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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.
박성원,Park, Seong-Won 한국방위산업진흥회 1994 國防과 技術 Vol.- No.181
항공우주산업과 첨단무기체계의 발달로 전차의 중요성이 간과되어 가는 것이 지금의 현실이다 그러나 지상전력에 있어 전차의 중요성은 더 말할 나위가 없다. 이것은 지난 걸프전에서 소련의 T-72 전차를 모든 면에서 압도한 M1의 위력에서 잘 나타났다 이에 세계 각국에서 추진되는 전차의 발전 추세를 살펴보는 것도 의미있을 것이다. 이 글은 국과연에서 정기적으로 발해하는 "국방기술동향" 제1호에 게재된 내용이다
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.