• 한국임상약학회지
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• 제26권2호
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• pp.121-127
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• 2016

Objective: Until now, there is minimal number of research for overall domestic status of orphan drug use in Korea. The purpose of this study is to identify the list of orphan drugs available in Korea and to understand the status of orphan drug usage in tertiary Hospitals and rare incurable disease Hospital. Methods: We made domestic orphan drug lists based on available orphan drugs in Korea. Based on this lists, we conducted e-mail survey from August, 2014 to September, 2014 to identify domestic status of orphan drug usage including the availability and management of orphan drugs. Results: There are three hundred and eighteen orphan drugs (184 ingredients) registered in Ministry of Food and Drug Safety. Among the three hundred and eighteen orphan drugs, Two hundred and twenty-eight drugs (102 ingredients) were selected. Information on each item was collected and documented with generic and brand names, manufacturers, wholesalers, indications, FDA approval status and insurance coverage. Forty-three tertiary hospitals and thirty-two rare incurable hospitals responded to the survey questionnaire (57.3%). According to the survey result, the antineoplastics and immunomodulating agents group has the highest percentage (40%) usage in the hospital. Of fortythree tertiary hospitals, thirteen hospitals manage orphan drugs separately (30.2%). Based on the reply, most of the healthcare professionals commented the drug information related to efficacy and safety including medication counseling of orphan drugs is insufficient. Conclusion: Through this study we anticipate providing an understanding of orphan drug usage status in Korea. We found the limited resources to the information on orphan drugs and this information requires updating on a regular basis. This can be the basis for further studies about preparing drug information, educational resources for rare disease patients.

• 한국식품영양과학회지
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• 제14권2호
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• pp.99-107
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• 1985

This study was carried out to investigate the nutritional and physical status of children, aged 11 and 12 years, living in Haenam Koon (living at home) and in Mokpo City (living in an orphan asylum) located in Chonnam during the period of August 2 nd to 9 th in 1984. The mean values of height, chestgirth, arm circumference and head circumference between both groups of living in the rural area and in the city orphan asylum were not statistically different and similar to Korean Growth Standard. Exceptionally, the values of height and weight of male living at home in the rural area were lower than Korean Growth Standard (p<0.01). The weight of male living in the city orphan asylum was higher han that of male living at home in the rural area (p<0.01), and the skin fold thickness of female living at home was higher than that of male living at home (p <0.01). The physical indices of children living in the city orphan asylum were more or less higher than those of living at home in the rural area, but the relative weight of male was only significant (p<0.05). The animal protein intake was $8.4{\sim}8.6%$ of total food intake of living at home in the rural area and 6.2% in living in the city orphan asylum. The intake of legumes of children living in the city orphan asylum was more two times than that of living at home in the rural area, but overall fruit intake of living at home was more about two times than that of living in the city orphan asylum. The average intake of all nutrients except thiamine and niacin was lower than RDA. Especially the calcium intake of children in both groups was the lowest of all nutrients ($28.9{\sim}40.6%$ of RDA). Children living at home in the rural area had significantly higher intake of fat and ascorbic acid than children living in the city orphan asylum (p<0.01).

• 한국임상약학회지
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• 제16권2호
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• pp.107-112
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• 2006

This study was aimed to investigate the current status and expectations of orphan drugs in Korea. The Korea Orphan Drug Center was established to supply many medicines for the patients with rare diseases. Among the medicines supplied by the Center, the number designated as the orphan drugs by the KFDA is quite few. However, a few of medicines are not under circulation even if the items are designated as the orphan drugs. Neoplasm-related medicines, infectious and parasitic disease-related medicines, endocrine, nutritional and metabolic disease-related medicines are the ones circulated most. There are several unapproved drugs among the medicines supplied by the Center. It's because the director of the Center can import the goods without a process to getting an approval from the KFDA. The Korea Orphan Drug Center has contributed to the selection of the medicines for treating the rare disease. On the contrary, some problems remain in the supply process. The safety and effectiveness of the medicines supplied by the Center are not guaranteed. So far, rare diseases have no specific legal definition, and therefore are only referred to in terms of the population of patients, which prevent from establishing the range of medicines. The introduction of Special Access Program or Access to Unapproved Therapeutic Goods will be the solution of these problems. In addition, it is another solution to keep intimate relations with the Rare and Intractable Disease Center and the Medicine Safety Information Center which will be open soon.

