• Title/Summary/Keyword: ERT

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A Review of Recent Research in Treatment Approaches of Mucopolysaccharidosis (MPS)

  • Yang, Aram;Kim, Jinsup;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.3 no.2
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    • pp.37-40
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    • 2017
  • Mucopolysaccharidosis (MPS) is caused by accumulation of the glycosaminoglycans in all tissues due to decreased activity of the lysosomal enzyme. Patients exhibit multisystemic signs and symptoms in a chronic and progressive manner, especially with changes in the skeleton, cardiopulmonary system, central nervous system, cornea, skin, liver, and spleen. In the past, treatment of MPS was limited to enzyme replacement therapy (ERT). The outcome for affected patients improved with the introduction of new technologies as hematopoietic stem cell transplantation, relegated to specific situations after ERT became available. Intrathecal ERT may be considered in situations of high neurosurgical risk but still it is experimental in humans. New insights on the pathophysiology of MPS disorders are leading to alternative therapeutic approaches, as gene therapy, inflammatory response modulators and substrate reduction therapy. In this paper, we will highlight the recent novel treatment and clinical trials for MPS and discuss with the goal of fostering an understanding of this field.

Verification of Reinforcement with Grouting Materials in a Small Scale Reservoir Dike using Surface and Borehole Electrical Resistivity survey (지표 및 시추공 전기비저항 탐사를 중심으로)

  • Song, Sung-Ho;Yong, Hwan-Ho;Kim, Yang-Bin
    • Geophysics and Geophysical Exploration
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    • v.12 no.3
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    • pp.239-245
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    • 2009
  • To verify the reinforcing effect of grouting materials composed of colloid cement and ordinary portland cement on the water leakage region in a small scale dike, we performed a tubecasing method and applied surface electrical resistivity survey including electrical resistivity tomography (ERT) to find resistivity variation before and after grouting. Hydraulic conductivities after grouting show 10 times lower than those of before grouting. These variation indicates that the cement grout blocks the leakage pathway effectively. As the results of dipole-dipole resistivity survey along the dike, resistivity distribution after grouting did not represent noticeable spatial variation in time. Resistivity monitoring results at the dike with vertical electrical sounding (VES) showed that the region of decreasing apparent resistivity was occupied by the grout after grouting. Predicted resistivities from the inversion of ERT data well matched with results of VES at the same regions. From the ERT using check holes to inspect the effect of grouting, we could find that the ERT is quite effective to identify spatially the grout region in a dike.

Effect of Enzyme Replacement Therapy on Growth in Korean Patients with Mucopolysaccharidosis Type I (I형 뮤코다당증 환자들에서 효소 보충 요법이 성장에 미치는 영향)

  • Huh, Rimm;Cho, Sung Yoon;Chang, Mi Sun;Lee, Jieun;Kwun, Younghee;Kim, Su Jin;Sohn, Young Bae;Park, Sung Won;Maeng, Se Hyun;Kwon, Eun-Kyung;Han, Sun Ju;Jung, Jooyoun;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.13 no.2
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    • pp.111-119
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    • 2013
  • Purpose: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by deficiency of the enzyme ${\alpha}$-L-iduronidase, which leads to a broad spectrum of multisystemic manifestations. Short stature and decreased growth velocity are prominent features of MPS I. The aim of the present study was to evaluate the effect of enzyme replacement therapy (ERT) on growth of Korean MPS I patients from a single center. Methods: Height data were obtained by retrospective chart review of 10 Korean patients with MPS I who had received ERT for a minimum of 3 years. Height was expressed as standard deviation scores (SDS) based on normative data. Annual growth rates were calculated before and during ERT. A piecewise regression model was used to analyze height z-scores before and after treatment. Individual analysis was performed for impact of phenotype [(severe (Hurler) versus attenuated (Hurler-Scheie, Scheie)] on growth. Results: Annual growth was 3.3 cm (z-score= -0.21) in the year before ERT and 6.2 cm (z-score= 0.17), 5.8 cm (z-score= 0.07), and 3.8 cm (z-score= -0.4) in the first, second, and third years of ERT, respectively. Regression analysis showed improvement in the slope after ERT (difference= 0.04; P=0.022). Estimated slope differences between severe and attenuated phenotypes were statistically significant before (P=0.001) and after treatment (P<0.0001), although no significant difference was noted when stratified by phenotype. Conclusion: ERT with aldurazyme appears to have a positive impact on linear growth in patients with MPS I.

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The Design and Implementation of a Real-Time Database Engine For Embedded Systems (내장형 시스템을 위한 실시간 데이터베이스 엔진 설계 및 구현)

  • 김흥섭;문승진
    • Journal of Internet Computing and Services
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    • v.3 no.5
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    • pp.19-29
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    • 2002
  • With the progress of information-oriented society, many device with advanced technologies invented by many companies. However, the current firmware technologies have many problems to meet such high level of new Technologies, Recently it become necessary new manufactures of information-oriented society to require such embedded operating system as their system-level platform. Embedded linux, which could be on alternative proposal of existing high-cost embedded operating system, become available commercially by many companies Linux has many programs, But, embedded databases require very high cost. In this paper, we suggest ERT DE which has a small size and is suitable for embedded real-time technologies.

