• Korean Journal of Clinical Pharmacy
• /
• v.26 no.2
• /
• pp.121-127
• /
• 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.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.14 no.2
• /
• pp.99-107
• /
• 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).

• Korean Journal of Clinical Pharmacy
• /
• v.16 no.2
• /
• pp.107-112
• /
• 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
• /
• v.31 no.5
• /
• pp.419-426
• /
• 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.

• Journal of the Korean Society of Food Science and Nutrition
• /
• v.14 no.2
• /
• pp.108-116
• /
• 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).

• Journal of Korean Academy of Nursing
• /
• v.7 no.2
• /
• pp.117-127
• /
• 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.

• The Korean Society of Law and Medicine
• /
• v.21 no.1
• /
• pp.223-259
• /
• 2020

With COVID-19 spreading rapidly around the world, research and development issues on treatments and vaccines for the virus are of high interest. Among them, Remdesivir was the first to show noticeable therapeutic effects and began clinical trials, with each country authorizing the use of the drug through emergency approval. However, Gilead Co., Ltd., the developer of Remdesivir, received a lot of criticism from civic groups for submitting the application for the marketing authorization as an orphan drug. This is because when a new drug got a marketing authorization as an orphan drug could be granted an exclusive status for seven year. The long-term exclusive status of an orphan drug comes from the policy purpose of motivating pharmaceutical companies to develop treatment opportunities for patients suffering from rare diseases, which was not appropriate to apply to infectious disease treatments. This paper provides a review of the problems and improvement directions of the domestic system through comparative legal consideration against the United States, Europe and Japan for the statutes which give exclusive status to medicines. The domestic system has a fundamental problem that it does not have explicit provisions in the statute in the manner of granting exclusive status, and that it uses the review system to give it exclusive status indirectly. In addition, in the case of orphan drugs, the "Rare Diseases Management Act" and the "Regulations on Examination of Items Permission and Reporting of Drugs" provide overlapping review periods, and despite the relatively long monopoly period, there seems to be no check clause to recover exclusive status in the event of a change in circumstances. Given that biopharmaceuticals are difficult to obtain patents, the lack of such provisions is a pity of domestic legislation, although granting exclusive rights may be a great motivation to induce drug development. In the United States, given that the first biosimilar also has a one-year monopoly period, it can be interpreted that domestic legislation is quite strictly limited to granting exclusive status to biopharmaceuticals. The need for improvement of the domestic system will be recognized in that it could undermine local pharmaceutical companies' willingness to develop biopharmaceuticals in the future, and in that it is also necessary to harmonize international regulations. Taking advantage of the emergence of COVID-19 as an opportunity, we look again at the problems of the domestic system that grants exclusive rights to medicines and hope that an overall revision of the relevant legislation will be made to establish a unified legal basis.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.8 no.1
• /
• pp.77-88
• /
• 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.

• Journal of the Korean Society of Food Culture
• /
• v.6 no.4
• /
• pp.413-419
• /
• 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.

• Asia-pacific Journal of Multimedia Services Convergent with Art, Humanities, and Sociology
• /
• v.8 no.10
• /
• pp.471-479
• /
• 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.