난치성 소아 간질의 미주신경자극술 효과

강훈철,김흥동,황용순,박상근 대한간질학회 2003 대한간질학회지 Vol.7 No.2

The smart EV charging system based on the big data analysis of the power consumption patterns

강훈철,강기범,안현권,이승현,안태효,좌정우 한국인터넷방송통신학회 2017 International Journal of Internet, Broadcasting an Vol.9 No.2

The high costs of electric vehicle supply equipment (EVSE) and installation are currently a stumbling block to the proliferation of electric vehicles (EVs). The cost-effective solutions are needed to support the expansion of charging infrastructure. In this paper, we develope EV charging system based on the big data analysis of the power consumption patterns. The developed EV charging system is consisted of the smart EV outlet, gateways, powergates, the big data management system, and mobile applications. The smart EV outlet is designed to low costs of equipment and installation by replacing the existing 220V outlet. We can connect the smart EV outlet to household appliances. Z-wave technology is used in the smart EV outlet to provide the EV power usage to users using Apps. The smart EV outlet provides 220V EV charging and therefore, we can restore vehicle driving range during overnight and work hours.

Lennox-Gastaut 증후군으로 발현된 3-Methylcrotonyl-CoA Carboxylase 결핍증 1례

강훈철,한유석,이홍진,김흥동,Kang, Hoon Chul,Han, Yu Sok,Lee, Hong Jin,Kim, Heung Dong 대한유전성대사질환학회 2004 대한유전성대사질환학회지 Vol.4 No.1

3-Methylcrotonyl-CoA carboxylase (MCC) is a biotin-dependent enzyme involved in leucine metabolism. We describe a patient with MCC deficiency who manifested with Reye syndrome-like illness with status epilepticus, metabolic acidosis, hypoglycemia, hyperammonemia, elevated liver enzymes and neurologic impairments after the viral gastroenteritis and then, has suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with leucine restriction diet and supplementation of biotin and carnitine but was not so effective. He has suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairement.

난치성 소아 간질에서의 수술적 치료

강훈철,김흥동,황용순,박상근 대한간질학회 2002 대한간질학회지 Vol.6 No.2

신생아 발작의 조기 발견

강훈철 한국모자보건학회 2005 한국모자보건학회 학술대회 연제집 Vol.2005 No.2

Disease-specific pluripotent stem cells

강훈철 대한소아청소년과학회 2010 Clinical and Experimental Pediatrics (CEP) Vol.53 No.8

Induced pluripotent stem (iPS) cells are generated by epigenetic reprogramming of somatic cells through the exogenous expression of transcription factors. Recently, the generation of iPS cells from patients with a variety of genetic diseases was found to likely have a major impact on regenerative medicine, because these cells self-renew indefinitely in culture while retaining the capacity to differentiate into any cell type in the body, thereby enabling disease investigation and drug development. This review focuses on the current state of iPS cell technology and discusses the potential applications of these cells for disease modeling; drug discovery; and eventually, cell replacement therapy.

유소아 발달과 발달 장애

강훈철,김흥동 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.3

중증 영아형 근간대성 간질의 임상적 고찰

강훈철,Dambajamts, Oyungerel,김흥동 대한간질학회 2002 대한간질학회지 Vol.6 No.2

전사인자를 이용한 질병특이 유도전능줄기세포

강훈철(Hoon-Chul Kang) 대한소아신경학회 2010 대한소아신경학회지 Vol.18 No.1

The recent years have seen great advances in reversal of programming of differentiated somatic cells towards pluripotency by methods not involving nuclear transfer. Although differentiation of cells involves complex genetic and epigenetic changes, it is now possible to generate cells with many properties of pluripotent embryonic stem cells by transduction of differentiated cells with only four transcription factors: Oct3/4, Sox2, Klf4 and c-Myc. In advance, the protocol for improving the efficiency of generating induced pluripotent stem(iPS) cells and establishing clinically applicable cells has been developed. Recently, the generation of iPS cells from patients with a variety of genetic diseases with either Mendelian or complex inheritance including X-linked adrenoleukodystrophy, amyotrophic lateral sclerosis, Duchenne and Becker muscular dystrophy, Parkinson's disease, Huntington's disease, Gaucher disease type III, Down syndrome is also possible. Such disease-specific iPS cells offer an unprecedented opportunity to recapitulate both normal and pathologic human tissue formation in vitro, thereby enabling disease investigation, drug development and cell therapy.

