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안면 비대칭 환자에서 Cone Beam Computed Tomography를 이용한 하악골 해부학적 변이의 분석
박성원,오승환,이재인,Park, Seong-Won,Oh, Sung-Hwan,Lee, Jae-In 대한악안면성형재건외과학회 2012 Maxillofacial Plastic Reconstructive Surgery Vol.34 No.1
Purpose: The study was performed to compare patients with anatomical variations in facial asymmetry with patients in the normal range using cone-beam computed tomography (CBCT) and to take the preoperative condition into consideration in the case of a sagittal split ramus osteotomy (SSRO). Methods: The study was conducted on 46 adult patients composed of 2 subdivided groups, an asymmetry group (n=26) and a symmetry group (n=20). The asymmetry group was divided between patients with hemimandibular hyperplasia (HH, n=8) and hemimandibular elongation (HE, n=18). Using cross-sectional computed tomography images, the thickness of cancelleous bone in the buccal area of the mandible, thickness of buccal cortex in the buccal aspect of the mandible, thickness of cancellous bone in the inferior aspect of the mandible, thickness of buccal cortex in the inferior aspect of the mandible, and cross-sectional surface area of the mandible were measured. Results: In the asymmetry group, the cross-sectional area of the mandible including the inferior alveolar nerve positioned on the affected side was significantly different from the symmetry group. Thickness of cancelleous bone in the buccal aspect of the mandible, thickness of cancelleous bone in the inferior aspect of the mandible, and cross-sectional surface area of the mandible in the affected site of hemimandibular hyperplasia was significantly smaller than in the symmetry group. Conclusion: The inferior alveolar nerve runs lower and in a more buccal direction and shows a smaller cross-sectional surface of the mandible in the hemimandibular hyperplasia patients with asymmetry.
박성원,김성용,김유동,Park, Sung-Won,Kim, Seong-Yong,Kim, You-Dong 대한자원환경지질학회 2014 자원환경지질 Vol.47 No.3
분쟁광물이란 특히, 콩고민주공화국(DRC)의 동부지역과 같이 분쟁지역에서 채굴되는 광물에 대하여 부르는 용어이다. 보통 분쟁광물이란 주석, 콜탄(컬럼바이트-탄탈라이트), 중석, 금과 같은 광물로 주로 콩고민주공화국(DRC)의 동부지역에서 채굴되고 있는 광물로서, 이러한 분쟁광물은 여러 가지 전자기기의 제조에 사용되고 있다. 콩고민주공화국(DRC)과 이웃 나라에서 분쟁을 종식시키기 위해 자금원이 되어왔던 분쟁광물의 개발이나 교역의 근원을 차단시키고 분쟁국에 있어서 무장그룹에 의한 분쟁을 억제시킬 필요가 있다. 미국 증권거래위원회(Securities and Exchange Commission, SEC)는 2012년 8월 22일 금융규제개혁법 제 1502조에 근거하는 규칙을 채택하였는데, 이 법에 따라 미국 기업들은 증권거래위원회에 분쟁광물을 이용하여 제품을 생산할 경우 분쟁광물의 원산지에 대한 보고를 하여야 한다. 이 법은 분쟁 광물의 원산지를 추적하여 특히, 분쟁 광물의 자금원이 되고 있는 콩고 민주공화국을 필두로 분쟁국에 있어서의 무장세력의 직 간접 자금원을 끊어 분쟁을 억제하는 것을 그 목적으로 하고 있다. Conflict minerals refer to minerals mined in conditions of armed conflict, especially as in the eastern provinces of the Democratic Republic of the Congo. The common conflict minerals are cassiterite, wolframite, coltan(columbite-tantalite ore), and gold, which are mined and extracted from the Eastern Congo. These minerals are essentially used in the manufacture of a variety of devices, including consumer electronics. To end the violent conflict in the Democratic Republic of the Congo (DRC) and in surrounding countries, it is necessary to block the supply route of conflict minerals which has been partially financed by the exploitation and trade of conflict minerals. The Dodd-Frank Wall Street Reform and Consumer Protection Act, passed into law in July 2010 and it contains requirements that U.S. companies report to the Securities and Exchange Commission(SEC) on the origin of conflict minerals and show due diligence of OECD. The goal of the act is to cut direct and indirect funding of armed groups engaged in conflict.
박성원,손영배,김세화,조성윤,지선태,진동규,Park, Seong-Won,Son, Yeong-Bae,Kim, Se-Hwa,Jo, Seong-Yun,Ji, Seon-Tae,Jin, Dong-Gyu 대한유전성대사질환학회 2010 대한유전성대사질환학회지 Vol.10 No.1
Enzyme replacement of therapy (ERT) is one of the most promising therapeutic strategies for the treatment of lysosomal storage disorders. ERT is available in three types of Mucopolysaccharidosis (MPS): for MPS I (Aludrazyme$^{(R)}$), MPS II (Elaprase$^{(R)}$) and MPS VI (Naglazyme$^{(R)}$) patients who are over 5 years old. But recently, early diagnosis can be done by expert clinicians and even in prenatal case. We describe the case of ERT under 5 years old MPS patients. Up to June, 2010 in Samsung Medical Center, there are 6patients who were diagnosed as MPS and started ERT under 5 years old. 3 patients were MPS I, 3 patients were MPS II. 2 patient who was diagnosed as MPS I was female and others were male. Their age at diagnosis were 4 to 37month-old (4, 13, 16, 25, 27, 37 month-old) and they are now 9 to 60 month-old (9, 39, 32, 81, 60 month-old). The youngest patient was started ERT at 4 month-old and others were started at their 13 to 49 month-old (13, 29, 27, 28, 49 month-old). First manifested symptoms of patients were macrocephaly, kyphosis and coarse face appearance. Especially, in 2 of them, one was MPS I and the other was MPS II had elder brother with same disease. And the youngest one was diagnosed by the iduronate-2-sulfatase (IDS) gene analysis from chorionic villi sampling. His mother knew that she was a heterozygous carrier of IDS gene mutation because her younger brother died from MPS II. All of them confirmed as MPS by the enzyme assay in leukocytes and fibroblast skin culture. We started ERT with ${\alpha}$-L-iduronidase(Aldurazyme$^{(R)}$) to MPS I and did recombinant human iduronate-2-sulfatase (Elaprase$^{(R)}$) to MPS II patients as recommended dose as over 5 years old. But for MPS II patient who was 4 month old, we started ERT by recombinant human IDS (Elaprase$^{(R)}$) with reduced dose 0.1 mg/kg and increased dose every 2 weeks by 0.1mg/kg up to 0.5mg/kg IV infusion. During ERT, all patients had no adverse effects and the excretion of GAGs were decreased. We have evaluated other clinical symptoms such as liver/ spleen volume, heart function and neurologic evaluation. We describe a successful ERT to MPS I and MPS II patient under 5 years old without any adverse event. It indicates that ERT in young children are well tolerated and that it has several effects which may confer clinical benefits with long-term therapy.