A Case of an 18-month-old Boy with Type 3 Gaucher Disease Presenting with Hepatosplenomegaly and Growth Retardation: The Clinical Course after Enzyme Replacement Therapy

임영신,황정윤,김진섭,양아람,박형두,전태연,조성윤,진동규,Lim, Young Shin,Hwang, Jeongyun,Kim, Jinsup,Yang, Aram,Park, Hyung Doo,Jeon, Tae Yeon,Cho, Sung Yoon,Jin, Dong-Kyu The Korea Society of Inherited Metabolic Disease 2017 대한유전성대사질환학회지 Vol.17 No.2

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by beta-glucosidase deficiency. An 18 month-old male with hepatosplenomegaly, anemia, thrombocytopenia, and growth retardation referred to our hospital. The patient showed neurological symptoms, such as supranuclear gaze palsy and developmental delay. Bone marrow biopsy performed to rule out malignancy and the results revealed no malignant cell; however, abnormal histiocytes suggesting storage disease was noted. Based on hepatosplenomegaly, bicytopenia and unexplained neurologic manifestations, enzyme activity and genetic analysis were conducted emergently with a strong suspicion of GD. Beta-glucosidase activity in leukocyte was decreased. GBA sequencing to confirm the diagnosis revealed compound heterozygous pathogenic variants (i.e., c.754T>A, c.887G>A), both previously reported as the cause of neuronopathic GD. Under the diagnosis of type 3 GD, the patient immediately received enzyme replacement therapy (ERT). After 17 months of ERT, the size of spleen decreased, and hemoglobin and platelet count returned to normal. In addition, the activity of chitotriosidase and angiotensin converting enzyme decreased. However, myoclonic movement and generalized seizure occurred at the age of 19 months and antiepileptic drug was started. Other neurological deterioration including supranuclear gaze palsy and developmental delay also persisted. A new therapy to overcome neurologic problems should be developed for patients with type 3 GD.

레이저-옵티컬 디스드로미터를 활용한 대전지역의 강우에너지 특성 평가

임영신 ( Young Shin Lim ),김종옥 ( Jong Wook Kim ),김진관 ( Jin Kwan Kim ),박병익 ( Byong Ik Park ) 한국지형학회 2012 한국지형학회지 Vol.19 No.2

To evaluate the kinetic energy of the raindrops, the drop size distribution and the terminal velocity of the raindrops had been measured from January to September 2010 using the laser-optical disdrometer in KIGAM, Daejeon, Korea. The relationship between kinetic energy (KE) and rainfall intensity (I) was computed as logarithmic and exponential model, respectively, under the rainfall intensity of about 142mm/h. The exponential model is more suitable for the relationship of KE-I than the logarithmic model, because the exponential model presented better fit for KE over 50mm/h of rainfall intensity. Meanwhile, the differences of the total kinetic energy existed in rainfall events with almost same total rainfall depth, and KE values of Daejeon at high rainfall intensity underestimated rather than the others under temperate climate. Therefore, these differences of KE in rainfall events and geographical regions imply the result from the variations of rainfall intensity within a rainfall event.

무안경식 3차원 모니터용 실시간 눈 추적 알고리즘

임영신(Young-Shin Lim),김준식(Joon-Seek Kim),주효남(Hyonam Joo) 제어로봇시스템학회 2009 제어·로봇·시스템학회 논문지 Vol.15 No.8

In this paper, a real-time eye tracking method using fast face detection is proposed. Most of the current eye tracking systems have operational limitations due to sensors, complicated backgrounds, and uneven lighting condition. It also suffers from slow response time which is not proper for a real-time application. The tracking performance is low under complicated background and uneven lighting condition. The proposed algorithm detects face region from acquired image using elliptic Hough transform followed by eye detection within the detected face region using Haar-like features. In order to reduce the computation time in tracking eyes, the algorithm predicts next frame search region from the information obtained in the current frame. Experiments through simulation show good performance of the proposed method under various environments.

