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Kunpeng Feng, Jiwen Cui, Hong Dang, Weidong Wu, Xun Sun, