulsap, and a pair of Hwasap with a cloud design depicted on it. A Sap was wrapped twice with Chojuji paper or Jeojuji paper, and for the third time with Yeonchangji paper. Then, it was covered with a white ramie, a hemp, a cotton, a silk satin, etc. Bobul(an axe shape and 亞-shape design) was drawn on both sides of Sap, and a rising current of cloud was drawn at the peripheral area mainly with red or scarlet pigments. Sap, which were excavated from aristocrats'lime-filled and lime-layered tombs, are the type of Sap which were separated from its handle. These excavated Sap are those whose long handles were burnt during the death carriage procession, leaving Sap, which later were erected on both sides of the coffin. The manufacturing process of excavated relics can be inferred by examining them. The excavated relics are classified into those with three points and those with two points according to the number of point. Of the three-point type(Type I), there is the kind of relic that was woven into something like a basket by using a whole wood plate or cutting bamboo into flat shapes. The three-point Sap was concentrated comparatively in the early half of Joseon, and was manufactured with various methods compared with its rather unified overall shape. In the meantime, the two-point Sap was manufactured with a relatively formatted method; its body was manufactured in the form of a rectangle or a reverse trapezoid, and then its upper parts with two points hanging from them were connected, and the top surface was made into a curve(Type II) or a straight line(Type III) differentiating it from the three-point type. This manufacturing method, compared with that of the three-point type, is simple, but is not greatly different from the three-point type manufacturing method. In particular, the method of crafting the top surface into a straight line has been used until today. Of the examined 30 Sap examples, those whose production years were made known from the buried persons'death years inscribed on the tomb stones, were reexamined, indicating that type I was concentrated in the first half of the $16^{th}$ century. Type II spanned from the second half of the $16^{th}$ century to the second half of the $17^{th}$ century, and type III spanned from the first half of the $17^{th}$ century to the first half of the $18^{th}$ century. The shape of Sap is deemed to have changed from type I to type II and again from type II to type III In the $17^{th}$ century, which was a time of change, types II and III coexisted. Of the three types of Sap, types II and III re similar because they have two points; thus a noteworthy transit time is thought to have been the middle of the $16^{th}$ century. Type I compared with types II and III is thought to have required more efforts and skills in the production process, and as time passed, the shape and manufacturing methods of Sap are presumed to have been further simplified according to the principle of economy. The simplification of funeral ceremonies is presumed to have been furthered after Imjinwaeran(Japanese invasion of Joseon, 1592~1598), given that as shown in the Annals of King Seonjo, state funerals were suspended several times. In the case of Sap, simplification began from the second half of the $16^{th}$ century, and even in the $18^{th}$ century, rather than separately crafting Sap, Sap was directly drawn on the coffin cover and the coffin. However, in this simplification of form, regulations on the use of Sap specified in Liji were observed, and thus the ceremony was rationally simplified.

Indonesia, Malaysia Airline's aircraft accidents and the Indonesian, Korean, Chinese Aviation Law and the 1999 Montreal Convention

