Longitudinal study on ocular manifestations in a cohort of patients with Fabry disease

Purpose This study aims to assess the evolution of ocular manifestations in a cohort of Fabry patients. Methods This is a prospective observational study conducted from 2013 to 2017 (5 consecutive exams). All subjects underwent a comprehensive ocular examination including oriented case history, refraction, corneal topography, biomechanical corneal properties and pachometry assessments, aberrometry, anterior segment evaluation, double-frequency visual field (FDT), intra-ocular pressure, and ocular fundus. At baseline, 41 subjects enrolled but 9 dropped-out and 4 files were not kept for analysis (missing data). Remaining 28 subjects were classified into: Group 1 -hemizygotes (HMZ), all on enzyme replacement therapy (ERT) (N = 10); Group 2 -heterozygotes (HTZ) actively ERT-treated (N = 8), and Group 3 -HTZ not treated (N = 10). Results There is a high intra and inter-subjects variability. At baseline, prevalence of the ocular manifestations found is similar to published data: cornea verticillata (89.2%), conjunctival vessels tortuosity (85.7%), corneal haze (67.8%), retinal vessels tortuosity (64.2%), anterior cataract (39.2%) and posterior cataract (28.5%). Prevalence for new elements are found: upper lid vessels toricity (96.4%) and micro-aneurysms (42.8%). At the end, micro-aneurysms (+82%), posterior cataract (+75%) corneal haze (+21%) anterior cataract (+17%) and retinal vessels tortuosities (+4%) evolved in prevalence and severity despite the fact that 68% of the patients were on ERT. Treated heterozygotes evolved more than other groups (p>0.05). Conclusion ERT does not seem to halt the clinical evolution of several ocular manifestations. Longer observational time and objective grading systems may be required to fully confirm these findings

Recent Advances in Signal Processing

The signal processing task is a very critical issue in the majority of new technological inventions and challenges in a variety of applications in both science and engineering fields. Classical signal processing techniques have largely worked with mathematical models that are linear, local, stationary, and Gaussian. They have always favored closed-form tractability over real-world accuracy. These constraints were imposed by the lack of powerful computing tools. During the last few decades, signal processing theories, developments, and applications have matured rapidly and now include tools from many areas of mathematics, computer science, physics, and engineering. This book is targeted primarily toward both students and researchers who want to be exposed to a wide variety of signal processing techniques and algorithms. It includes 27 chapters that can be categorized into five different areas depending on the application at hand. These five categories are ordered to address image processing, speech processing, communication systems, time-series analysis, and educational packages respectively. The book has the advantage of providing a collection of applications that are completely independent and self-contained; thus, the interested reader can choose any chapter and skip to another without losing continuity

