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이홍진 대한신경과학회 2004 대한신경과학회지 Vol.22 No.1
Diseases of inborn errors of metabolism (IEMs) are very rare but the overall prevalence of IEMs is not low, and in the United States, about 5~10% of admitted patients have some genetic predispositions. Clinical manifestations of IEMs are very diverse, but most frequent manifestations are neurological symptoms and signs. IEMs in Korea have been underestimated because of prejudice, underdevelopment of diagnostic tools and ignorance. The Korean Pediatric Society has done a retrospective study in order to know the relative incidence of IEMs in 2001. All hospitals with over 100 beds participated in the study. The most frequent disease was Wilson disease (201 cases for 10 years) followed by phenylketonuria (98 cases for 10 years) and Hunters disease (69 cases for 10 years). Disorders of mineral metabolism were the most frequently diagnosed disease groups (252 cases for 10 years) followed by organic acidopathies (220 cases), aminoacidopathies (139 cases), mucopolysaccharidosis (131 cases), disorders of carbohydrate metabolism (84 cases), sphingolipidosis (69 cases), urea cycle disorders (39 cases), peroxisomal disorders (27 cases), porphyrias (16 cases), disorders of purine and pyrimidine metabolism (14 cases), disorders of membrane transport (13 cases), fatty acid oxidation disorders (9 cases), oligosaccharidosis (2 cases), and mucolipidosis (1 case). Clearly, Koreans are not protected from IEMs and a systematic approach is needed to make diagnosis more easy and accurate. J Korean Neurol Assoc 22(1):1~10, 2004
이홍진,Lee, Hong Jin 대한유전성대사질환학회 2014 대한유전성대사질환학회지 Vol.14 No.1
Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis of many of these diseases, if diagnosed early enough before irreversible damage occurs. This makes it essential that the practicing pediatrician, especially neonatologists be familliar with the clinical presentations and systematic approaches of these disorders. Characteristic clinical presentations, methods of systematic approach and typing of various disorders is discussed in this review. The signs of neurological dysfunctions of many IEMs manifesting in the neonatal period is very nonspecific, such as poor feeding, poor sucking, apnea or tachypnea, vomiting, hypertonia, hypotonia, seizure, letharginess, consciousness change and coma. Many other non-metabolic severe disorders of neonatal period such as neonatal sepsis and intracerebral hemorrhage share these nonspecific symptoms. Hyperammonemia, metabolic acidosis, ketosis and hyperlatic acidemia are observed in many of these conditions but there are exceptions in which conditions all basal laboratory tests are normal, such as NKH, sulfite oxidase deficiency and peroxisomal disorders. According to the results of basal laboratory tests, IEMs in the neonatal period can be categorized in to 6 types. Grouping of IEMs into 6 types will make confirmatory tests and early emergency treatment more efficient.
한국형 사이클로트론(KOTRON-13)을 이용한 $[^{11}C]CO_2$ 생산과 다양한 $^{11}C$-표지 방사성의약품 생산 적용
이홍진,박준형,문병석,이인원,이병철,김상은,Lee, Hong Jin,Park, Jun Hyung,Moon, Byung Seok,Lee, In Won,Lee, Byung Chul,Kim, Sang Eun 대한핵의학기술학회 2012 핵의학 기술 Vol.16 No.2
최근 늘어나는 [$^{18}F$]FDG-PET 검사 증대와 더불어 새로운 방사성의약품으로 [$^{11}C$]아세테이트 검사가 신설되고 다양한 연구용 $^{11}C$-표지 방사성의약품 이용이 증대되고 있다. 본 연구에서는 성공리에 수행한 한국형 사이클로트론의 $^{11}C$-표적시스템을 이용하여, $[^{11}C]CO_2$ 생산 최적화 및 임상에서 사용가능한 $^{11}C$-표지 방사성의약품 생산 적용 연구를 수행하였다. Purpose : The KOTRON-13 cyclotron was developed and installed in regional cyclotron centers to produce short-lifetime medical radioisotopes. However, this cyclotron has limited capacity to produce $^{11}C$ so far. In present study, we developed an effective $^{11}C$ target system combining with fluorine-18 target and applied to the production of various $^{11}C$ radiopharmaceuticals. Materials and Methods : To develop the optimal $^{11}C$ target system and effective its cooling system, we designed the $^{11}C$ target system by Stopping and Range of Ions in Matter (SRIM) simulation program and considered the cavity pressure during irradiation at target grid. In this investigation, we modified target materials, cavity shapes and the position of cooling system in $^{11}C$ target and then evaluated $[^{11}C]CO_2$ production at different beam currents, thickness of the target foil, oxygen content of nitrogen gas and target gas loading pressure. Also, we evaluate the production of several $^{11}C$ radiopharmaceuticals such as [$^{11}C$]PIB, [$^{11}C$]DASB, and [$^{11}C$]Clozapine. Results : $[^{11}C]CO_2$ was produced about 74 GBq for 30min irradiation at 60 ${\mu}A$ of beam current as following conditions: thickness of the target foil: 19 nm HAVAR, oxygen content of nitrogen: under 50 ppb, target gas loading pressure: 24 bar. Additionally, the cooling system was stable to produce $[^{11}C]CO_2$ at high beam current. The radiochemical yields of [$^{11}C$]PIB, [$^{11}C$]DASB, and [$^{11}C$]Clozapine showed about 26-38% with over 127 GBq/umol of specific activity. Conclusion : The carbon-11 target system in the KOTRON-13 cyclotron was successfully developed and showed stable production of $[^{11}C]CO_2$. These results showed that our $^{11}C$ target system will be compatible with other commercial system for the routine $^{11}C$ radiopharmaceuticals production in the KOTRON-13 cyclotron.