• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.35-42
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• 2018

Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• International Area Studies Review
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• v.22 no.1
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• pp.215-235
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• 2018

Policy concerns related to raising fertility rates are not only common interests among the OECD countries, but they are also issues of great concern to South Korea whose fertility rate is the lowest in the world. The fertility rate in South Korea continues to decline, even though most of the national budget has been spent on measures to address this and many studies have been conducted on the increase in the fertility rates. In this regard, this study aims to verify the effectiveness of the detailed factors affecting the fertility rate that have been discussed in the previous studies on fertility rates, and to investigate the overall trend toward enhancing the quality of life and increasing the fertility rate through macroscopic and structural studies under the recognition of problems related to the policy approaches through the case studies of the European countries. Toward this end, this study investigated if a high quality of life in advanced countries contributes to the increase in the fertility rate, which country serves as a state model that has a high quality of life and a high fertility rate, and what kind of social and policy environment does the country have with regard to childbirth. The analysis of the OECD Better Life Index (BLI) and CIA fertility rate data showed that the countries whose people enjoy a high quality of life do not necessarily have high fertility rates. In addition, under the recognition that a country with a high quality of life and a high birth rate serves as a state model that South Korea should aim for, the social characteristics of Iceland, Ireland, and New Zealand, which turned out to have both a high quality of life and a high fertility rate, were compared with those of Germany, which showed a high quality of life but a low fertility rate. According to the comparison results, the three countries that were mentioned showed higher awareness of gender equality; therefore, the gender wage gap was small. It was also confirmed that the governments of these countries support various policies that promote both parents sharing the care of their children. In Germany, on the other hand, the gender wage gap was large and the fertility rate was low. In a related move, however, the German government has made active efforts to a paradigm shift toward gender equality. The fertility rate increases when the synergy lies in the relationship between parents and children; therefore, awareness about gender equality should be firmly established both at home and in the labor market. For this reason, the government is required to provide support for the childbirth and rearing environment through appropriate family policies, and exert greater efforts to enhance the effectiveness of the relevant systems rather than simply promoting a system construction. Furthermore, it is necessary to help people in making their own childbearing decisions during the process of creating a better society by changing the national goal from 'raising the fertility rate' to 'creating a healthy society made of happy families'

• Korean Journal of Poultry Science
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• v.45 no.4
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• pp.253-260
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• 2018

The hepatocyte nuclear factor 4 alpha ($HNF4{\alpha}$) gene is related to lipid transport, including abdominal fat and growth, in chickens. Interestingly, the A543G SNP within the $HNF4{\alpha}$ gene has previously been reported to be associated with body weight in both broilers and Korean native chickens (KNCs). However, its exact position within the HNF4 is not yet reported. This study aimed to identify the position of the A543G SNP and to identify additional SNPs that can be used as genetic markers in KNCs. A total of 128 KNCs were used for the sequencing and analysis of these genetic associations. As a result, A543G SNP was located in intron 4 of the $HNF4{\alpha}$ gene; it is reported as rs731246957 in the NCBI database. Fourteen SNPs were detected in the sequenced portion of the $HNF4{\alpha}$ gene; three of these, rs731246957, rs736159604 and new SNP, intron 6 (249), were significantly related with growth in the chickens. In this study, the TT genotype of rs731246957, previously reported as A543G SNP, the GG genotype of rs736159604 and GT of new SNP have are highly associated with body weight from birth to 40 weeks of age in KNCs (P<0.01). These results suggest that rs736159604, rs731246957 and intron 6 (249) SNPs within the $HNF4{\alpha}$ gene could function as growth-related markers in the selective breeding of KNCs.

• Korean Journal of Heritage: History & Science
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• v.42 no.3
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• pp.66-87
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• 2009