• BMB Reports
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• 제31권5호
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• pp.419-426
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• 1998

The nuclear hormone receptor superfamily currently includes approximately equal numbers of conventional receptors and orphan receptors, which do not have known ligands. Here, we review recent progress from this laboratory on three orphans, two of which are moving from orphan to conventional receptor status. Perhaps the most unusual is CAR, which is a constitutive transactivator in the absence of ligands but becomes transcriptionally inactive in the presence of its ligands, which are androgen metabolites. The response of CAR to its ligands is thus opposite to that of the conventional receptor paradigm. RIP14 (also known as FXR) is activated by both all-trans retinoic acid and a synthetic retinoid previously thought to specifically target the retinoic acid receptors (RARs), and thus appears to be a novel retinoid receptor. Finally, SHP is a novel orphan that lacks a DNA binding domain and interacts with a number of other receptor superfamily members. While it generally inhibits its targets, including CAR, the retinoid X receptor (RXR), and the estrogen receptor (ER), it stimulates transactivation by the orphan SF-1.

• 한국식품영양과학회지
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• 제14권2호
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• pp.108-116
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• 1985

This study was designed to investigate the nutritional anemic state and hematological findings of children, aged 11 and 12 years, living in Haenam Koon (living at home) and in Mokpo City (living in an orphan asylum) located in Chonnam. The mean red blood cell number of male was higher than female in both groups of living in the rural area and in the city orphan asylum (p<0.01), but the white blood cell count was not significantly different. The levels of average hemoglobin, hematocrit and mean corpuscular hemoglobin concentration of the children in both groups were similar, and 19.8% of children living at home in the rural area and 32.1% of children living in the city orphan asylum were anemic. The mean levels of serum total protein, albumin and A/G ratio in children of both group were not statistically different, and 18.4% of children living at home in the rural area and 13.2% of children living in the city orphan asylum were insufficient in the serum total protein value. The average serum cholesterol level of children living at home in the rural area was higher than that of children living in the city orphan asylum(p<0.01), and that of female living at home was higher than that of male (p<0.05). The mean levels of serum iron, total iron binding capacity and transferrin saturation of children living at home in the rural area were significantly higher than those of children living in the city orphan asylum (p<0.01).

• 대한간호학회지
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• 제7권2호
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• pp.117-127
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• 1977

Depressive status of the adolescence in 437 normal, 416 orphan, 133 physically handicapped and 109 deaf-mute children were compared with the Self- Rating Depression Scale developed by Zung, from April 26 to July 4, 1976. The subjects were divided into two groups, early adolescence for age of 10 to 14 and ate adolescence of age of 15 to 18. The results were as follows : 1. The depression score was higher in late adolesce co than in early adolescence except deaf - mutes, 2. The depression score was not different significantly between the sexes in all groups. 3. The depression score was significantly higher in the group of orphan, physically handicapped and deaf - mute children then in the normal. 4. The depression score was significantly higher in the orphans than in the physically handicapped children. It showed tendency to be higher in the deaf - mutes than in the physically handicapped children. 5. In comparison with the rank order of the Self - Rating Depression Scale items, normal group was inclined to have psychological symptoms in higher rank order, but orphan and deaf - mute group was inclined to have somatic symptoms in higher rank order.

• 의료법학
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• 제21권1호
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• pp.223-259
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• 2020

COVID-19가 순식간에 세계적으로 퍼져나가면서 이 바이러스에 대한 치료제 및 백신에 대한 연구 개발 이슈가 뜨겁다. 그 중에서도 렘데시비르(Remdesivir)가 가장 먼저 눈에 띄는 치료효과를 나타내며 본격적인 임상시험에 돌입하였고, 각 국가들은 긴급 승인을 통해 해당 의약품의 사용을 허가하고 있다. 그런데 렘데시비르의 개발사인 주식회사 길리어드는 렘데시비르를 희귀의약품으로 품목허가 신청하여 시민단체들로부터 많은 질타를 받았다. 그 이유는 희귀의약품으로 신약이 품목허가를 취득하는 경우 7년의 독점적 지위를 부여받을 수 있기 때문이다. 희귀의약품에게 장기간 동안 독점적 지위를 부여하는 것은 희귀질환으로 고생하는 환자들에게 치료기회를 부여하기 위해 제약회사에게 개발 동기를 부여하는 정책적 취지로부터 나온 것으로, 감염병 치료제에 적용하기에는 적절하지 않았기 때문이다. 본 논문은 이러한 정책적 의도를 바탕으로 의약품에 독점적 지위를 부여하고 있는 법령에 대하여 미국, 유럽, 일본을 상대로 비교법적 고찰을 통해 국내 제도의 문제점 및 개선 방향에 대해 살펴보는 내용을 담고 있다. 국내 제도는 독점적 지위 부여 방식에 있어 법령에 명시적인 조항을 두고 있지 않고, 재심사제도를 활용하여 우회적으로 독점적 지위를 부여하고 있다는 근본적인 문제를 갖고 있다. 이와 더불어 희귀의약품의 경우 「희귀질환관리법」과 「의약품의 품목허가·신고 심사 규정」에서 중복적으로 재심사기간을 규정하고 있다는 점, 상대적으로 독점기간을 길게 부여하고 있음에도 불구하고 사정변경 발생 시 독점적 지위를 회수할 수 있는 견제 조항이 존재하지 않는다는 점 등이 보완되어야 할 부분으로 보인다. 생물의약품의 경우에는 국내에는 우회적으로라도 의약품의 독점적 지위를 부여할 수 있는 규정이 존재하지 않는다. 생물의약품의 경우 특허를 받기 어렵다는 점에서, 독점권을 부여하는 것이 의약품 개발을 유도하는 큰 동기가 될 수 있음에도 이러한 조항이 없다는 점은 국내 법제의 아쉬운 점이라 볼 수 있다. 미국의 경우 최초의 동등생물의약품에도 1년의 독점기간을 부여한다는 점에 비추어 볼 때, 국내 법제는 생물의약품에 독점적 지위를 부여하는 것에 대하여 상당히 엄격하게 제한하고 있다고 해석된다. 이는 추후 국내 제약사의 생물의약품 개발 의지를 저하시킬 수 있다는 점에서, 또한 국제 규제의 조화를 맞출 필요가 있다는 점에서 국내 제도의 개선의 필요성이 인정된다 할 것이다. COVID-19의 발현을 기회로 삼아, 의약품에 독점권을 부여하고 있는 국내 제도가 가지고 있는 문제점에 대하여 다시 한 번 살펴보고, 통일된 법적 근거를 마련할 수 있도록 관련 법제에 대한 전반적인 개정이 이루어 질 것을 기대해보는 바이다.

