Clinical Spectrum and Treatment Outcomes of Patients with Developmental and/or Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep

Nuri Tchah(Nuri Tchah ),Donghwa Yang(Donghwa Yang),Heung Dong Kim(Heung Dong Kim),Joon Soo Lee(Joon Soo Lee),Se Hee Kim(Se Hee Kim),Hoon-Chul Kang(Hoon-Chul Kang) 대한소아신경학회 2022 대한소아신경학회지 Vol.30 No.4

Purpose: Developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (D/EE-SWAS) is a spectrum of conditions characterized by various phenotypes of cognitive, linguistic, and behavioral regression associated with spike-and-wave activation in sleep. We aimed to investigate the phenotypic spectrum and treatment outcomes of pediatric patients with D/EE-SWAS. Methods: We retrospectively analyzed the medical records of pediatric patients diagnosed with D/EE-SWAS and treated at Severance Children’s Hospital from 2006 to 2022. We extracted information from their medical records on electroencephalography before and after treatment, types of treatment, seizure frequency, and developmental profiles. The primary outcome was reduction of the spike-wave index on electroencephalography after treatment. Results: Twenty-one patients with a median age of 5.3 years (interquartile range, 4.1 to 6.6) at diagnosis were included. Ten patients had delayed development. The patients received various anti-seizure medications. Fourteen received long-term, high-dose steroid therapy, 10 were placed on a ketogenic diet, four received intravenous steroid pulse therapy, and one each was treated with intravenous immunoglobulin and cannabidiol. The most effective treatments were steroid therapy and a ketogenic diet, which were also effective in reducing seizures and improving cognition. Side effects during treatment were transient and treatable. Conclusion: We described the clinical spectrum of pediatric patients with D/EE-SWAS. Steroid therapy and a ketogenic diet can be considered effective therapeutic options for patients with D/EE SWAS.

Expression profiles of miRNAs in human embryonic stem cells during hepatocyte differentiation.

Kim, Nury,Kim, Hyemin,Jung, Inkyung,Kim, Yeji,Kim, Dongsup,Han, Yong-Mahn Elsevier 2011 HEPATOLOGY RESEARCH Vol.41 No.2

<P>? Human embryonic stem cells (hESCs) are able to self-renew and differentiate into a variety of cell types. Although miRNAs have emerged as key regulators in the cellular process, a few studies have been reported about behaviors of miRNAs during differentiation of hESCs into a specialized cell type. Here, we demonstrate that different kinds of miRNAs may function in a lineage-specific manner during the differentiation of human embryonic stem cells (hESCs).</P>

Quality of Life in Food Allergy: Validation of the Korean Version of the Food Allergy Quality of Life Questionnaire Parent Form (K-FAQLQ-PF) and Risk Factor Analysis

Kim Sukyung,Kim Minji,Kim Jiwon,Park Boram,Min Nuri,Jung Minyoung,Yu Seoyoung,Lee Ji Young,Yoo Hye Won,Kim Hye-Young,Ahn Kangmo,Kim Jihyun 대한천식알레르기학회 2023 Allergy, Asthma & Immunology Research Vol.15 No.1

Purpose: This study aimed to evaluate the reliability and validity of the Korean version of the Food Allergy Quality of Life Questionnaire-Parent Form (K-FAQLQ-PF) and to identify clinical characteristics related to low quality of life (QoL) in Korean children with food allergy (FA). Methods: Parents of 0–12-year-old patients with FA were enrolled. The English version of FAQLQ-PF was translated into Korean. Construct validation was confirmed by the Food Allergy Independent Measure-Parent Form (FAIM-PF) and the Child Health Questionnaire Parent Form 28 (CHQ-PF28). Logistic regression analyses were used to evaluate associations between potential risk factors and QoL outcomes. Results: A total of 182 patients with a median age of 5.0 years were enrolled in the study. Cronbach’s α coefficient values indicating internal consistency were higher than 0.8. Intraclass correlation coefficient values for test-retest reliability were good for all age groups (r > 0.6). Total K-FAQLQ-PF scores were positively correlated with the FAIM-PF (r = 0.56, P < 0.05) and were negatively correlated with the parental impact-emotional domain in the CHQ-PF28 (r = −0.44, P < 0.05). In multivariable logistic regression analysis, low QoL was significantly associated with female sex (adjusted odds ratio [aOR], 2.07; 95% confidence interval [CI], 1.03–4.18), age ≥ 5 years (aOR, 2.84; 95% CI, 1.31–6.16), FA diagnosis before the age of 3 years (aOR, 3.96; 95% CI, 1.13–13.93), the presence of atopic dermatitis (aOR, 2.21; 95% CI, 1.07–4.57), and residence in non-metropolitan areas (aOR, 3.44; 95% CI, 1.73–6.85). Conclusions: According to parental perceptions, the K-FAQLQ-PF is a valid and reliable tool to assess psychosocial QoL in Korean children with FAs. Age, sex, residential area, and comorbid AD can affect the QoL of pediatric patients with FA.