L2 습득이론에 따른 영어 마찰음에 대한 한국 어린이 영어 학습자의 인지 능력

임영신 ( Youngshin Lim ),장우혁 ( Wooheok Chang ) 대한언어학회 2019 언어학 Vol.27 No.1

In this study, we investigate how L2 acquisition theories can successfully account for the relative difficulties in the perception of English fricatives by Korean young learners. Regarding the acquisition of L2 phonemes, the previous studies can be divided into two main approaches: (1) phoneme-based analysis which is mainly concerned with Loanword Phonology and Perceptual Assimilation Model vs. (2) feature-based analysis. Noticing that these analyses make different predictions on the acquisition of L2 sounds, we conducted AX discrimination task and 4I-oddity task to find which types of English fricatives (voicing, place, and manner of articulation) Korean young learners have difficulties in perceiving. 30 Korean elementary school students participated in the tasks and 27 minimal pairs including word-initial English fricatives were employed. Crucial to the analysis of the experimental results is that with one particular L2 acquisition theory it is impossible to explain why Korean learners can or cannot perceive English fricatives distinctively. That is, Korean young learners’ perceptibility of English fricatives is attributed to both phonemic differences and feature differences between L1 and L2. Furthermore, in some cases, the fact that Korean young learners fail to discriminate certain pairs of English fricatives should be better accounted for by considering phonetic characteristics of L1 and L2 fricatives.

RUSLE를 이용한 사굴링 댐 저수지 유역의 토양 유실량 및 침식 위험 평가

임영신 ( Lim Young Shin ),기신우 ( Ki Shinwoo ),김예원 ( Kim Ye Won ),( Luki Subehi ),김진관 ( Kim Jin Kwan ) 한국지형학회 2024 한국지형학회지 Vol.31 No.4

This study aimed to estimate soil loss and identify high-erosion-risk areas in the Saguling Dam reservoir catchment using the Revised Universal Soil Loss Equation (RUSLE), as the catchment faces serious issues of soil erosion, water pollution, and reservoir sedimentation. Input data were prepared by considering the spatial and interannual variability of rainfall erosivity and land cover conditions. The simulation estimated a total soil loss of 19,662,905 tons in 2020 under long-term average rainfall conditions. Reflecting changes in land cover over time, the average annual soil loss was calculated as 5.28, 8.19, and 8.02 tons/ha/year for 1989, 2000, and 2020, respectively. High-erosion-risk areas were primarily concentrated in valleys and steep slope croplands directly connected to these valleys, with a notable overlap with LS factor values ranging from 12 to 17.2. The Cisangkeuy, Cirasea, and Citarik catchments, located in the southern and eastern parts of the area, were identified as the most vulnerable to soil loss. Under wet rainfall conditions, rainfall erosivity increased significantly, resulting in an estimated doubling of high-erosion-risk areas compared to those identified under dry conditions. An effective soil conservation strategy should focus on managing steep slope croplands, while also considering the spatio-temporal variability of factors influencing soil loss.

SWAT을 이용한 최상류 소유역 토양침식 평가: 안성 월동저수지 유역을 대상으로

임영신 ( Young Shin Lim ),변종민 ( Jongmin Byun ),김진관 ( Jin Kwan Kim ) 한국지형학회 2021 한국지형학회지 Vol.28 No.1

Successful sediment management at the watershed scale requires an understanding of the erosion, transport and sedimentation processes at the specific site scale. However, studies on the sediment runoff characteristics in a small uppermost watershed, which serves as a sediment supply function, are very rare. Therefore, this study attempted to investigate the fluctuations in major sediment supply areas and sediment runoff in the uppermost mountain small watershed, and for this purpose, ArcSWAT (Soil and Water Assessment Tools with GIS interface) was applied to the Woldong reservoir catchment located in Gosammyeon, Anseong-si, Gyeonggi-do. The model results were manually calibrated using the monitoring data of the Woldong reservoir sedimentation rate from 2005 to 2007. It was estimated that annual average of 34.4 tons/year of sediment was discharged from the Woldong reservoir basin. This estimate almost coincided with the monitoring data of the Woldong reservoir during the low flow period but tended to be somewhat underestimated during the high flow period. Although the SWAT model does not fully reflect the erosion process of gully and in-channel, this underestimation is probably due to the spatial connectivity of sediment transport and the storage and reactivation of the sediment being transported. Most of the forested hillslopes with a well-developed organic horizon were evaluated as having a low risk of erosion, while the places with the highest risk of erosion were predicted to be distributed in the logged area with some weeds or shrubs (classified as pasture) with relatively steeper slopes, and in the bare land. The results of this study are expected to be useful in developing strategies for sediment control and reservoir management.

