The fate of spargana inoculated into the cat brain and sequential changes of anti - sparganum IgG antibody levels in the cerebrospinal fluid

Kyu-Chang Wang(왕규창),Sun Huh(허선),Sung-Tae Hong(홍성태),Jong-Yil Chai(채종일),Kil Soo Choi(최길수),Soon-Hyung Lee(이순형) 대한기생충학열대의학회 1990 The Korean Journal of Parasitology Vol.28 No.1

림프구성 누두신경뇌하수체염 : 증례 보고 및 문헌고찰

손영제,왕규창,최기영,지제근,조병규,Son, Young-Je,Wang, Kyu-Chang,Choe, Gheeyoung,Chi, Je Keun,Cho, Byung-Kyu 대한신경외과학회 2000 Journal of Korean neurosurgical society Vol.29 No.6

Lymphocytic infundibuloneurohypophysitis is a rare inflammatory disorder, which may be caused by autoimmune response, affecting the infundibulum and the posterior lobe of the pituitary gland. The authors present a 15-year-old man with central diabetes insipidus, whose sellar magnetic resonance imaging (MRI) showed a focal thickening of the infundibulum and loss of high signal of the normal neurohypophysis on T1-weighted images. The histopathological diagnosis was lymphocytic infundibuloneurohypophysitis. When a patient presents with central diabetes insipidus and infundibular thickening on brain MRI, this entity should be included in the differential diagnosis though it is very rare. The significance of this entity in treatment is that it is basically not a surgical one, and surgery is needed only for tissue diagnosis. Symptomatic treatment and observation make it regress spontaneously.

소아의 원발성 악성 연수막 흑색종 - 증 례 보 고 -

손영제,왕규창,김연미,신상훈,지제근,조병규,Son, Young-Je,Wang, Kyu-Chang,Kim, Youn Mee,Shin, Sang-Hoon,Chi, Je G.,Cho, Byung-Kyu 대한신경외과학회 2000 Journal of Korean neurosurgical society Vol.29 No.9

Primary malignant leptomeningeal melanoma is very rare, accounting for less than 0.1 percent of tumors in the central nervous system. Patients with primary intracranial malignant melanoma tend to be younger than metastatic intracranial melanoma, most commonly in the fourth decade of life. This tumor is extremely rare and the biologic behavior is aggressive especially in children. The authors report a case of primary malignant leptomeningeal melanoma in a twelve-year-old boy which was initially diagnosed as meningitis. On autopsy, associated extensive leptomeningeal melanosis was confirmed and believed to be the origin of the tumor. This case emphasizes the pattern of clinical presentation and the significance of leptomeningeal melanosis in primary leptomeningeal melanoma.

섬유형성이상 환자의 눈 소견

김남주,왕규창,김석화,손우길,황정민,Nam Ju Kim,Kyu Chang Wang,Suk Wha Kim,Woo Gil Son,Jeong-Min Hwang 대한안과학회 2006 대한안과학회지 Vol.47 No.5

Purpose: Fibrous dysplasia is an uncommon disease that replaces normal bone tissue with fibrous and osteoid tissue. We evaluated patients with fibrous dysplasia involving orbital bones to identify the ocular characteristic findings. Methods: We recruited 6 patients with fibrous dysplasia of the craniofacial bone. Best corrected visual acuity and facial asymmetry were measured. Slit lamp biomicroscopy, tonometry, strabismus examination, and funduscopy were performed. Results: Strabismus, exophthalmos and optic neuropathy were found in 5, 4, and 2 patients, respectively. One patient underwent a strabismus surgery. One patient had McCune-Albright syndrome, exhibiting a caf?-au-lait spot of the skin and precocious puberty. All 6 patients had facial asymmetry and higher superior orbital rims of the affected side in 5 patients. Conclusions: Ophthalmologic examination may be mandatory for patients with fibrous dysplasia involving the orbit, especially to prevent optic neuropathy. If optic atrophy, strabismus or exophthalmos is found in patients with facial asymmetry, the possibility of fibrous dysplasia must be considered.

