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김우중,곽재건,민준철,김웅한 대한흉부외과학회 2020 Journal of Chest Surgery (J Chest Surg) Vol.53 No.2
Insertion of an epicardial pacemaker is a useful treatment for pediatric patients with an abnormal heart rhythm. However, there are limitations and concerns when implanting epicardial pacemakers in infants and neonates due to their small body size. We report a patient who experienced rare complications after implantation of a permanent pacemaker.
Clinical and Genetic Spectrum of ATP1A3-Related Disorders in a Korean Pediatric Population
김우중,심영규,최선아,김수연,김헌민,황희,최지은,김기중,채종희,임병찬 대한신경과학회 2020 Journal of Clinical Neurology Vol.16 No.1
Background and Purpose The aim of this study was to expand the understanding of the genotype-phenotype spectrum of ATP1A3-related disorders and to evaluate the therapeutic effect of a ketogenic diet in patients with alternating hemiplegia of childhood (AHC). Methods The clinical information of 13 patients with ATP1A3 mutations was analyzed by performing retrospective chart reviews. Patients with the AHC phenotype who consented to ketogenic diet were included in the trial. Results Ten patients presented with the clinical phenotype of AHC, two patients presented with rapid-onset dystonia parkinsonism, and one patient presented with cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss. Two novel mutations of the AHC phenotype were identified: p.Ile363Thr and p.Asn743Ser. The clinical phenotypes of three mutations differed from those in previous reports: p.Arg597Pro, p.Thr769Pro, and p.Arg756Cys. One of the two patients who started a ketogenic diet experienced seizure provocation and so immediate stopped consuming the diet, while the other patient continued the ketogenic diet for 1 year, but this produced no clear benefit such as reduction of paroxysmal symptoms. Conclusions Our study is the first case series of ATP1A3-related disorders to be described in Korea and which further expands the understanding of its genotype-phenotype spectrum. A ketogenic diet showed no clear benefit for the patients with AHC.
김우중,Yu‑Seok Lee,Yeong‑Han Chun 대한전기학회 2020 Journal of Electrical Engineering & Technology Vol.15 No.1
In Korean power system, 376 MW of battery energy storage system (BESS) functions as primary control reserve. The current total requirement of primary control reserve is 750 MW, which is procured by 376 MW of BESS and 374 MW of conventional generators. The BESS installed in the Korean power system can supply its energy for 10 min after loss of generators. If all the energy of the BESS is supplied during the system frequency restoration, this can cause a shortage in primary control reserve. Therefore, a new approach for reserve procurement is required to cope with the shortage in primary control reserve in case the second contingency might happen. Pumped storage hydro generators are good candidates for the additional reserve sources. In this paper, we propose a methodology for procuring additional primary control reserve considering their availability varying in real time and droop characteristics. In the case study, the efect of the proposed methodology is verifed through a dynamic simulation