• 대한소아치과학회지
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• 제8권1호
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• pp.77-88
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• 1981

The purpose of this study was to make a comprehensive study & evaluation of the oral status of mental retarded children. The auther examined intraorally 486 (male; 311, female;175) mental retarded children. The result was as follows; (General mental retarded children means the children who live in their parent's home, & orphan mental retarded children means the children who live in orphanage.) 1. The dft rate was 31.6% in general mental retarded children (G.m.r.c.) & 20.7% in orphan mental retarded children (O. m. r. c.). The dft index was 3.73 in G.m.r.c. & 2.15 in O.m.r.c. 2. The DMFT rate was 24.6% in female G.m.r.c., 16.7% in male G.m.r.c., 12.7% in female O.m.r.c., 8.4% in male O.m.r.c. The DMFT index was 4.94 in female G.m.r.c., 4.01 in male G.m.r.c., 1.40 in male O.m.r.c., 2.75 in female O.m.r.c. 3. The malocclusion prevalence was 57.3%. the class I malocclusion was 14.2% Class II malocclusion 19.3%, Class III malocclusion 23.5%. The children with Down's syndrome had 60.0% of class III malocclusion prevalence. 4. The dental calculus index was 1.97 in male O.m.r.c., 1.81 in female O.m.r.c., 1.30 in male G.m.r.e., 1.13 in female G.m.r.c. 5. The dental plaque index was 3.06 in female G.m.r.c., 3.00 in male Gm.r.e. 2.70 in male O.m.r,c., 2.32 in female O.m.r.c.

• 한국식생활문화학회지
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• 제6권4호
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• pp.413-419
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• 1991

This study aims to evaluate the degree of malnutrition of children by three nutritional indicators-Weight for Age, Hight for Age, Weight for Height. The results are as follows; The average height and weight of children are much lower than Korea average height and weight. In terms of Weigh for Age, 20-40% of the samples belongs to below-2 standard deviation from the mean of reference group. 90% of the samples falls into the region below the mean. The standard deviation score indicates negative sign for eighty to ninety percent of the samples. The malnutrition of the samples is also confirmed in terms of Height for Age. In terms of Weight for Height, the mean of the samples is not significantly different from that of the reference group. Tests of the nutritional status under basis of three nutritional indicators show that approximately 50% of the samples had suffered from malnutrition and 17% of the samples have suffered from malnutrition.

• 예술인문사회 융합 멀티미디어 논문지
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• 제8권10호
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• pp.471-479
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• 2018

This paper on the socioeconomic role of One Dollar Campaign project initiated by Rwanda Diaspora in the reconstruction of the country after 1994 genocide against Tutsi tends to show how Rwanda Diaspora contributes to the socioeconomic development of their home country. The 1994 genocide against Tutsi in Rwanda has destroyed the country and the Rwandan society; and rebuilding the trust, unity, hope and socioeconomic status of genocide survivors in particular and of all Rwandans in general had to engage all Rwandans wherever they were. The focus of this paper is to assess the change on socioeconomic status of the beneficiaries of the one dollar campaign project initiated by Rwanda Diaspora which aimed to help genocide survivor orphans to have a shelter. The results showed that the amount of money contributed by Rwanda Diaspora for this project is of paramount importance as it exerts a positive impact on genocide orphan survivors' daily life, change of their socioeconomic status and to the country's development in general.