전적출후 재발한 소아 두개인두종의 재발인자에 대한 분석

김승기,왕규창,정영섭,심기범,조병규,Kim, Seung-Ki,Wang, Kyu-Chang,Chung, Young Seob,Sim, Ki-Bum,Cho, Byung-Kyu 대한신경외과학회 2001 Journal of Korean neurosurgical society Vol.30 No.1

Objectives : A substantial number of craniopharyngiomas recur despite gross total excision. The purpose of our study was to investigate pattern of recurrence and to verify prognostic factors for recurrence after gross total excision of craniopharyngiomas in children. Methods : A series of 36 patients with craniopharyngiomas were reviewed. All patients had undergone gross total excision and none of them received radiotherapy after initial surgery. Fifteen were girls and twenty-one were boys, with a mean age of 7.3 years(range, one to 15 years). The mean follow-up period was 52 months(range, one to 149 months). Recurrence was noted in 14 patients within 83 months(mean 31.4 months). Results : The overall three-year recurrence free survival rate was 65%, and the five-year recurrence-free survival rate was 55%. Regular neuroimaging follow-up at six to 12-month intervals detected tumor recurrence of a smaller size before symptoms developed(p<0.05). At the first surgical procedure, the optic nerve/chiasm(n=23) was the most common adhesion site. The most frequent sites of recurrence were the optic nerve/chiasm(n=6) and the pitiutary fossa(n=6). Tumor location was the single significant clinical predictor of recurrence. The five-year recurrence-free survival rate was 39% for those who had an intrasellar tumor component and 81% for those who did not (p<0.05). Conclusion : Craniopharyngiomas with intrasellar components should be followed cautiously and regular followup of patients should be emphasized, even when the tumors are totally resected.

신경성장촉진 인자가 인간 배아줄기세포 유래 도파민 분비 신경세포형성에 미치는 영향

이금실,김은영,신현아,조황윤,왕규창,김용식,이훈택,정길생,이원돈,박세필,임진호,Lee, Keum-Sil,Kim, Eun-Young,Shin, Hyun-Ah,Cho, Hwang-Yoon,Wang, Kyu-Chang,Kim, Yong-Sik,Lee, Hoon-Taek,Chung, Kil-Saeng,Lee, Won-Don,Park, Se-Pill,Lim, Jin 대한생식의학회 2004 Clinical and Experimental Reproductive Medicine Vol.31 No.1

Objective: This study was to examine the in vitro neural cell differentiation patterns of human embryonic stem (hES) cells following treatment of various neurotrophic factors [basic fibroblast growth factor (bFGF), retinoic acid (RA), brain derived neurotrophic factor (BDNF) and transforming growth factor (TGF)-$\alpha$], particulary in dopaminergic neuron formation. Methods: The hES cells were induced to differentiate by bFGF and RA. Group I) In bFGF induction method, embryoid bodies (EBs, for 4 days) derived from hES were plated onto gelatin dish, selected for 8 days in ITSFn medium and expanded at the presence of bFGF (10 ng/ml) for another 6 days followed by a final differentiation in N2 medium for 7, 14 and 21 days. Group II) For RA induction, EBs were exposed of RA ($10^{-6}M$) for 4 days and allowed to differentiate in N2 medium for 7, 14 and 21 days. Group III) To examine the effects of additional neurotrophic factors, bFGF or RA induced cells were exposed to either BDNF (10 ng/ml) or TGF-$\alpha$ (10 ng/ml) during the 21 days of final differentiation. Neuron differentiation and dopamine secretion were examined by indirect immunocytochemistry and HPLC, respectively. Results: The bFGF or RA treated hES cells were resulted in similar neural cell differentiation patterns at the terminal differentiation stage, specifically, 75% neurons and 11% glial cells. Additionally, treatment of hES cells with BDNF or TGF-$\alpha$ during the terminal differentiation stage led to significantly increased tyrosine hydroxylase (TH) expression of a dopaminergic neuron marker, compared to control (p<0.05). In contrast, no effect was observed on the rate of mature neuron (NF-200) or glutamic acid decarboxylase-positive neurons. Immunocytochemistry and HPLC analyses revealed the higher levels of TH expression (20.3%) and dopamine secretion (265.5 $\pm$ 62.8 pmol/mg) in bFGF and TGF-sequentially treated hES cells than those in $\alpha$ RA or BDNF treated hES cells. Conclusion: These results indicate that the generation of dopamine secretory neurons from in vitro differentiated hES cells can be improved by TGF-$\alpha$ addition in the bFGF induction protocol.