위치 기반 서비스를 이용한 학내 정보 인공지능 챗봇 연구

김누리 ( Nuri Kim ),김유나 ( Yuna Kim ),김정선 ( Jeongsun Kim ),석영현 ( Yeonghyeon Suk ),김영종 ( Youngjong Kim ) 한국정보처리학회 2019 한국정보처리학회 학술대회논문집 Vol.26 No.1

위치 기반 서비스를 중점으로 교내의 각종 정보들을 제공하는 챗봇시스템을 구축한다. 교내 공지사항, 학사정보와 실시간 전화 연결 서비스 및 자연어 처리를 기반으로한 다양한 정보들을 제공한다.

Effect of ethanolamine passivation of ZnO nanoparticles in quantum dot light emitting diode structure

Noh, Kyeongchan,Kim, Minsu,Lee, Sang-Hyun,Yun, Hwang-Sik,Lim, Tae-Hyeon,Choi, Yeongho,Kim, Ki-Ju,Jiang, Yiran,Beom, Keonwon,Kim, Minju,Kim, Young-Gwang,Lee, Pilwoo,Oh, Nuri,Kim, Bong Hoon,Shin, Chansu Elsevier 2019 CURRENT APPLIED PHYSICS Vol.19 No.9

<P><B>Abstract</B></P> <P>The aging of ZnO nanoparticles in quantum dot light-emitting diode (QD-LED) structures was studied. Coarsening of as-synthesized ZnO nanoparticles is observed in both solution and thin film structures, which potentially deteriorates the performance of QD-LED devices over time. First, the temperature effect on ZnO coarsening was investigated, and it was revealed that aging of ZnO nanoparticles is faster at higher temperature due to a diffusion-controlled mechanism of nanoparticle coarsening. To observe aggregation of ZnO in the film state, the electron transporting part (ZnO/Al) of the QD-LED structure was prepared. The current density of a ZnO film and an electron-only device (QD/ZnO between two electrodes) was also measured. Resistance of the film increased as a function of aging time, which corresponded with observations of the ZnO film by optical microscopy. Aggregation of ZnO nanoparticles was directly measured by the root-mean-square value using atomic force microscopy. Ethanolamine (EA) stabilizer was added to the ZnO solution to disperse the ZnO nanoparticles without aggregation. The effect of EA on the surface passivation of the ZnO found to suppress pinhole formation, as revealed by scanning electron microscopy observations. Finally, the device lifetime was measured for QD-LEDs with EA-stabilized ZnO to understand the effect of ZnO aging on long-term QD-LED device operation.</P>

Visuomotor anomalies in achiasmatic mice expressing a transfer-defective Vax1 mutant

Min Kwang Wook,Kim Namsuk,Lee Jae Hoon,Sung Younghoon,Kim Museong,Lee Eun Jung,Kim Jong-Myeong,Kim Jae-Hyun,Lee Jaeyoung,Cho Wonjin,Yang Jee Myung,Kim Nury,Kim Jaehoon,Lee C. Justin,Park Young-Gyun,Le 생화학분자생물학회 2023 Experimental and molecular medicine Vol.55 No.-

In binocular animals that exhibit stereoscopic visual responses, the axons of retinal ganglion cells (RGCs) connect to brain areas bilaterally by forming a commissure called the optic chiasm (OC). Ventral anterior homeobox 1 (Vax1) contributes to the formation of the OC, acting endogenously in optic pathway cells and exogenously in growing RGC axons. Here, we generated Vax1AA/AA mice expressing the Vax1AA mutant, which is incapable of intercellular transfer. We found that RGC axons cannot take up Vax1AA protein from the Vax1AA/AA mouse optic stalk (OS) and grow slowly to arrive at the hypothalamus at a late stage. The RGC axons of Vax1AA/AA mice connect exclusively to ipsilateral brain areas after failing to access the midline, resulting in reduced visual acuity and abnormal oculomotor responses. Overall, our study provides physiological evidence for the necessity of intercellular transfer of Vax1 and the importance of the bilateral RGC axon projection in proper visuomotor responses.

