• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• Journal of the Korean Society for Railway
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• v.18 no.5
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• pp.439-446
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• 2015

The turnout system, which determines the direction of the train, is not only a key system but also a vulnerable system. Failure of this system may lead to a delay of the train or even casualties. In this light, it is necessary to precisely the conditions of the turnout system. Currently, ROADMASTER of Germany is used as a diagnostic system in Korea. However, a new diagnostic system should be developed for optimized operation of the turnout system with maintenance that is suitable for the Korean railway environment. In this paper, a Fault Tree Analysis for the representative faults of the turnout system is conducted and physical quantities, which can be the cause of the fault, are classified according to the component and function. Also, the measuring factors for the monitoring are derived and a decision making theory is suggested. On the basis of the results, we propose a new turnout diagnostic system that can provide more driverse and precise information than the conventional system.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.22 no.6
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• pp.27-35
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• 2021

Generally, a proper evaluation method of the aging phenomenon of PV modules is required as the electrical performance and lifespan of PV modules can degrade significantly due to several environmental factors, while they are generally known as devices that are used semi-permanently for more than 20 years. On the other hand, there is a lack of objectivity in the existing evaluation method of the aging phenomenon, which compares the adjusted PV output based on STC with the initial PV module specifications due to the data distortion while adjusting the measured data. Therefore, this study implemented a test device for an aging diagnosis to measure and collect actual data from a PV module section and modeled the data for aging using MATLAB S/W to minimize the variability of the PV output, communication error, and delay. Furthermore, this study confirmed the usefulness of the presented test device for aging diagnosis of the PV modules by diagnosing the total period and yearly-basis degradation rate of aging PV modules as 25.73% and 1.55%, respectively, according to the on-site output characteristics of the PV modules by season.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.5
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• pp.369-374
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• 2008

Purpose: F-18 FDG PET/CT has excellent sensitivity and specificity for staging non-Hodgkin lymphomas, but to the author's knowledge few studies to date have evaluated FDG PET/CT in peripheral T cell lymphoma. We evaluated the usefulness of F-18 FDG PET/CT in staging of patients with peripheral T cell lymphoma, especially indolent cutaneous T cell lymphomas. Materials and Methods: Twenty five patients (M:F=17:8, age $53.7{\pm}14.8$ yrs) with biopsy-proven indolent cutaneous T cell (CL) or noncutaneous T cell lymphomas (NCL) underwent PET/CT scans for staging at baseline. Peak standardized uptake values (p-SUV) of all abnormal foci were measured and compared between cutaneous and noncutaneous lesions. F-18 FDG PET/CT was performed on 6 patients with indolent CL and on 19 patients with NCL. Results: All 6 patients with indolent CL had no significant FDG avidity in the skin despite histologically positive cutaneous lesions. However, FDG avidity appeared in extracutaneous lesions (lymph nodes) in two patients with CL where CT imaging suggested lymphoma involvement (mean p-SUV $4.26{\pm}0.37$ in noncutaneous lesions in CL). In NCL, FDG avidity was demonstrated in all lesions where CT imaging suggested lymphoma involvement (mean p-SUV, $8.52{\pm}5.00$ in noncutaneous lesions in NCL). Conclusion: F-18 FDG PET/CT has the limitation of usefulness for the evaluation of the skin in indolent CL. In contrast, F-18 FDG PET/CT is sensitive in staging evaluation of extracutaneous lesions regardless of CL or NCL.

• Journal of Yeungnam Medical Science
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• v.27 no.2
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• pp.165-172
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• 2010

Primary thyroid lymphoma is a relatively rare thyroid tumor and usually a non-Hodgkin type. Its most common histologic type is the diffuse large B cell lymphoma followed by mucosa-associated lymphoid tissue(MALT). It is known to be frequently associated with autoimmune thyroiditis such as Hashimoto's thyroiditis. We report three cases of thyroid lymphoma at a single institution with are view of the literature.

• Journal of the Korea institute for structural maintenance and inspection
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• v.23 no.7
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• pp.152-163
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• 2019

In this paper, effect of steel fiber inclusion, compressive strength of matrix, shear reinforcement and shear span to depth ratio on the flexural behavior of UHPFRC(Ultra High Performance Fiber Reinforced Concrete) were investigated with test of 10-UHPFRC beam specimens. All test specimens were subjected to the flexural static loading. It was shown that steel fiber significantly improve the shear strength of UHPFRC beams. 2% volume fraction of steel fiber change the mode of failure from shear failure to flexural failure and delayed the failure of compressive strut with comparatively short shear span to depth ratio. UHPFRC beams without steel fiber had a 45-degree crack angle and fiber reinforced one had lower crack angle. Shear reinforcement contribution on shear strength of beams can be calculated by 45-degree truss model with acceptable conservatism. Using test results, French and Korean UHPFRC design recommendations were evaluated. French recommendation have shown conservative results on flexural behavior but Korean recommendation have shown overestimation for flexural strength. Both recommendations have shown the conservatism on the flexural ductility and shear strength either.