소아 모야모야병에서 뇌확률지도를 이용한 수술전후 혈역학적 변화 분석

이호영 ( Ho Young Lee ),이재성 ( Jae Sung Lee ),김승기 ( Seung Ki Kim ),왕규창 ( Kyu Chang Wang ),조병규 ( Byung Kyu Cho ),정준기 ( June Key Chung ),이명철 ( Myung Chul Lee ),이동수 ( Dong Soo Lee ) 대한핵의학회 2008 핵의학 분자영상 Vol.42 No.3

척수수막류 환아에서 MTHFR(methylenetetrahydrofolate reductase) 유전자 내 677C→T 돌연변이 빈도에 대한 연구

이백희(Baeck Hee Lee),왕규창(Kyu Chang Wang),정해일(Hae Il Cheong) 대한소아신경학회 1998 대한소아신경학회지 Vol.6 No.1

목 적 : 임신 4주전과 임신 첫 4주 동안 엽산의 공급으로 신경관 결손의 발생을 50%이상 예방할 수 있었다는 보고를 통해 엽산이 신경관 폐쇄에 중요한 역할을 한다는 것이 알려졌다. MTHFR은 엽산대사에 중요한 효소이다. MTHFR gene의 위치와 돌연변이가 밝혀짐에 따라 신경관 결손이 있는 환아와 그들 가족에서 MTHFR variant 빈도가 증가되어 있다는 보고들이 있었다. 본 실험은 MTHFR 유전자 내 677c-lT 돌연변이의 빈도를 측정하여 신경관 결손군에서 대조군에 비해 돌연변이의 빈도가 증가되었는지 알아보기 위해 시행하였다. 방 법 : 1997년 1월부터 1997년 12월까지 서울대학교 어린이병원과 단국대학교 소아과에서 신경관 결손(척수수막류)으로 진단 받고 추적 관찰중인 환아 21명과 환아부모 40명, 형제자매 7명을 대상으로 하였으며, 대조군으로 정상아를 1회 이상 분만한 경험이 있는 연령 25세부터 50세 사이의 성인 25명과 신경관 결손을 갖고 있지 않는 16세미만의 아동 18명을 대상으로 하였다. 혈액 채취는 EDTA tube에 전혈 4-5cc를 뽑아 gDNA를 추출하였다. 추출된 DNA를 template로 MTHFR 유전자 내 염기 서열 643부터 841 사이의 염기쌍을 증폭하였다. PCR은 통상의 방법으로 35 cycle시행하였다. PCR 산물은 butanol 추출 혹은 QIAquick spin column(Qiagen, CA, USA)을 이용하여 정제한 뒤 Hinfl로 처리하고 그 절단 결과를 8% polyacrylamide gel에서 전기영동 후 ethium bromide 염색으로 확인하였다. 결 과 : 대상 인원 총 111명 중 신경관 결손 가족군은 68명이었고 대조군은 43명이었다. 신경관 결손 가족군 중 환아의 어머니는 30명, 아버지 10명 환아는 21명이었고 자매 형제는 7명이었다. genotype에 따라 두 군을 비교하면 신경관 결손군 중 정상인 경우(-/-)는 32명으로 47.0%이고 heterozygote인 경우(+/-) 28명으로 41.2%이며 homozygote(+/+) 빈도수는 8명으로 11.8% 이었다. 대조군인 경우 정상은 11명으로 25.6%, heterozygote인 경우 25명으로 58.1%이며 homozygote인 경우는 7명으로 16.3%를 차지하였다(Table 1). 신경관 결손군에서 환아 21명 중 homozygote인 경우는 2명으로 9.5% 어머니는 30명 중 4명으로 13.3%, 아버지는 10명 중 1명으로 10%이며 형제 자매는 7명 중 1명으로 10.3%의 빈도를 보였다. 결 론: 1) 신경관 결손군에서 MTHFR variant polymorphism 의 빈도가 대조군에 비해 증가되어 있지 않았다. 돌연변이 빈도는 인종적 차이가 있을 수 있으나 정확한 결론을 얻기 위해 좀더 많은 대상이 참여되어야 할 것으로 생각된다. 2) Hyperhomocysteinemia를 일으킬 수 있는 MTHFR locus 이외의 또 다른 polymorphism과 MTHFR 이외에 homocysteine을 증가시킬 수 있는 엽산과 vitamin Bl2에 관련된 효소들에 대한 연구를 계속하여야 할 것으로 사료된다. Purpose : There is evidence that folic acid given before and during the first 4 weeks of pregnancy can prevent more than 50% of neural tube defect. It suggested that folic acid play a great role when a neural tube closes. Methylenetetrahydrofolate reductase (MTHFR) is an important enzyme in folate metabolism. Since the gene locus and mutation of MTHFR were identified, increased frequency of MTHFR variant was reported in neural tube defect. We studied the frequency of the 677C→T mutation in the MTHFR gene to determine whether this MTHFR gene variant is more common in persons with neural tube defect and their families compared to a control population. Methods : The study group consisted of 21 patients with meningomyelocele, 40 their parents and 7 siblings who were managed in Seoul National University College of Medicine and Dankook University Hospital during the period from Jan. 1997 to Dec. 1997. The control group consisted of 25 parents aged between 25 to 50 years who had given birth to, at least, one normal infant and 18 children under 16 years without neural tube defect. DNA was extracted from peripheral blood and a segment of the MTHFR gene was amplified using PCR technique. And treated with restriction enzyme, Hinfl. The restriction pattern was analyzed. Results : The frequency of the three genotypes were as follows: normal(-/-), 47.0% ; heterozygote (+/-), 41.2%; and homozygote(+/+), 11.8% in neural tube defect group and (-/-), 25.6%; (+/-), 58.1%; (+/+), 16.3% in control group. The MTHFR gene variant was present 9.5% of those with meningomyelocele, 13.3% of their mothers 10% of their fathers, and 10.3% of siblings. Conclusion : 1)There is no increase of the frequency of MTHFR variant polymorphism in neural tube defect compared to control group. These observations indicate that while there may be racial differences in the mutation frequency, expanded studies involving larger numbers of subjects are required. 2) To elucidate the role of various genetic factors influencing on homocysteine levels and vitamin nutrition, research on other genetic variants, such as folic acid and vitamin Bl2-related enzymes and receptors, are recommended.