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QoS Packet-Scheduling Scheme for VoIP Services in IEEE 802.16e Systems

  • Jang, Jae-Shin;Lee, Jong-Hyup;Cheong, Seung-Kook;Kim, Young-Sun
    • Journal of Communications and Networks
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    • v.11 no.1
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    • pp.36-41
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    • 2009
  • The IEEE 802.16 wireless metropolitan area network (WMAN) standard is designed to correct expensive communication costs in CDMA-based mobile communication systems and limited coverage problems in wireless LAN systems. Thus, the IEEE 802.16e standard can provide mobile high-speed packet access between mobile stations and the Internet service provider through the base station with cheap communication fees. To efficiently accommodate voice over IP (VoIP) services in IEEE 802.16 systems, an uplink quality of service packet-scheduling scheme is proposed, and its performance is evaluated with an NS-2 network simulator in this paper. Numerical results show that this proposed scheme can increase the system capacity by 100% more than in the unsolicited rand service (UGS) scheme and 30% more than the extended real-time polling service (ertPS) scheme, respectively.

Nondestructive Imaging of Subspace Objects by 2D Electrical Resistance Tomography (2차원 전기비저항토모그래피를 이용한 지하물체의 비파괴 영상화)

  • Kim, Ho-Chan;Boo, Chang-Jin;Kim, Se-Ho;Jwa, Chong-Keun;Oh, Seong-Bo;Ko, Bong-Woon;Kim, Moon-Chan;Kim, Yong-Seok
    • Proceedings of the KIEE Conference
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    • 2005.07d
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    • pp.2619-2621
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    • 2005
  • Electrical resistance tomography(ERT) maps resistivity values of the soil subsurface and characterizes buried objects. The characterization includes location, size, and resistivity of buried objects. In this paper, Gauss-Newton and truncated least squares(TLS) are presented for the solution of the ERT image reconstruction. Computer simulations show that the spatial resolution of the reconstructed images by the TLS approach is improved as compared to that obtained by the Gauss-Newton method.

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Image Reconstruction with Prior Information in Electrical Resistance Tomography

  • Kim, Bong Seok;Kim, Sin;Kim, Kyung Youn
    • Journal of IKEEE
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    • v.18 no.1
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    • pp.8-18
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    • 2014
  • Electrical resistance tomography (ERT) has high temporal resolution characteristics therefore it is used as an alternative technique to visualize two-phase flows. The image reconstruction in ERT is highly non-linear and ill-posed hence it suffers from poor spatial resolution. In this paper, the inverse problem is solved with homogeneous data used as a prior information to reduce the condition number of the inverse algorithm and improve the spatial resolution. Numerical experiments have been carried out to illustrate the performance of the proposed method.

Numerical and laboratory investigations of electrical resistance tomography for environmental monitoring

  • Heinson Tania Dhu Graham
    • Geophysics and Geophysical Exploration
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    • v.7 no.1
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    • pp.33-40
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    • 2004
  • Numerical and laboratory studies have been conducted to test the ability of Electrical Resistance Tomography-a technique used to map the electrical resistivity of the subsurface-to delineate contaminant plumes. Two-dimensional numerical models were created to investigate survey design and resolution. Optimal survey design consisted of both downhole and surface electrode sites. Resolution models revealed that while the bulk fluid flow could be outlined, small-scale fingering effects could not be delineated. Laboratory experiments were conducted in a narrow glass tank to validate theoretical models. A visual comparison of fluid flow with ERT images also showed that, while the bulk fluid flow could be seen in most instances, fine-scale effects were indeterminate.

Long-Term Clinical Course of a Korean Patient with Chronic Neuropathic (type III) Gaucher Disease

  • Lee, Jun Hwa
    • Journal of Interdisciplinary Genomics
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    • v.1 no.2
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    • pp.15-18
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    • 2019
  • Gaucher disease (GD) is an autosomal recessive inborn error of metabolism resulting from a deficiency in ${\beta}$-glucocerebrosidase (GBA) activity that leads to the accumulation of glucocerebroside in macrophages in multiple organs, such as the bone marrow, liver, spleen, and brain. GD can be classified into three clinical types: type 1 (non-neuropathic form, OMIM #230800); type II (acute neuropathic form, OMIM #230900); and type III (chronic neuropathic form, OMIM #231000). Type III is the subacute form of neuropathic GD. The best available treatment for GD is long-term enzyme (imiglucerase) replacement therapy (ERT) performed every two weeks. This report describes the long-term clinical course of a patient with type III GD who was treated with ERT for 18 years.

A Study on Two-Dimensional Variational Mode Decomposition Applied to Electrical Resistivity Tomography

  • Sanchez, Felipe Alberto Solano;Khambampati, Anil Kumar;Kim, Kyung Youn
    • Journal of IKEEE
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    • v.26 no.3
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    • pp.475-482
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    • 2022
  • Signal pre-processing and post-processing are some areas of study around electrical resistance tomography due to the low spatial resolution of pixel-based reconstructed images. In addition, methods that improve integrity and noise reduction are candidates for application in ERT. Lately, formulations of image processing methods provide new implementations and studies to improve the response against noise. For example, compact variational mode decomposition has recently shown good performance in image decomposition and segmentation. The results from this first approach of C-VMD to ERT show an improvement due to image segmentation, providing filtering of noise in the background and location of the target.