Optogenetic toolkit reveals the role of Ca²⁺ sparklets in coordinated cell migration

Kim, Jin Man,Lee, Minji,Kim, Nury,Heo, Won Do National Academy of Sciences 2016 PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF Vol.113 No.21

<P>Cell migration is controlled by various Ca2+ signals. Local Ca2+ signals, in particular, have been identified as versatile modulators of cell migration because of their spatiotemporal diversity. However, little is known about how local Ca2+ signals coordinate between the front and rear regions in directionally migrating cells. Here, we elucidate the spatial role of local Ca2+ signals in directed cell migration through combinatorial application of an optogenetic toolkit. An optically guided cell migration approach revealed the existence of Ca2+ sparklets mediated by L-type voltage-dependent Ca2+ channels in the rear part of migrating cells. Notably, we found that this locally concentrated Ca2+ influx acts as an essential transducer in establishing a global front-to-rear increasing Ca2+ gradient. This asymmetrical Ca2+ gradient is crucial for maintaining front-rear morphological polarity by restricting spontaneous lamellipodia formation in the rear part of migrating cells. Collectively, our findings demonstrate a clear link between local Ca2+ sparklets and front-rear coordination during directed cell migration.</P>

Lineage-specific Expression of miR-200 Family in Human Embryonic Stem Cells during In Vitro Differentiation

Kim, Yeji,Kim, Nury,Park, Sang-Wook,Kim, Hyemin,Park, Han-Jin,Han, Yong-Mahn Korean Society for Stem Cell Research 2017 International journal of stem cells Vol.10 No.1

<P>Although microRNAs have emerged as key regulators in diverse cellular processes, the roles of microRNAs are poorly understood in human embryonic stem cells (hESCs) during differentiation into specialized cell types. In this study, we used a microRNA array with 799 human microRNA probes to examine the expression profiles of microRNAs in hESCs during differentiation into endodermal and mesodermal lineages <I>in vitro</I>. Among the microRNAs analyzed, 7 and 20 microRNAs were enriched in the developmental process of hESCs into mesodermal and endodermal lineages, respectively. In particular, the expression levels of miR-200 family, which is known to regulate the epithelial to mesenchymal transition (EMT), gradually increased in hESCs during differentiation into hepatocytes while they gradually decreased during differentiation into vascular endothelial cells. Downregulation of ZEB1, a direct target of miR-200 family, and E-CADHERIN, a target protein of ZEB1, was observed in hESCs during differentiation into endodermal and mesodermal lineages, respectively. These results indicate that miR-200 family has an important role in determining the cell fate between endodermal and mesodermal lineages from the pluripotent state.</P>

ELK3-GATA3 axis modulates MDA-MB-231 metastasis by regulating cell-cell adhesion-related genes

Kim, Kwang-Soo,Kim, Jiewan,Oh, Nuri,Kim, Mi-Young,Park, Kyung-Soon Elsevier 2018 Biochemical and biophysical research communication Vol.498 No.3

<P><B>Abstract</B></P> <P>GATA3 is a master regulator that drives mammary epithelial cell differentiation, and the suppression of GATA3 expression is associated with the development of aggressive breast cancer. However, the mechanism through which GATA3 loss drives cancer development is poorly understood. Previously, we reported that ELK3 suppression in MDA-MB-231 (ELK3 KD) resulted in the reprogramming of these cells from a basal to luminal subtype, which was associated with the induction of GATA3 expression, and that the ELK3-GATA3 axis orchestrated the metastatic characteristics of MDA-MB-231. Here, we show that GATA3 suppression in ELK3 knockdown MDA-MB-231 cells (ELK3/GATA3 DKD) restores the metastatic ability comparably to that of control MDA-MB-231 cells, even though the epithelial cell morphology and TGF-β signaling of ELK3 KD are not recovered in ELK3/GATA3 DKD. The expression of <I>E</I>-cadherin and tight junctional proteins, including occludin, claudin and ZO-1, which is activated in ELK3 KD, is suppressed in ELK3/GATA3 DKD. These results reveal the possibility that the ELK3-GATA3 axis determines the metastatic characteristics of MDA-MB-231 by regulating the expression of cell-cell adhesion factors.</P> <P><B>Highlights</B></P> <P> <UL> <LI> GATA3 suppression in ELK3 knockdown MDA-MB-231 restores metastatic characteristics both <I>in vitro</I> and <I>in vivo.</I> </LI> <LI> ELK3-GATA3 axis regulates metastasis of MDA-MB-231 independent of TGF-β signaling. </LI> <LI> ELK3-GATA3 axis regulates metastasis of MDA-MB-231 through the regulation of adhesion and tight junction proteins. </LI> </UL> </P>

Soziokulturelle Konflikte zwischen Ost- und Westdeutschen im vereinten Deutschland

