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Nusinersen for spinal muscular atrophy types Ⅱ and Ⅲ:a retrospective single-center study in South Korea

World Journal of Pediatrics 2023年第5期19卷 450-459页

作者： Hui Jin Shin Ji-hoon Na Hyunjoo Lee young-Mock Lee Department of Pediatrics Gangnam Severance HospitalYonsei University College of Medicine211 Eonju-RoGangnam-GuSeoul135-720Korea

Background This study investigated the efficacy and safety of nusinersen,an antisense oligonucleotide,in patients with spinal muscular atrophy(SMA)types II(OMIM:253,550)or III(OMIM:253,400),including those with severe scoliosis or requiring respiratory support via mechanical *** Data from 40 patients with genetically confirmed SMA who were treated with nusinersen at our institute from March 2019 to April 2022 were retrospectively *** these,30 patients with an age of onset<3 years and not on permanent ventilation were *** and genetic characteristics were investigated,and motor function was evaluated based on the Hammersmith Functional Motor Scale-Expanded(HFMSE)*** The mean age of symptom onset was 1.2 *** patients were diagnosed with SMA type II(27/30,90%).Nusinersen was administered via computed tomography-guided or direct intrathecal injection in 87%(26/30)and 13%(4/30)of the patients,*** the 6-,14-,22-,and 26-month follow-ups,72%,71%,88%,and 86%of patients showed motor improvement,respectively,with mean changes in HFMSE scores of 2.10,2.88,4.21,and 5.29,*** analysis showed that the use of noninvasive ventilation was associated with poorer outcomes of motor *** Patients with SMA type II or III who received nusinersen treatment showed significant improvement in motor function.A longer treatment duration led to a higher number of patients with improved motor *** significant side effects of nusinersen were *** with SMA,even those with severe scoliosis or on respiratory support,can be safely treated using nusinersen.

关键词： Hammersmith Functional Motor Scale Expanded Motor function Nusinersen South Korea Spinal muscular atrophy

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Preliminary experimental study of a supercritical CO2 power cycle test loop with a high-speed turbo-generator using R134a under similarity conditions

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Frontiers in Energy 2017年第4期11卷 452-460页

作者： Junhyun CHO Hyungki SHIN Jongjae CHO young-Seok KANG Ho-Sang RA Chulwoo roh Beomjoon LEE Gilbong LEE Byunghui KIM young-Jin BAIK Thermal Energy System Laboratory Korea Institute of Energy Research 152 Gajeong-ro Yusung-gu Daejeon 34129 Republic of Korea Engine Component Research Team Korea Aerospace Research Institute (KARl) 169-84 Gwahak-ro Yusung-gu Daejeon 34133 Republic of Korea InGineers 21 Saman-ro 16 Beon-gil Gimhae-si Gyeongsangnam-do 621-220 Republic of Korea

Research on applying a supercritical carbon dioxide power cycle （S-CO2） to concentrating solar power （CSP） instead of a steam Rankine cycle or an air Brayton cycle has been recently conducted. An S-CO2 system is suitable for CSP owing to its compactness, higher efficiency, and dry-cooling capability. At the Korea Institute of Energy Research （KIER）, to implement an S-CO2 system, a 10 kWe class test loop with a turbine- alternator-compressor （TAC） using gas foil bearings was developed. A basic sub-kWe class test loop with a high- speed radial type turbo-generator and a test loop with a capability of tens of kWe with an axial type turbo- generator were then developed. To solve the technical bottleneck of S-CO2 turbomachinery, a partial admission nozzle and oil-lubrication bearings were used in the turbo- generators. To experience the closed-power cycle and develop an operational strategy of S-CO2 operated at high pressure, an organic Rankine cycle （ORC） operating test using a refrigerant as the working fluid was conducted owing to its operational capability at relatively low- pressure conditions of approximately 30 to 40 bar. By operating the sub-kWe class test loop using R134a as the working fluid instead of CO2, an average turbine power of 400 W was obtained.