가족성 해면혈관종에서 염색체 7q CCM1 염기서열의 가족간 연관성 분석

심기범(Ki-Bum Sim),이창섭(Chang Sub Lee),김승기(Seung-Ki Kim),왕규창(Kyu-Chang Wang),김영임(Young-Im Kim),조병규(Byung-Kyu Cho) 한국독성학회 2005 Toxicological Research Vol.21 No.2

Although the pathogenesis of cerebral cavernous malformation (CCM) is unknown, a familial predisposition has been recognized, with up to 55% of patients having an affected relatives. Genetic linkage studies have recently mapped a gene causing CCM to a segment of the long arm of chromosome 7 (7q). We report herein a genetic linkage analysis conducted on a Korean three generation family with CCM. It’s first report in Korean family. A Korean family in which one member had undergone surgery for intracerebral hematoma (ICH) and confirmed the CCM, was evaluated. They were examined clinically (n=18) and by magnetic resonance (MR) imaging (n=10). Polymorphic markers (D7S1813, D7S1789) spanning the CCM1 locus on 7q were genotyped by the polymerase chain reaction and analysis of linkage was performed in this family (n=17). Six had multiple lesions on brain MR image, one of them being symptomatic, and five were asymptomatic. Seven remaining members were asymptomatic and refused MR image study. One had died of ICH from presumed CCM. Analysis of the pedigree was consistent with an autosomal dominant pattern of inheritance. All affected patients were linked to CCM1. Linkage to CCM1 can account for inheritance of CCM in this family.They had some striking features with a low clinical penetrance and the presence of multiple lesions. These findings have implications for genetic testing of this disorder and represent an important step toward identification of the gene responsible for the pathogenesis of this disease.

Telomerase 과발현을 이용한 신경줄기세포유래 세포치료제의 확립

홍석호 ( Seok Ho Hong ),백선하 ( Sun Ha Paek ),왕규창 ( Kyu Chang Wang ) 한국조직공학과 재생의학회 2005 조직공학과 재생의학 Vol.2 No.3

Since the evidences that neural stem cells (NSCs), either endogenous or exogenous, actively participate in regenerative process after destructive pathology in the (CNS) were given, various therapeutic strategies of cell-based regeneration has been introduced and attempted. While neurally-committed progenitor cells or NSCs are theoretically suitable for repairing CNS after injury, the acquirement of sufficient cells remains to be overcome for clinical applications. We propose clonal expansion of NSCs in vitro using telomerase overexpression as a practical means for obtaining NSCs for therapeutic purpose, and discuss their usefulness and limitations from the clinical viewpoint.