Kim Nury(김누리) 한국카프카학회 2005 카프카연구 Vol.12 No.1

베를린 장벽이 무너진 지 15년이 지났지만 동서독 사람들의 머릿속 장벽은 여전히 건재하다. “분단은 장벽 시절보다 더 심각하다”(그라스), “독일인 다섯 명 중 하나는 장벽이 다시 세워지길 바란다.”(슈테른), “지금처럼 많은 동독인들이 자신을 패배자로 느낀 적은 없었다.”(슈피겔) 최근 몇 달간 독일에서 들려오는 풍문들은 사태가 날로 악화되고 있음을 알려준다. 왜 이런 지경이 되었을까. 동서독간의 경제적 격차가 예상보다 빠른 속도로 해소되고, 정치제도도 안정적으로 정착되는 상황에서 동서독 사람들 간의 사회문화적 갈등은 오히려 심화되는 이유는 무엇인가. 이 글은 이러한 문제에 대한 타당한 이유를 찾아보려는 시도이다. 사실 사회문화적 갈등의 조짐은 통일 초기부터 상존하고 있었다. 동서독 사람들간에 이질감이 심화되고, 이미 역사에서 사라진 동독의 정체성이 부활하는가 하면, 오스탈지아라는 이름의 동독에 대한 향수병이 확산되어 왔던 것이다. 수많은 지표가 이를 증명한다. 그럼 성공적인 체제통합에도 불구하고 이처럼 머릿속 장벽이 높아만 가는 이유는 무엇인가. 이러한 현상을 설명하기 위해 현재 여러 가지 가설들이 경쟁하고 있는데, 그 가운데 가장 대표적인 것은 ‘기형테제’, ‘전환테제’, ‘식민화테제’이다. 기형태제란 동독인들이 통일 이후 서독의 ‘민주제도’에 대해 보이는 불만과 적개심은 동독의 독재정권하에서 기형적으로 왜곡된 ‘권위주의적 성격’에 기인한다고 보는 입장이다. 이 테제에 따르면 동독인들이 평동의 가치와 사회보장의 중요성을 강조하는 것도 과거 권위주의 시대에 습성화된 기형적 심리구조의 탓이다. 전환테제는 동서독인 간의 사회문화 갈등은 전환기에 겪게 되는 자연스런 현상이라고 보는 입장이다. 사회주의 체제가 자본주의 체제로 전환하는 과정에서 일정한 갈등은 불가피하다는 것이다. 전환테제는 전환기에 동독인들이 겪은 일상의 체험이 불만의 원인이라고 보는 체험가설과 서독이 주도한 체제전환 과정에서 동독인들이 느낀 박탈감과 열등감을 보상받으려는 심리가 동독인의 저항적 정체성의 핵심이라고 보는 보상가설로 나뉜다. 식민화테제는 독일통일의 본질을 서독에 의한 동독의 식민화로 보는 입장이다. 신탁청에 의한 인민소유 재산의 민영화과정에서 동독 소유재산의 95%가 서방 자본의 손에 넘어간 역사적 사실이 식민화테제의 결정적인 근거를 제공한다. 경제적 식민화 이외에도 문화적, 사회적 식민화 현상에 대한 연구와 분석이 여전히 활발히 진행되고 있다. 이상 세 개의 테제는 통일 후 독일의 사회문화 갈등을 설명하는 데 가장 자주 동원되는 논리기제이다. 어떤 테제가 가장 유효한지는 현재로선 속단하기 어렵다. 보다 포괄적인 견지에서 세 테제를 상호보완적으로 적용할 때 통일독일의 사회문화 갈등에 대한 설득력 있는 해명이 가능할 것이다. 여기서 한 가지 주목할 점은 이 세 테제가 특정한 정치 분파의 시각과 상응한다는 점이다. 기형태제는 보수주의자와 극우파들이, 전환테제는 자유주의자들이, 식민화테제는 좌파들이 즐겨 사용하는 논리다. 현재까지의 연구결과를 놓고 볼 때 통일독일 문화갈동의 원인을 단순히 권위주의 체제에서 기형화된 심리구조에서 찾는 기형태제나 통일과정에서 경험된 경제적 불평등에서 찾는 식민화 테제는 무리가 있어 보인다. 1989년 동독혁명을 성공시킨 자들은 다름 아닌 ‘기형화되었다는’ 동독시민들 자신이었고, 현재 동독인들의 대다수가 개인적 경제상황에 만족을 표시하고 있기 때문이다. 따라서 현재의 동서독 갈등은 통일독일의 미래를 독점할 뿐 아니라 동독인들의 과거까지도 멋대로 재단하는 서독인들의 오만과 독주에 맞서 자신의 존재가치를 높이려고 애쓰는 동독인들의 ‘인정투쟁’ 과정에서 심화되고 있는 것처럼 보인다.