关键词： supercritical CO2 power cycle turbomachin-ery compressor turbine

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Proxy-Based Hierarchical Distributed Mobility Management for Tactical Networks

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Computers, Materials & Continua 2022年第2期70卷 2381-2399页

作者： Myoung-hun Han Bong-Soo roh Kyungwoo Kim Dae-hoon Kwon Jae-Hyun Ham KyungHyun Yoon Sanghyun Seo Agency for Defense Development Daejeon34186Korea Department of Computer Science and Engineering Chung-Ang UniversitySeoul06974Korea College of Art&Technology Chung-Ang UniversityAnseong17546Korea

An important requirement in a military domain is a highly reliable mobility management method,especially when components of the networks aremoving in tactical network *** increase reliability,themobility management technology of the tactical network should be able to reflect the characteristics of the tactical network,such as a limited environment,failure,and hierarchical unit *** this paper,we propose a proxy-based hierarchical distributedmobilitymanagement scheme,which is highly focused on tactical *** the characteristics of tactical networks,the proposed scheme is composed of the following:1)a proxy-based method,2)a distributed mobility management method that synchronizes a mobility database between entities,and 3)a method of managing mobility by dividing the tactical network into upper and lower *** analysis and modeling and simulation results demonstrate that the method outperforms the existing state-of-the-art method in overcoming entity failure,handover cost,and delay in tactical environments.

关键词： Mobility management tactical networks PMIP DMM

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Effects of different flui replacements on serum HSP70 and lymphocyte DNA damage in college athletes during exercise at high ambient temperatures

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Journal of Sport and Health Science 2016年第4期5卷 448-455页

作者： Hee-Tae roh Su-Youn Cho Wi-young So Il-young Paik Sang-hoon Suh Department of Physical Education Dong-A University Exercise Physiology Laboratory Department of Physical EducationYonsei University Sports and Health Care Major College of Humanities and ArtsKorea National University of Transportation

Purpose：The aim of this study was to investigate the effects of flui replacement by water or sports drinks on serum heat shock protein 70（HSP70） levels and DNA damage during exercise at a high ambient ***：Ten male college athletes with an athletic career ranging from 6 to 11 years were recruited from Yonsei *** subjects ran on a treadmill at 75% of heart rate reserve during 4 different trials：thermoneutral temperature at 18℃（T）,high ambient temperature at 32℃ without flui replacement（H）,high ambient temperature at 32℃ with water replacement（HW）,and high ambient temperature at 32℃ with sports drink replacement（HS）.During each condition,blood samples were collected at the pre-exercise baseline（PEB）,immediately after exercise（IAE）,and60 min ***：Skin temperature significant y increased during exercise and was significant y higher in H compared to T and HS at ***,serum HSP70 was significant y increased in all conditions at IAE compared to PEB and was higher in H compared to T at the former time *** y increased lymphocyte DNA damage（DNA in the tail,tail length,tail moment） was observed in all trials at IAE compared to PEB,and attenuated DNA damage（tail moment） was observed in HS compared to H at ***：Acute exercise elevates serum HSP70 and induces lymphocyte DNA *** replacement by sports drink during exercise at high ambient temperature can attenuate HSP response and DNA damage by preventing dehydration and reducing thermal stress.

关键词： Acute exercise DNA damage Fluid replacement Heat stress HSP70 Sports drink

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High-temperature Oxidation Behavior of Zircaloy-4 and Zirlo in Steam Ambient

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Journal of Materials Science & Technology 2010年第9期26卷 827-832页

作者： Hyung hoon Kim Jun Ho Kim Jin young Moon Ho Seong Lee Jeong Joo Kim young Suck Chai School of Materials Science and Engineering Kyungpook National University School of Mechanical Engineering Yeungnam University214-1 Dae-dongGyeongsan-si

The oxidation characteristics for Zircaloy-4 and Zirlo in the temperature range of 700-1200°C under steam supply condition were investigated by using a modified thermo-gravimetric *** specimens were oxidized for 3600 s at each temperature and then quenched in a *** oxidation rate constants were measured from the weight gains to evaluate the oxidation behavior in Zircaloy-4 and *** weight gain rates of Zirlo were lower than those of Zircaloy-4,leading to the low rate *** different oxidation behaviors between both cladding materials were considered to be due to the difference in their chemical compositions.