The definition of the structure of wooden chamber tomb(piled stone-type) is as follows. It is a tomb with wooden chamber, and stones were piled on top of the wooden chamber, and then a wooden structure was placed on top of the piled stones, and more stones were piled on top of the wooden structure, and sealed with clay. Of course this definition can vary according to periods, the buried, etc. Gyeongju-style piled stone type wooden chamber tombs have some distinguished characteristics compared to general definition of piled stone type wooden chamber tombs. Outside the wooden chamber, either stone embankments or filled-in stones were layed out, and pilet-in stones are positioned right above the wooden chamber, and almost every class used this type, and finally, it is exclusively found in Gyeongju area. First generations of this Gyeongju-style piled stone type wooden chamber tombs appeared in first half of 5th century. These tombs inherited characteristics like ground plan, wooden chamber, double chamber(inner chamber and outer chamber), piled stones, burial of the living with the dead, piled stones, from precedent wooden chamber tombs. However these tombs have explicit new characteristics which are not found in the precedent wooden chamber tombs such as stone embankments, wooden pillars, piled stones(above ground level), soil tumuluses. stone embankments and wooden pillars are exclusively found on great piled stone type above-ground level wooden chamber tombs such as the Hwangnamdaechong(皇南大塚). Stone embankments, wooden pillars, piled stones(above ground level) are all elements of building process of soil tumuluses. stone embankments support outer wall of above-ground level wooden chambers and disperse the weight of tumuluses. Wooden pillars functioned as auxiliary supports with wooden structures to prevent the collapse of stone embankments. Piled stones are consists of stones of regular size, placed on the wooden structure. And after the piled stones were sealed with clay, tumulus was built with soil. Piled stones are unique characteristics which reflects the environment of Gyeongju area. Piled stone type wooden chamber tombs are located on the vast and plain river basin of Hyeongsan river(兄山江). Which makes vast source of sands and pebbles. Therefore, tumulus of these tombs contains large amount of sands and are prone to collapse if soil tumulus was built directly on the wooden structure. Consequently, to maintain external shape of the tumulus and to prevent collapse of inner structure, piled stones and clay-sealing was made. In this way, they can prevent total collapse of the tombs even if the tumulus was washed away. The soil tumulus is a characteristic which emerges when a nation or political entity reaches certain growing stage. It can be said that after birth of a nation, growing stage follows and social structure will change, and a newly emerged ruling class starts building new tombs, instead of precedent wooden chamber tombs. In this process, soil tumulus was built and the size and structure of the tombs differ according to the ruling class. Ground plan, stone embankments, number of the persons buried alive with the dead, quantity and quality of artifacts reflect social status of the ruling class. In conclusion, Gyeongju-style piled stone type wooden chamber tombs emerged with different characteristics from the precedent wooden chamber tombs when Shilla reached growing stage.

• Korean Journal of Heritage: History & Science
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• v.44 no.1
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• pp.52-71
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• 2011

This study was initiated with the intent to consider the features of Gugokwonlim and to compare Gabsagugok(甲寺九曲) to Yongsangugok(龍山九曲) against the backdrop of Mt. Gyeryong by revealing their nature and confirming the names and exact locations. A literature review, interviews with local people and field studies confirmed that Gabsagugok and Yongsangugok are each composed of 9 seasonal features. The former is made up of Yongyuso(龍遊沼) - Iilcheon(二一川) - Baengnyonggang(白龍岡) - Dalmuntaek(達門澤) - Geumgyeam(金鷄?) - Myeongwoldam(明月潭) - Gyemyeongam(鷄鳴巖) - Yongmunpok(龍門瀑) - Sujeongbong(水晶峰) while the latter is made up of Simyongmun(尋龍門) - Eunnyongdam(隱龍潭) - Waryonggang(臥龍剛) - Yuryongdae(遊龍臺) - Hwangnyongam(黃龍岩) - Hyeollyongso(見龍沼) - Ullyongtaek(雲龍澤) - Biryongchu(飛龍湫) - Sillyongyeon(神龍淵). Both Gabsagugok and Yongsangugok are part of Gugokwonlim built in the valleys of Mt. Gyeryong in the late Joseon Dynasty by Byeoksu Yun Deok-yeong (1927) and Chwieum Gwon Jun-myeon (1932), respectively, with a 5 year difference. Gabsagugok was supposedly designed to reflect an individual taste for the arts and to admire principles of Juyeok (ch. Zhouyi) and the beauty of nature. On the contrary, Yongsangugok appears to be the builder's expression of his longing for independence day, likened to the life of a dragon after receiving the sad news of Japan's annexation of Korea. Such differences show that these two builders had very different intentions from one another. The letters of Gabsagugok have a semi cursive style and were deeply engraved on the rock in a square shape. Consequently they have not been worn away except for those in Yongyuso, the first Gok. In contrast, the letters in Yongsangugok have an antiquated, cursive-Yija style but because they were engraved relatively lightly, serious wear and damage occurred. In terms of location, Gabsagugok was built around Ganseongjang adjacent to the 5th Gok while Yongsangugok was set up around the 5th Gok, Hwangnyongam. Meanwhile, the important motif which forms the background of Gabsagugok seemingly highlights the geographic identity of Mt. Gyeryong using the dragon and the chicken as themes. It also appears to symbolize the principles of Juyeok focusing on Kan of the Eight Trigrams for divination; this requires an in-depth study for confirmation. The main motif and theme of Yongsangugok is the dragon. It infuses the builder's intentions in Sangsinri Valley by communicating with nature through a story of a dragon's life from birth to ascension. It is assumed that he tried to use this story to express his hope for restoring the national spirit and reconstructing the country.