Cognitive Engagement in AI-Supported Integrated Language Learning: A Comparative Study of CBI and TBI Using Bloom's Taxonomy

임영신(Young-shin Lim) 제주대학교 교육과학연구소 2024 교육과학연구 Vol.26 No.4

This study explores the impact of AI on integrative language instruction, specifically comparing Task-based Instruction (TBI) and Content-based Instruction (CBI) through the lens of Bloom’s revised taxonomy. The research examines how AI influences language learners' cognitive processes and knowledge acquisition. Data were collected from 17 participants and three instructors using an open-ended questionnaire, semi-structured interviews, and teacher’s reflective notes, and were analyzed using content analysis. Findings revealed three key pedagogical implications: (1) In TBI, learners predominantly used procedural knowledge and applied cognitive processes, while CBI encouraged a broader range of cognitive skills. (2) The integration of AI in these instructional methods activated higher-order thinking skills, particularly for error detection and progress monitoring. (3) Divergent perspectives emerged between learners and instructors; learners appreciated AI’s role in enhancing language learning, whereas instructors expressed concerns about AI potentially undermining learners’independent learning and language competence. The study suggests that while AI can support language learning, it is crucial to ensure that its application complements rather than hinders learners’cognitive and communicative development. 본 연구는 AI의 영향을 탐구하며, Bloom의 수정된 분류체계를 통해 통합 언어 교육인 과업 중심 교육(TBI)과 내용 중심 교육(CBI)을 비교 분석하고자 하였다. 특히, 본 연구는 AI가 언어 학습자의 인지 과정과 지식 습득에 미치는 영향을 통합적으로 이해하고자 데이터 다각화(data triangulation)로 자료를 수집하였다. 17명의 참여자와 세 명의 강사로부터 개방형 설문조사지, 반구조화 인터뷰, 그리고 교사의 성찰 일지를 통해 데이터를 수집하고, 내용 분석을 통해 수집된 자료 중 개방형 설문지 응답을 분석하였다. 연구 결과는 세 가지의 주요 교육적 시사점을 다음과 같이 제시한다. (1) TBI에서는 학습자들이 주로 절차적 지식과 적용 인지 과정을 사용한 반면, CBI에서는 더 다양한 인지 기술이 활용되는 것을 확인하였다. (2) 이러한 교수 방법에 AI의 발달된 기능인 오류에 대한 인지와 학습과정 모니터링이 언어교육에 접목되었을 때 고차원 사고 기술이 활용되는 것을 관측하였다. (3) 반면에, 실험 참가자 집단의 AI활용에 대한 인식에 관한 조사결과, 학습자와 강사 간에는 상반된 관점이 나타나는 것을 확인하였다. 이렇게 두 집단의 상반된 인식에 대한 결과에 기반하여, 본 연구는 AI의 언어교육 적용에 대한 긍정적이면서 부정적인 효과에 대한 통합적인 접근이 필요하다고 제언한다.

초등 영어 학습자들의 영어교육 인식에 대한 비판적 고찰

임영신(Lim YoungShin) 학습자중심교과교육학회 2020 학습자중심교과교육연구 Vol.20 No.2

This study is to investigate in a critical perspective how the socio-political ideology has reflected in English education and English as a lingua franca in Korea in the perception of Korean primary school students. The study is analyzed in mixed methods first, with a total of 54 Korean primary school students participated in quantitative research to see the general perspective in English and English education in Korea. Based on the result of the survey, semi-structured interviews within the qualitative research method are employed with 3 primary school students. The findings are summarized as follows: first, neo-colonialism has been reflected in the perspective of Korean primary school students in English and English education by perceiving native speakers’ pronunciation and English superior to non-native speakers. In addition, it is analyzed that Korean students perceive English as a lingua franca as they point out that the significance of English is in communicating with other foreigners. Second, they perceive English is necessary for the present and future society as it deeply affects their entry to university and their career. Third, the contrastive influence on linguistic identity is demonstrated in-depth interviews with different learning motivation as it has different linguistic status between English and other foreign languages. Based on this analysis pedagogical implications for English in a critical perspective have been suggested.

코로나19 상황 중 뇌성마비 장애학생의 홈트레이닝 참여정도가 프로그램 만족 및 지속의도에 미치는 영향

임영신(Lim, Youngshin),한아름(Han, Areum),이민구(Lee, Mingoo),김매이(Kim, May) 한국여가레크리에이션학회 2021 한국여가레크리에이션학회지 Vol.45 No.3

전기노인과 중기노인의 여가참여 유형과 노인차별 경험 및 삶의 만족 간의 관계

임영신(Lim, Youngshin),김매이(Kim, May) 한국여가레크리에이션학회 2024 한국여가레크리에이션학회지 Vol.48 No.4