난치성 소아 간질 환자에서 Topiramate 효과와 안전성

강훈철(Hoon Chul Kang),고창준(Chang Jun Coe) 대한소아신경학회 2001 대한소아신경학회지 Vol.9 No.2

목 적 : Topiramate는 개발 당시 성인의 난치성 부분 간질에 효과가 있는 보조 약물로 알려졌지만 소아에 있어서 부분 간질에서 뿐 아니라 다양한 간질 형태와 간질 증후군에서 약물의 효과가 있음이 보고되고 있다. 이에 저자들은 topiarmate가 임상에서 사용된 지난 2년간의 임상 경험을 후향적으로 분석하여 난치성 소아 간질에서의 효과와 부작용에 관하여 확인하고자 본 연구를 실시하였다. 방 법: 본 연구는 1999년도 2월부터 2001년 2월까지 연세대학교 세브란스병원 소아신경과를 내원한 환자 중, 기존의 항간질제를 두가지 이상 최고용량으로 투여하여도 간질이 조절되지 않는 환자로서 topiramate를 6개월 이상 투약한 114명을 대상으로 하였다. 대상 환아의 나이 2-18세였고, 간질 유병 기간은 2년 이상으로, 기존 약물에 난치성을 보이는 모든 간질 형태와 간질 증후군을 대상으로 하였다. 환자와 보호자와의 상담과 전화설문 그리고 의무 기록 분석을 시행하였으며 통계처리는 기술적 방법과 qui-square의 선형대 선형분석(linear to linear association)을 이용하여 비교 분석하였다. 결 과: 1) 환아 114명 중 남자는 74명, 여자는 40명이였으며 평균 연령이 11.0세였다. 간질 형태 및 간질 증후군은 복합 부분 간질 46명, 이차성 전신 간질을 동반하는 부분간질 24명, 전신 강직 간대성 간질 26명, 탈력 간질 6명, 근간대성 간질 4명, Lennox-Gastaut 증후군 8명으로 부분 간질이 70명(61.4%), 전신 간질이 44(38.6%)명이었다. 평균 간질 유병 기간은 6년 8개월이었으며, 투약 전 한달 간 평균 간질 발생 횟수는 67.8회였고, 최빈값은 16회였다. Topiramate 평균 투약 기간은 14개월이었고, topiramate 초기 투여 용량은 1 ㎎/㎏/day였으며, 환아의 반응에 따라 2주 간격으로 1-3 ㎎/㎏/day 속도로 증량하였다. 최대 투여 용량은 5-9 ㎎/㎏/day로 유지하였다. 2) 대상 환아 114명 중 투약 전 한달에 비해 투약 시작 후 최근 4개월간 간질 발작 횟수가 50% 이상 감소한 경우는 60명으로 52.6%였으며, 12명(10.5%)에서는 100% 간질 발작이 소실되었다. 간질 형태 및 간질 증후군별로 복합 부분 간질과 이차성 전신 간질을 동반하는 부분 간질, 전신 강직 간대성 간질, 탈력 간질의 경우는 효과가 높은 경향을 확인하였다. 이에 비해 Lennox-Gastaut 증후군에서는 효과가 낮은 경향이었으며 근간대성 간질 4명은 의미 있는 간질 발생 횟수의 감소가 없었다. 병인에 따른 topiramate의 효과는 부분 간질과 전신 강직 간대성 간질에서 특발성과 잠재성 또는 증후성이 통계적으로 의미 있는 효과의 차이는 없었다. 114명의 환아 중 48명 42.1%에서 약물 부작용으로 졸리움, 보챔과 식욕 감소를 호소하였는데 약물 투여를 중단할 정도로 심각한 경우는 없었다. 결 론 : 이상의 결과를 볼 때 topiramate는 다양한 간질 형태와 간질 증후군에 효과가 있었으며 특별성 간질 뿐 아니라 잠재성 혹은 증후성 간질에서도 효과를 보였으나, Lennox-Gastaut 증후군과 근간대성 간질은 부분 간질이나 전신 강직 간대성 간질에 비해 효과가 낮은 경향을 확인할 수 있었다. 약물의 안전성은 기존의 보고와 유사한 신경계 부작용과 식욕 감퇴가 관찰되었으나 약물 중간을 요할 만큼 심각한 부작용은 없었다. Purpose : This is a clinical study to evaluate the efficacy and safety profiles of topiramate up to 9 ㎎/㎏/day as adjunctive therapy in various refractory childhood epilepsies including Lennox-Gastaut syndrome in Korea. Methods : Subjects were selected accorting to the following criteria in various childhood epileptic children, refractory seizure even the patients had been on maximal doses of more than at least 2 other anticonvulants, any type of seizure or epileptic syndrome of childhood, those who had been on topiramate more than 6 months. This is a clinical retrospective study performed for 2 years from Feb. 1999 to Feb. 2001 at the Severance hospital pediatric neurological clinic. The topiramate was given the patients as add-on therapy and we observed the seizure frequency, type of seizure, development of any adverse effects, and the difference of result between the idiopathic and symptomatic or cryptogenic group. Of the 238 patients at our clinic on topiramate for 2 years, 114 patients with refractory were enrolled in this study. Those data were analysed with descriptive methods and qui-squares to evaluate the efficacy and safety profiles of topiramate. Results: 60 patients out of 114(52.6%) manifested reduction of seizure occurrence more than 50%, while 12 patients(10.5%) were seizure free. 39 patients(55.7%) out of 70 with partial seizure reduced seizure occurrence more than 50% by add-on therapy and 7 patients(10.0%) had no further seizures. In the group of generalized tonic clonic seizure, 15 patients(57.7%) reduced seizure over 50% and 5(19.2%) had no seizures with add-on therapy. Of patients with Lennox-Gastaut syndrome, 25.0% showed a greater than 50% reduction but no patient proved to be seizure-free. 4 patients with myoclonic seizure did not respond to add-on therapy. There was no difference of seizure frequency both idiopathic and symptomatic or cryptogenic group of partial and generalized tonic clonic seizure. The number of patients manifesting adverse reactions was 48(42.1%) patients in order of frequency, the reactions were somnolence in 26, irritability in 16 and anorexia in 6 patients. Conclusion : Topiramate is believed to be an effectsive, safe anticonvulsant when used as adjunctive therapy for various refractory childhood epilepsies. However, patients with Lennox-Gastaut syndrome and myoclonic seizures were reacted less favorably to topiramate.