• MISULJARYO - National Museum of Korea Art Journal
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• v.97
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• pp.69-86
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• 2020

Haenghaeng Ilgi (Diary of a Royal Trip) (koo 7152; hereafter Ilgi) in the collection of the National Museum of Korea provides an account of Crown Prince Sado's incognito royal visit to Pyeongyang in the fourth month of 1761. Ilgi was written by Ham Daeil, a low-ranking military officer in Pyeongyang. Ham was singled out for praise by Crown Prince Sado (1735-1762) and served the prince from the seventh day of the fourth month to the first day of the fifth month. Ilgi documented the deeds of Crown Prince Sado during this period and provides detailed information on his incognito visit to Pyeongyang, which was not officially recorded. Ilgi shows characteristics differentiating it from many other diaries. For example, the quality of the paper, neat handwriting, and well-organized sentences differ from those of common diaries. These distinctions indicate that Ilgi is closer to an official document than a private diary written by an individual. Since Ilgi records Crown Prince Sado's incognito visit to Pyeongyang, is only vaguely known otherwise, its contents need to be examined in terms of whether or not they are fully factual. As the first step in such verification, It is traced that Ham Daeil's family history which turned out to match what is written in Ilgi. Moreover, Ilgi mentions about Prince Crown Sado's writing a piece of calligraphy, and a matching piece of calligraphy written by Sado still survives today. It can be confirmed that the contents of Ilgi are factual in at least these regards. However, although Crown Prince Sado was known to have met people from various social classes during his visit to Pyeongyang, Ilgi focuses only on Crown Prince Sado and Ham Daeil. This suggests the possibility that the surviving version of Ilgi may be an edited condensation based on original texts containing more complete information on Crown Prince Sado's visit. Ilgi is presumed to have been produced during the generation of Ham Jeonghui, a son of Ham Daeil. The dates of birth and death of Ham Daeil fall during the reign of King Yeongjo. It is unlikely that any records regarding Crown Prince Sado would have been published while King Yeongjo, who was hostile to Sado, ruled the country. Ilgi also provides strong evidence that Ham Jeonghui presented the subsequent king, King Jeongjo, with the calligraphy by Crown Prince Sado and the diary. It is unclear if the book Ham Jeonghui presented him was the same as the extant version of Ilgi. Nonetheless, considering the situation at the time when King Jeongjo was pursuing several projects to honor his father Sado, it is highly probable that Ilgi was produced during the reign of King Jeongjo. The periods of presenting the calligraphy and the diary respectively overlapped with the production of Hyeollyungwon, the royal tomb of Crown Prince Sado, and Sado's sixtieth birthday. Therefore, is it considered reasonable that Ham Jeonghui produced Ilgi to promote his own social ambitions.

• (The) Research of the performance art and culture
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• no.32
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• pp.489-524
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• 2016

This study examines the birth of a genre, the $Sekky{\bar{o}}bushi$ Misemono Opera, focusing on how it accepted and modernized Katarimono $Sekky{\bar{o}}bushi$. Unlike earlier studies, it argues that Terayama was clearly different from other first-generation Angura artists, in that he rebirthed the medieval story $Sekky{\bar{o}}bushi$ as a modern Misemono Opera. Shintokumaru (1978) was directed by Terayama $Sh{\bar{u}}ji$, a member of the first generation of Japan's 1960s Angura Theatre Movement. It takes as its subject the Katarimono $Sekky{\bar{o}}bushi$ Shintokumaru, a story set to music that can be considered an example of the modern heritage of East Asian storytelling. $Sekky{\bar{o}}$ Shintokumaru is set in Tennoji, Japan. The title character Shintoku develops leprosy as a result of his stepmother's curse and is saved through his fiancee Otohime's devoted love and the spiritual power of the Bodhisattva Avalokitesvara. In this work, Terayama combined the narrative style of $Sekky{\bar{o}}bushi$ with J.A. Caesar's shamanistic rock music and gave it the subtitle 'Misemono Opera by $Sekky{\bar{o}}bushi$'. He transforms its underlying theme, the principle of goddesses and their offspring in a medieval religious world and the modori (return) instinct, into a world of mother-son-incest. Also, the pedestrian revenge scene from $Sekky{\bar{o}}bushi$ is altered to represent Shintokumaru as a drag queen, wearing his stepmother's clothes and mask, and he unites sexually with Sensaku, his stepbrother, and ends up killing him. The play follows the cause and effect structure of $Sekky{\bar{o}}bushi$. The appearance of katarite, a storyteller, propelling the narrative throughout and Dr. Yanagida Kunio is significant as an example of the modern use of self-introduction as a narrative device and chorus. Terayama $Sh{\bar{u}}ji^{\prime}s$ memories of desperate childhood, especially the absence of his father and the Aomori air raids, are depicted and deepened in structure. However, seventeen years after Terayama's death, the version of the play directed by Ninagawa Yukio-based on a revised edition by Kishida Rio, who had been Terayama's writing partner since the play's premier-is the today the better-known version. All the theatrical elements implied by Terayama's subtitle were removed, and as a result, the Rio production misses the essence of the diverse experimental theatre of Terayama's theatre company, $Tenj{\bar{o}}$ Sajiki. Shintokumaru has the narrative structure characteristic of aphorism. That is, each part of the story can stand alone, but it is possible to combine all the parts organically.