섬사상 박테리오파지의 분석∙전기분석적 응용 및 역전기투석을 이용한 민감도 조절 가능 이온 측정 플랫폼의 개발

학위논문(박사)--서울대학교 대학원 :자연과학대학 화학부,2019. 8. 정택동.Chapter 1: Analytical and Electroanalytical Applications of Filamentous Bacteriophages Filamentous bacteriophage is one of the most highlighted biomaterial, thanks to its unique properties: 1) Easy modification of its major coat protein with genetic engineering and bioconjugation, 2) The regularity of the virions along with large population and fast generation time, and 3) The high aspect ratio, 7 nm in width and 880 nm in length, which make it an excellent biomaterial for nanomaterial applications. First, filamentous bacteriophages are applied as a scaffold of antibody immobilization for bead-based 3D suspension multiplex immunoassay. The size and the fluorescence signal of virus-tethered gold microbead is measured at a DC impedance-based flow cytometer. The large quantity of antibody loading and flexible movement of filamentous virus enabled signal enhancement, elevating sensitivity by up to 5.7-fold compared to the beads without virus. This system successfully accomplished multiplex immunoassay involving four biomarkers: prostate specific antigen, cardiac troponin I, myoglobin, and creatine kinase MB in undiluted human serum. Second, filamentous bacteriophages are genetically engineered to display 3,4-dihydroxyl-L-phenylalanine (DOPA) onto the major coat protein, where cobalt oxide-based oxygen evolution catalyst (Co-OEC) are deposited spontaneously. It was demonstrated that the catalyst formed on the virus shows improved durability and decrease oxidation state compared with electrodeposited Co-OEC. The DOPA-displaying virus proposes unconventional way of generating electrocatalysts by introducing DOPA as a nucleation site as well as a dopant for Co-OEC. Chapter 2: Development of Sensitivity-Tunable Ion Sensing Platform based on Reverse Electrodialysis Reverse elecrodialysis (RED) is a salinity gradient-based power generation system, of which the output voltage is proportional to the 1) concentration difference between river water and seawater, and 2) the number of stacks comprising RED. Inspired by those features mentioned above, RED-based ion sensing platform is proposed. The cation exchange membranes and anion exchange membranes constituting traditional RED are substituted to home-made ion- selective membranes and reference membranes, which generate sample dependent and independent potential, respectively, and the sensitivity is freely modulated by changing the number of stacks. This sensing platform is integrated with a colorimetric detection system based on polyaniline, which is a well-known electrochromic dye, electrodeposited on one bipolar electrode to display sample concentration-dependent colors. Combined with the colorimetric detection system, the RED-based ion sensing platform serves as a power generator as well as an ion concentration selective signal producer, which realize self-powered and disposable ion sensor with tunable sensitivityAbstract i Table of contents iii List of Tables v List of Figures v Chapter 1. Analytical and Electroanalytical Applications of Filamentous Bacteriophages 1 1.1 Introduction 1 1.2. Multiplex immunoassay using virus-tethered gold micro-bead by DC impedance-based cytometry 2 1.2.1. Introduction 2 1.2.2. Experimental 5 1.2.3. Result and Discussion 11 1.2.4. Conclusion 23 1.3. Chemically Deposited Cobalt-Based Oxygen-Evolution Electrocatalysts on DOPA-Displaying Filamentous Bacteriophages 25 1.3.1. Introduction 25 1.3.2. Experimental 26 1.3.3. Result and Discussion 32 1.3.4. Conclusion 46 1.4. Genetic engineering of filamentous bacteriophage (fd) for the application in SERS active probe. 47 1.4.1. Introduction 47 1.4.2. Experimental 48 1.4.3. Result and Discussion 51 1.4.4. Conclusion 59 1.5. References 60 Chapter 2. Development of Sensitivity-Tunable Ion Sensing Platform based on Reverse Electrodialysis 65 2.1. Introduction 65 2.2. Experimental 66 2.3. Result and Discussion 71 2.4. Conclusion 95 2.5. References 96 국문초록 99Docto

CRFやAMPA受容体シグナルを介した精神疾患様行動の制御に関するin vivo 研究

筑波大学 (University of Tsukuba)201

Geodetic Sciences

Space geodetic techniques, e.g., global navigation satellite systems (GNSS), Very Long Baseline Interferometry (VLBI), satellite gravimetry and altimetry, and GNSS Reflectometry & Radio Occultation, are capable of measuring small changes of the Earth�s shape, rotation, and gravity field, as well as mass changes in the Earth system with an unprecedented accuracy. This book is devoted to presenting recent results and development in space geodetic techniques and sciences, including GNSS, VLBI, gravimetry, geoid, geodetic atmosphere, geodetic geophysics and geodetic mass transport associated with the ocean, hydrology, cryosphere and solid-Earth. This book provides a good reference for geodetic techniques, engineers, scientists as well as user community

Hopkinton annual town and school report 2012.

This is an annual report containing vital statistics for a town/city in the state of New Hampshire

Hopkinton annual town and school report 2012.

This is an annual report containing vital statistics for a town/city in the state of New Hampshire

Annual reports of the town of Gilford, New Hampshire for the year ending December 31, 2013.

This is an annual report containing vital statistics for a town/city in the state of New Hampshire

Annual reports of the town of Gilford, New Hampshire for the year ending December 31, 2013.

This is an annual report containing vital statistics for a town/city in the state of New Hampshire