강훈철 교수
Hoon‐Chul Kang
연세대학교 소아청소년과 · 의학
연구실 소개
강훈철 교수의 연구실은 주로 난치성 소아 간질 환자를 대상으로 한 ketogenetic 다이어트(ketogenic diet)의 임상적 효과와 안전성에 중점을 두고 있습니다. 특히 고지방·저탄수화물 다이어트가 뇌질환, 특히 미토콘드리아 기능 이상을 동반한 간질 환자에게 미치는 영향을 체계적으로 분석하며, 다양한 변형 다이어트(기본 케토제닉 다이어트, 수정 아티킨스 다이어트 등)의 비교 연구를 진행하고 있습니다. 또한 유전적 원인에 기반한 조기 발병 간질의 진단을 높이기 위해 NGS 기반 표적 유전자 시퀀싱 기술을 활용한 유전자 진단 연구도 함께 수행하고 있습니다.
연구 현황
연구 성과 추이
표시된 성과는 수집된 데이터 기준으로 산출되며, 일부 차이가 있을 수 있습니다.
주요 논문
15PURPOSE: This study was undertaken to evaluate the exact limitations of the ketogenic diet (KD) and to collect data on the prevention and management of its risks. METHODS: Patients (129) who were on the KD from July 1995 to October 2001 at our epilepsy center were assessed in the study. Early-onset (within 4 weeks of the commencement of the KD until stabilization) and late-onset complications (occurring after 4 weeks) were reviewed. RESULTS: The most common early-onset complication was dehydrati
PURPOSE: To evaluate the clinical efficacy and safety of the ketogenic diet (KD) for patients with intractable childhood epilepsy and mitochondrial respiratory chain (RC) complex defects. METHODS: A retrospective analysis evaluated outcomes in 14 children with intractable epilepsy and RC complex defects who were treated with the classic KD involving a 4:1 lipid to nonlipid ratio (% by weight), but without an initial fast and fluid restriction. Outcome measures included seizure frequency, electro
PURPOSE: We wished to evaluate the efficacy and safety of the ketogenic diet (KD), and we also evaluated the prognosis of the patients after successful discontinuation of the diet in infants, children, and adolescents with refractory epilepsy. METHODS: This was a retrospective study of epilepsy patients treated with the KD during 1995 through 2003 at the Korean multicenters. Outcome measures included seizure frequency, electroencephalography (EEG), adverse reactions, and antiepileptic drug (AED)
OBJECTIVE: We aimed to compare the efficacy, safety, and tolerability of a modified Atkins diet (MAD) with the classic ketogenic diet (KD) for the treatment of intractable childhood epilepsy. METHODS: From March 2011 to March 2014, 104 patients aged 1-18 years who had refractory epilepsy were randomly assigned to each diet group (ClinicalTrials.gov, number NCT2100501). A seizure diary record was used to compare seizure frequencies with the baseline prediet seizure frequency at the third and sixt
PURPOSE: To evaluate the efficacy, safety and tolerability of a modified Atkins diet in intractable childhood epilepsy. METHODS: Fourteen children with epilepsy were treated prospectively with a modified Atkins diet. Outcome measures included seizure frequency, adverse reactions and tolerability to the diet; blood beta-hydroxybutyrate and urine ketones were also measured. RESULTS: Six months after diet initiation, seven (50%) remained on the diet, five (36%) had >50% seizure reduction, and three
BACKGROUND: We intended to evaluate diagnostic utility of a targeted gene sequencing by using next generation sequencing (NGS) panel in patients with intractable early-onset epilepsy (EOE) and find the efficient analytical step for increasing the diagnosis rate. METHODS: We assessed 74 patients with EOE whose seizures started before 3 years of age using a customized NGS panel that included 172 genes. Single nucleotide variants (SNVs) and exonic and chromosomal copy number variations (CNVs) were
PURPOSE: To evaluate the cognitive and behavioral effects of topiramate (TPM) versus carbamazepine (CBZ) using efficacious doses of each drug as monotherapy for children with benign rolandic epilepsy. METHODS: A multicenter, randomized, open-label, observer-blinded, parallel-group clinical trial was conducted. TPM was introduced at a dose of 12.5 mg/day with the minimum target dose of 50 mg/day in patients <30 kg and 75 mg/day in patients >30 kg over 4 weeks. CBZ was started at a dose of 10 mg/k
PURPOSE: To compare the prognoses between short-term (8 months) and conventional long-term (> 2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD). METHODS: Of 40 patients who achieved seizure-free outcomes and showed improvement in hypsarrhythmic patterns within 6 months of the KD, with a 3:1 fat to nonfat ratio as an add-on treatment, 16 patients were randomized into the short-term trial group and the diet was tapered throughout
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