  • Kim, Doo-Hwan
    • The Korean Journal of Air & Space Law and Policy
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    • v.30 no.2
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    • pp.37-81
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    • 2015
  • AirAsia QZ8501 Jet departed from Juanda International Airport in, Surabaya, Indonesia at 05:35 on Dec. 28, 2014 and was scheduled to arrive at Changi International Airport in Singapore at 08:30 the same day. The aircraft, an Airbus A320-200 crashed into the Java Sea on Dec. 28, 2014 carrying 162 passengers and crew off the coast of Indonesia's second largest city Surabaya on its way to Singapore. Indonesia's AirAsia jet carrying 162 people lost contact with ground control on Dec. 28, 2014. The aircraft's debris was found about 66 miles from the plane's last detected position. The 155 passengers and seven crew members aboard Flight QZ 8501, which vanished from radar 42 minutes after having departed Indonesia's second largest city of Surabaya bound for Singapore early Dec. 28, 2014. AirAsia QZ8501 had on board 137 adult passengers, 17 children and one infant, along with two pilots and five crew members in the aircraft, a majority of them Indonesian nationals. On board Flight QZ8501 were 155 Indonesian, three South Koreans, and one person each from Singapore, Malaysia and the UK. The Malaysia Airlines Flight 370 departed from Kuala Lumpur International Airport on March 8, 2014 at 00:41 local time and was scheduled to land at Beijing's Capital International Airport at 06:30 local time. Malaysia Airlines also marketed as China Southern Airlines Flight 748 (CZ748) through a code-share agreement, was a scheduled international passenger flight that disappeared on 8 March 2014 en route from Kuala Lumpur International Airport to Beijing's Capital International Airport (a distance of 2,743 miles: 4,414 km). The aircraft, a Boeing 777-200ER, last made contact with air traffic control less than an hour after takeoff. Operated by Malaysia Airlines (MAS), the aircraft carried 12 crew members and 227 passengers from 15 nations. There were 227 passengers, including 153 Chinese and 38 Malaysians, according to records. Nearly two-thirds of the passengers on Flight 370 were from China. On April 5, 2014 what could be the wreckage of the ill-fated Malaysia Airlines was found. What appeared to be the remnants of flight MH370 have been spotted drifting in a remote section of the Indian Ocean. Compensation for loss of life is vastly different between US. passengers and non-U.S. passengers. "If the claim is brought in the US. court, it's of significantly more value than if it's brought into any other court." Some victims and survivors of the Indonesian and Malaysia airline's air crash case would like to sue the lawsuit to the United States court in order to receive a larger compensation package for damage caused by an accident that occurred in the sea of Java sea and the Indian ocean and rather than taking it to the Indonesian or Malaysian court. Though each victim and survivor of the Indonesian and Malaysia airline's air crash case will receive an unconditional 113,100 Unit of Account (SDR) as an amount of compensation for damage from Indonesia's AirAsia and Malaysia Airlines in accordance with Article 21, 1 (absolute, strict, no-fault liability system) of the 1999 Montreal Convention. But if Indonesia AirAsia airlines and Malaysia Airlines cannot prove as to the following two points without fault based on Article 21, 2 (presumed faulty system) of the 1999 Montreal Convention, AirAsia of Indonesiaand Malaysia Airlines will be burdened the unlimited liability to the each victim and survivor of the Indonesian and Malaysia airline's air crash case such as (1) such damage was not due to the negligence or other wrongful act or omission of the air carrier or its servants or agents, or (2) such damage was solely due to the negligence or other wrongful act or omission of a third party. In this researcher's view for the aforementioned reasons, and under the laws of China, Indonesia, Malaysia and Korea the Chinese, Indonesian, Malaysia and Korean, some victims and survivors of the crash of the two flights are entitled to receive possibly from more than 113,100 SDR to 5 million US$ from the two airlines or from the Aviation Insurance Company based on decision of the American court. It could also be argued that it is reasonable and necessary to revise the clause referring to bodily injury to a clause mentioning personal injury based on Article 17 of the 1999 Montreal Convention so as to be included the mental injury and condolence in the near future.

Feasibility of Deep Learning Algorithms for Binary Classification Problems (이진 분류문제에서의 딥러닝 알고리즘의 활용 가능성 평가)

  • Kim, Kitae;Lee, Bomi;Kim, Jong Woo
    • Journal of Intelligence and Information Systems
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    • v.23 no.1
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    • pp.95-108
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    • 2017
  • Recently, AlphaGo which is Bakuk (Go) artificial intelligence program by Google DeepMind, had a huge victory against Lee Sedol. Many people thought that machines would not be able to win a man in Go games because the number of paths to make a one move is more than the number of atoms in the universe unlike chess, but the result was the opposite to what people predicted. After the match, artificial intelligence technology was focused as a core technology of the fourth industrial revolution and attracted attentions from various application domains. Especially, deep learning technique have been attracted as a core artificial intelligence technology used in the AlphaGo algorithm. The deep learning technique is already being applied to many problems. Especially, it shows good performance in image recognition field. In addition, it shows good performance in high dimensional data area such as voice, image and natural language, which was difficult to get good performance using existing machine learning techniques. However, in contrast, it is difficult to find deep leaning researches on traditional business data and structured data analysis. In this study, we tried to find out whether the deep learning techniques have been studied so far can be used not only for the recognition of high dimensional data but also for the binary classification problem of traditional business data analysis such as customer churn analysis, marketing response prediction, and default prediction. And we compare the performance of the deep learning techniques with that of traditional artificial neural network models. The experimental data in the paper is the telemarketing response data of a bank in Portugal. It has input variables such as age, occupation, loan status, and the number of previous telemarketing and has a binary target variable that records whether the customer intends to open an account or not. In this study, to evaluate the possibility of utilization of deep learning algorithms and techniques in binary classification problem, we compared the performance of various models using CNN, LSTM algorithm and dropout, which are widely used algorithms and techniques in deep learning, with that of MLP models which is a traditional artificial neural network model. However, since all the network design alternatives can not be tested due to the nature of the artificial neural network, the experiment was conducted based on restricted settings on the number of hidden layers, the number of neurons in the hidden layer, the number of output data (filters), and the application conditions of the dropout technique. The F1 Score was used to evaluate the performance of models to show how well the models work to classify the interesting class instead of the overall accuracy. The detail methods for applying each deep learning technique in the experiment is as follows. The CNN algorithm is a method that reads adjacent values from a specific value and recognizes the features, but it does not matter how close the distance of each business data field is because each field is usually independent. In this experiment, we set the filter size of the CNN algorithm as the number of fields to learn the whole characteristics of the data at once, and added a hidden layer to make decision based on the additional features. For the model having two LSTM layers, the input direction of the second layer is put in reversed position with first layer in order to reduce the influence from the position of each field. In the case of the dropout technique, we set the neurons to disappear with a probability of 0.5 for each hidden layer. The experimental results show that the predicted model with the highest F1 score was the CNN model using the dropout technique, and the next best model was the MLP model with two hidden layers using the dropout technique. In this study, we were able to get some findings as the experiment had proceeded. First, models using dropout techniques have a slightly more conservative prediction than those without dropout techniques, and it generally shows better performance in classification. Second, CNN models show better classification performance than MLP models. This is interesting because it has shown good performance in binary classification problems which it rarely have been applied to, as well as in the fields where it's effectiveness has been proven. Third, the LSTM algorithm seems to be unsuitable for binary classification problems because the training time is too long compared to the performance improvement. From these results, we can confirm that some of the deep learning algorithms can be applied to solve business binary classification problems.

