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리소좀 축적 질환(Lysosomal storage disease)에서의 효소 치료
진동규,Jin, Dong-Gyu 대한유전성대사질환학회 2011 대한유전성대사질환학회지 Vol.11 No.1
최근 유전공학의 발달로 리소좀 축적 질환에서 효소 치료제가 개발되어 실제 치료에 사용되고 있다. 현재 효소 보충 치료가 가능한 리소좀 축적 질환에는 고셔병(Gaucher disease), 파브리병(Fabry disease), 폼페병(Pompe disease), 뮤코다당체침착병(Mucopolysaccharidosis, MPS) 1형, 2형, 6형이 있으며 비교적 안전하면서 증상 완화에도 효과적으로 보인다. 그러나 이미 진행이 된 증상에 대해서는 비가역적이므로 조기에 진단을 하여 치료를 시작하는 것이 중요하다. 효소 보충 치료의 장기간에 걸친 치료 효과에 대해서 지속적인 평가가 필요하며 무엇보다 뼈와 중추신경계에 대한 효과는 제한적이므로 이에 대한 새로운 치료법의 개발이 필요하다.
외상이 경추 후종인대 골화증 환자의 수술결과에 미치는 영향
진동규,진병호,조용은,윤도흠,김영수,Chin, Dong-Kyu,Jin, Byung-Ho,Cho, Yong-Eun,Yoon, Do-Heum,Kim, Young-Soo 대한신경외과학회 2000 Journal of Korean neurosurgical society Vol.29 No.7
Objectives : Most cases of cervical ossification of the posterior longitudinal ligament(OPLL) present with myelopathy or myeloradiculopathy, which sometimes is further complicated by minor trauma to the spinal cord. The main purpose of surgery in these patients is the alleviation symptoms but also as protection against further deterioration. The purpose of this study is to analyze the influence of trauma on the surgical outcome in patients with cervical OPLL. Methods : Over the past 13 years, we have operated on 123 patients with myelopathy associated with cevical OPLL. Among these, thirty patients had cervical cord injury associated with major or minor trauma and their clinical and radiographic data were reviewed retrosepctively. Results : As to cause of trauma, seventeen were related with traffic accident and 13 were related with minor slipping injury. The pre- and post-operative motor power were significantly weaker in the patients with trauma, but the degree of motor improvement was significantly higher(trauma group : $0.90{\pm}1.49$, non-trauma group : $0.41{\pm}0.80$). Conclusion : These results indicate that even indirect minor trauma to the cervical spine can cause irreversible changes to the spinal cord if this is associated with underlying cervical stenosis with OPLL. Although less favorable results may be anticipated in patients with trauma, operative decompression could improve motor power and protect further deterioration.
진동규,윤수한,최중언,이혜연,정인혁 대한신경외과학회 1994 Journal of Korean neurosurgical society Vol.23 No.6
One hundred human cerebral hemispheres from 50 cadevers were dissected for evaluation on the variation of the tentorial sinus. Tentorial sinuses were found in 43 hemispheres which revealed a total of 63 branches. Tentorial sinuses were observed bilaterally in 14 cadevers and unilaterally in 15, but were absent in 21 cadevers. We divided the draining site of the tentorial sinuses at the straingt sinus into 3 zones ; Zone 1, anterior one third of the straight sinus, into which 15 out of 63 tentorial sinuses were found to be drained, zone 2 which is the most prevalent site, middle one third of the straight sinus draining 27 out of 63 tentorial sinuses and zone 3, posterior one third of the straight sinus draining 21 out of 63 tentonal sinuses.