• 기호학연구
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• no.54
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• pp.119-145
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• 2018

This paper attempts to divide the narrative into three levels and review the approach methodology to understand Princess Bari as a narrative. If the stratification of the narrative, the analysis of each levels, and the integrated approach to them are made, this can contribute to suggesting new directions and ways to understand and study Princess Bari. The story level of Princess Bari, the surface structure, is shaped by the space movement and the chronological sequential structure of the life task that started from the birth of the main character. This story shows how a woman who was denied her existence by her father as soon as she was born finds an ontological transformation and identities through a process. Especially, the journey of finding identity is mainly formed through the events that occur through the relationship with family members. This structure, which can be found in the narrative level, forms a deep structure with the oppositional paradigm of family members' conflict and reconciliation, life and death. The thought structure revealed in this story is the problem of life is the problem of family composition, and the problem of death is also the same. In response to how to look at the unified world of coexistence of life and death, this tradition group of myths makes a relationship with man and God. This story is mainly communicated in the Korean shamanistic ritual(Gut) that sent the dead to the afterlife. Although the shaman is the sender and the participants in the ritual are the receivers, the story is well known a message that does not have new information repeated in certain situations. In gut, the patrons and participants do not simply accept the narrative as a message, but accept themselves as codes for reconstructing their lives and behavior through autocommunication. By accepting the characters and events of as a homeomorphism relationship with their lives, people accept the everyday life as an integrated view of life and death, disjunction and communication, conflict and reconciliation, and the present viewpoint. It can not change the real world, but it changes the attitude of 'I' about life. And it is a change and transformation that can be achieved through personal communication like the transformation of Princess Bari into god in myth. Thus, Princess Bari shows that each meaning and function in the story level, composition level, and communication level is related to each other. In addition, the structure revealed by this narrative on three levels is also effective in revealing the collective consciousness and cultural system of the transmission group.

• (The) Research of the performance art and culture
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• no.34
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• pp.133-172
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• 2017

Mansuseongjeol was originally a term that referred to the birthday of Chinese emperors. In October 1897, when Emperor Gojong ascended the throne, his birthday could be called mansuseongjeol. The celebratory events for mansuseongjeol took place throughout various levels of society and regions. Various places and classes including government officials, foreigners, students, religious people, journalists, merchants, civic groups, and nation-wide open ports celebrated the birth of Emperor Gojong, and the classes celebrating the day became stronger and wider. In other words, in the sense that the celebration had the nature of being universal, national, and global in terms of classes, regions, and races, the event was distinguished. Due to such nature, celebrating culture proceeded in various ways and the imperial family provided donation. Celebration on mansuseongjeol extended to respect toward the emperor and patriotism so it contributed to strengthening the emperor's power and solidifying the unity of the people. Also, such an event was reported around the world through diplomats and reporters living in Korea, raising the national status of Korea in the world. However, after the breakout of Russo-Japanese War, Japan controlled the finance of the royal family, reduced the power of the emperor, and the celebration of mansuseongjeol was also reduced. Due to the suspension of royal family's financial support, crackdown from Japan, and the dethronement of Emperor Gojong, events for mansuseongjeol disappeared and only inside the palace, did formal celebrations continue centering on pro-Japan officials and Japanese people. The abdication of Emperor Gojong came with the collapse of Korean Empire and along with that, celebration of mansuseongjeol came to an end. In the circumstances, the culture of court banquet disintegrated, and the best performers of Korean Empire degenerated into mere entertainers. Though mansuseongjeol, the medium of expressing respect toward the emperor and patriotism, lost its status, it is significant that the cultural achievements, which were created during the process established with the support of the financial power of the royal family, serve as internal power that drove Korea's modern and contemporary cultural history.