The Characteristics and Fates of Pulmonary Tuberculosis Patients Seen at Medical Department of A Medium Sized General Hospital (부산에 한 중형 종합 병원 내과에서의 폐결핵 환자의 양상과 귀결)

  • Kim, Young-Hyo;Park, Ki-Chan;Bae, Seong;Lee, Sang-Hun;Chun, Myung-Ho;Lee, Sang-Ki;Jun, Kwang-Su;Lee, Chan-Se
    • Tuberculosis and Respiratory Diseases
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    • v.39 no.5
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    • pp.417-424
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    • 1992
  • Background: There were many reports about the clinical aspect and outcomes of pulmonary tuberculosis in health center but few in a medium sized general hospital. The purposes of this study were to find any characteristic differencies in the patients and the general outcomes of the treatments and also to give some suggestive points for the insurance policy making. Methods: We made a retrograde analysis of the medical records of 1981 patients (male 992 female 1,059) who attended our clinics of the 4th internal department, Daedong general hospital during two years from January 1989 to December 1990. Result: 1) Of 1981 patients, 96 were diagnosed as pulmonary tuberculosis taking relatively large proportion in the prevalence. The ratio of prevalence between male and female was 7.81% to 2.27%. The 61.46% were the first diagnosis & initial treatment cases and the remaining 38.54% were the retreatment cases with no statistical significance between sex. 2) The most prevalent age group was between 21~40 years old and the prevalence rate was 45.45% of male and 76.76% of female. The lowest age group in male patient was above 61 showed 3.03%, and there was no female patients above age 60 years old. This phenomena could be thought as the negligence for the treatment of pulmonary tuberculosis in the old age groups rather than true tuberculosis prevalence and it could be proved by the higher rates towards old age groups in the national tuberculosis prevalence survey. 3) There were 57.07% of the minimal case, 48.96% of the moderate, 18.75% of the far advanced. The sputum examination showed 37.07% were culture positive, 46.88% were the negative, and 15.63% of the patients had no stutum examination. Moreover, uncoperatives among the far advanced cases were notable showing 22.22% of the stutum examination, where 16.13% in the minimal cases. The stutum positive rate among the initial treatment cases were 41.07% and 55.00% for the retreatment cases. The sputum no examination rates were 17.86% and 12.50% respectively. 4) The classfication of the mode of disease onset showed 68.75% with gradual onset, 9.38% hemoptic, 3.13% acute pneumonic and 18.75% was found through the radiologic examination in various occasions. 5) The percentages of patients who continued their treatment for more than 8 month were 35.71% (for initial treatment), 25.00% (for retrement), 16.13% (for the minimal), 27.78% (for the far advanced). 6) The group of patients who were treated more than 8 months showed the negative conversion rate of 80% on sputum and marked improvement on chest x-ray in 56.67%. However, in far advanced or retreatment cases, the rate of negative conversion on sputum and the rate of improvement on chest x-ray were low being 60% and 20% for the former and 60% and 10% for the latter, each respectively. Conclusion: It would be strongly emphasized that the improvement of National medical insurance system and social welfare system in Korea must be definite to improve overall treatment and control of tuberculosis diseases as well as physician's devotious National tuberculosis control policy.

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Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.222-230
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    • 2005
  • Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

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