关键词： Zircaloy-4 Zirlo Oxidation

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Effect of robot-assisted gait training on the biomechanical properties of burn scars:a single-blind,randomized controlled trial

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Burns & Trauma 2022年第1期10卷 191-199页

作者： Yoon Soo Cho So young Joo Cheong hoon Seo Department of Rehabilitation Medicine Hangang Sacred Heart HospitalCollege of MedicineHallym UniversitySeoul 07247Republic of Korea

Background:Robot-assisted gait training(RAGT)is more effective in the range of motion(ROM)and isometric strength in patients with burns than conventional ***,concerns have been raised about whether RAGT might negatively affect the scars of patients with ***,we investigated the effects of RAGT-induced mechanical load on the biomechanical properties of burn ***:This was a single-blind,randomized clinical trial conducted on inpatients admitted to the Department of Rehabilitation Medicine between September 2020 and August *** was conducted for 30 min per day,five days a week for 12 weeks and the control group received conventional gait training for 12 *** pre-training ROM of lower extremity joints was evaluated and the levels of melanin,erythema,trans-epidermal water loss,scar distensibility and elasticity were assessed before training and at 4 and 12 weeks after ***,19 patients in the gait assistance robot(GAR)group and 20 patients in the control group completed the 12-week trial and all ***:There were no significant differences in the epidemiologic characteristics,pre-training ROM of joints and pre-training biomechanical properties of the burn scar between the groups(p>0.05 for all).None of the patients experienced skin abrasion around the burn scar where the fastening belts were applied or musculoskeletal or cardiovascular adverse events during the *** thickness significantly increased in both groups(p=0.037 and p=0.019)and scar distensibility significantly decreased in the control group(p=0.011)during the *** was significantly decreased in the GAR group during the training(p=0.038).The GAR and control groups showed significant difference in the change in the values of hysteresis between pre-training and 12 weeks after training(p=0.441 and p=0.049).Conclusions:RAGT significantly decreased hysteresis in hypertrophic burn scars and did not cause a significant decrease

关键词： Robot-assisted gait training Burn Scar biomechanical properties Hysteresis Burn rehabilitation

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Clinical significance and usefulness of soluble heparin binding-epidermal growth factor in gastric cancer

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World Journal of Gastroenterology 2015年第7期21卷 2080-2088页

作者： Hye Won Chung hoon young Kong Jong-Baeck Lim Department of Internal Medicine Division of Gastroenterology International St. Mary’s Hospital Incheon Metropolitan City Department of Laboratory Medicine Yonsei University College of Medicine Gangnam Severance Hospital

AIM:To evaluate the clinical usefulness of solubleheparin-binding epidermal growth factor(s HB-EGF)as a serum biomarker for gastric cancer(GC).METHODS:Serum s HB-EGF levels were measured by a commercially available human HB-EGF ELISA Kit and compared among 60 normal controls,30 highrisk patients,37 early gastric cancer(EGC),and30 advanced gastric cancer(AGC)through ANOVA *** between serum s HB-EGF and clinicopathological features of GC were analyzed through Spearman’s *** diagnostic performance of serum s HB-EGF for GC was evaluated through receiver operating characteristic(ROC)curve and logistic regression ***:Serum s HB-EGF levels were significantly higher in AGC group(314.4±127.5 pg/m L)than EGC(165.3±123.2 pg/m L),high-risk(98.7±67.3 pg/m L),and control(94.7±83.6 pg/m L)groups(post-hoc Bonferroni,all P<0.001),*** s HB-EGF levels were also significantly higher in EGC group than high-risk(P=0.049)and control(P=0.006)***,serum s HB-EGF levels closely correlated with depth of invasion(T-stage,γs=0.669,P<0.001),lymph node metastasis(N-stage,γs=0.407,P=0.001),and distant metastasis(M-stage,γs=0.261,P=0.030).ROC curve and logistic regression analysis demonstrated a remarkable diagnostic potential of serum s ***:Serum s HB-EGF is closely correlated with advanced stage GC and can be a promising serological biomarker for GC.