• Journal of the Korea institute for structural maintenance and inspection
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• v.19 no.2
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• pp.125-132
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• 2015

This paper presents an experimental investigation in order to evaluate fresh and hardened properties of LP (Limestone Powder) blended cement concrete. The cement contents of the mixtures are replaced by LP in the range of 10%, 15%, 25%, and 35%, while a control mixture is prepared with only OPC (Ordinary Portland Cement). The fresh concrete properties like slump and air content are similar to those of control mixture up to 35% of replacement ratio of LP, however a delay in setting time is evaluated. The hardened properties including compressive strength, flexural strength, and rapid freezing and thawing resistance shows similar results of control mixture up to 15% of replacement. Relatively lower strength development is evaluated over 25% replacement of LP. For accelerated carbonation test, resistance to carbonation rapidly decreases with increasing LP replacement ratio due to the limited amount of $Ca(OH)_2$. From the study, LP replacement under 15% can be adopted considering reduction of strength and resistance to carbonation.

• Proceedings of the Korean Society for Agricultural Machinery Conference
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• 2017.04a
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• pp.166-166
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• 2017

모돈의 건강 상태를 정량 지수화 하기 위한 연구를 수행 중이다. 지제이상, 섭식 불량, 수면 패턴 등의 운동 특성 분석을 위하여 복수의 초음파 센서를 이용하였다. 시계열 계측 신호를 분석하여 정량 지수화를 수행하는 과정에서 주파수 도메인 분석을 시도하였다. 이 과정에서 주파수 도메인의 분해능에 따른 편차 극복을 위한 비선형 모델링을 수행하였다. 또한 인접한 시계열 데이터 구간 간의 상관성 분석이 가능하면 대용량 데이터의 실시간 처리로 인한 지연 시간 극복 및 기대되는 예후에 대한 조기 진단이 가능할 것이다. 본 연구에서는 구글에서 제공하는 Tensorflow와 NVIDIA에서 제공하는 CUDA 엔진을 동시 적용한 심층 학습 시스템을 이용하였다. 전 처리를 위하여 주파수 분해능 (2분, 3분, 5분, 7분, 11분, 13분, 17분, 19분)에 따른 데이터 집합을 1단계로 두고, 상위 10 순위 안에 드는 파워 스펙트럼 밀도의 크기를 2단계로 하여, 총 2~10개의 입력 노드를 순차적으로 선정하였고, 동일한 방식으로 인접한 시계열의 파워 스펙터럼 밀도를 순위를 변화시켜 지정하였다. 대표적인 심층학습 모델인 Softmax regression with a multilayer convolutional network를 이용하여 Recursive feature selection 경우의 수를 $8{\times}9{\times}9$로 총 648 가지 선정하고, Epoch는 10,000회로 지정하였다. Calibration 모델링의 경우 Cost function이 10% 이하인 경우 해당 경우의 학습을 중단하였으며, 모델 간 상호 교차 검증을 수행하기 위하여 $_8C_2{\times}_8C_2{\times}_8C_2$ 경우의 수에 대한 Verification test를 수행하였다. Calibration 과정 상 모든 경우에 대하여 10% 이하의 Cost function 값을 보였으나, 검증 테스트 과정에서 모든 경우에 대하여 $r^2$ < 0.5 인 결정 계수 값이 나타났다. 단적으로 심층학습 모델의 과도한 적합(Over fitting) 방식의 한계를 보인 것이라 판단할 수 있다. 적합한 Feature selection 및 심층 학습 모델에 대한 지속적이고 추가적인 고려를 통해 과도적합을 해소함과 동시에 실효적이고 활용 가능한 Classification을 위한 입, 출력 노드 단의 전후 Indexing, Quantization에 대한 고려가 필요할 것이다. 이를 통해 모돈 생체 정보 정량화를 위한 지능형 현장 진단 기술 연구를 지속할 것이다.

• Journal of the Korean Institute of Illuminating and Electrical Installation Engineers
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• v.13 no.3
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• pp.73-81
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• 1999

Measurements of ultrasonic signals caused by partial discharges were perlonnrl by using an ultrasonic measurement technique to diagnose the electrical treeing deterioration in an epoxy mold-type power transformer. We also examined the characteristics of tree growth, partial discharge magnitude and ultrasonic pulse number with the increase of the deterioration time. From these results, it was found that the shape of the tree was a branch-type, and the rates of the tree growth were examined when ac voltages of 16[kV] and 20[kV] were applied Ultrasonic pulse number and discharge magnitude were rapidly increased as the length of the tree grows after the middle stage of the deterioration time, and it appeared that ultrasonic pulse number was proportional to discharge magnitude. Attenuation, time-delaying and directivity characteristics of ultrasonic signals propagated into epoxy resin by using ultrasonic oscillation and receiving systems are also re[prted as a basic data of ultrasonic mesasurements in mold-type power transformer.former.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.400-403
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• 2003

Congenital adrenal hyperplasia(CAH) is a recognized cause of precocious pseudopuberty. Some children with CAH also develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We review a case of CAH who eventually developed central precocious puberty nine months after initial treatment with corticosteroid. A 3-year-old boy visited complaining of rapid growth, a large penis and frequent penile erections. This patient was diagnosed with CAH with elevated 17-OH progesterone and cortical hypertrophy of adrenal gland on CT scan. His gonadotropin levels were within the normal prepubertal range. Even on treatment with corticosteroid he grew rapidly and had testicular enlargement, pubic hair development and rapid bone maturation. At second admission, his gonadotropin levels were elevated both basally and in response to LHRH stimulation, suggesting that the CAH led to early activation of pubertal gonadotropin secretion(true precocious puberty). He was treated with monthly depot injections of a LHRH analog in addition to the hydrocortisone. His second sexual characteristics regressed gradually and rate of linear growth and bone maturation decreased.