关键词： Biomarker Diagnostic Gastric cancer Prognostic Sol

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Fluid management in living donor hepatectomy:Recent issues and perspectives

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World Journal of Gastroenterology 2015年第45期21卷 12757-12766页

作者： Seong-Soo Choi Sung-hoon Kim young-Kug Kim Department of Anesthesiology and Pain Medicine Asan Medical Center University of Ulsan College of Medicine

The importance of the safety of healthy living liver donors is widely recognized during donor hepatectomywhich is associated with blood loss, transfusion, and subsequent post-operative morbidity. Although the low central venous pressure(CVP) technique can still be effective, it may not be advantageous concerning the safety of healthy donors undergoing hepatectomy. Emerging evidence suggests that stroke volume variation(SVV), a simple and useful index for fluid responsiveness and preload status in various clinical situations, can be applied as a guide for fluid management to reduce blood loss during living donor hepatectomy. Synthetic colloid solutions are also associated with serious adverse events such as the use of renal replacement therapy and transfusion in critically ill or septic patients. However, it is uncertain whether the intra-operative use of colloid solution is associated with similarly adverse effects in patients undergoing living donor hepatectomy. In this review article we discuss the recent issues regarding the low CVP technique and the high SVV method, i.e., maintaining 10%-20% of SVV, for fluid management in order to reduce blood loss during living donor hepatectomy. In addition, we briefly discuss the effects of intra-operative colloid or crystalloid administration for surgical rather than septic or critically ill patients.

关键词： Donor hepatectomy Central venous pressure Stroke v

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Predictive factors for early clinical response in community-onset Escherichia coli urinary tract infection and effects of initial antibiotic treatment on early clinical response

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World Journal of Clinical Cases 2020年第19期8卷 4342-4348页

作者： young Jun Kim Jeong-Mi Lee Jae-hoon Lee Department of Internal Medicine Wonkwang University College of MedicineIksan 54538South Korea Department of Public Health Wonkwang University College of MedicineIksan 54538South Korea Department of Internal Medicine and Institute of Wonkwang Medical Science Wonkwang University College of MedicineIksan 54538South Korea

BACKGROUND Urinary tract infection(UTI)is a common *** often requires hospitalization,and severe presentations,including sepsis and other complications,have a mortality rate of 6.7%-8.7%.AIM To evaluate the predictive factors for early clinical response and effects of initial antibiotic therapy on early clinical response in community-onset Escherichia coli(***)urinary tract infections(UTIs).METHODS This retrospective study was conducted at Wonkwang University Hospital in South Korea between January 2011 and December *** patients(aged>18 years)who were diagnosed with community-onset *** UTI were enrolled in this *** A total of 511 hospitalized patients were included.66.1%of the patients had an early clinical *** mean length of hospital stay in patients with an early clinical response were each 4.3 d shorter than in patients without an early clinical *** the multiple regression analysis,initial appropriate antibiotic therapy(OR=2.449,P=0.006),extended-spectrum(3-lactamase(ESBL)-producing ***(OR=2.112,P=0.044),improper use of broad-spectrum antimicrobials(OR=0.411,P=0.006),and a stay in a healthcare facility before admission(OR=0.562,P=0.033)were the factors associated with an early clinical *** broadspectrum antibiotic therapy was not associated with an early clinical *** ESBL producing ***,and the type of residence before hospital admission were the factors associated with an early clinical *** of initial antibiotic therapy was a predictive factor for an early clinical response,but broadspectrum of initial antibiotic therapy did not impact early clinical response.

关键词： Escherichia coli Urinary tract infections Adult Community acquired infections

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Adaptive Model Predictive Control for Yaw System of Variable-speed Wind Turbines

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Journal of Modern Power Systems and Clean Energy 2021年第1期9卷 219-224页

作者： Dongran Song Qing Chang Songyue Zheng Sheng Yang Jian Yang young hoon Joo the School of Automation Central South Universityand Hunan Provincial Key Laboratory of Power Electronics Equipment and GridChangsha 410083China the School of Energy Science and Engineering Central South UniversityChangsha 410083China the School of IT Information and Control Engineering Kunsan National UniversityKunsan 54150Korea

Due to varying characteristics of the wind condition, the performance of the wind turbines can be optimized by adapting the parameters of the control system. In this letter, an adaptive technique is proposed for the novel model predictive control(MPC) for the yaw system of the wind turbines. The control horizon is adapted to the one with the best predictive performance among multiple control horizons. The adaptive MPC is demonstrated by simulations using real wind data, and its performance is compared with the baseline MPC at fixed control horizon. Results show that the adaptive MPC provides better comprehensive performance than the baseline ones at different preview time of wind directions. Therefore, the proposed adaptive technique is potentially useful for the wind turbines in the future.

关键词： Wind turbine yaw system wind direction adaptive model predictive control

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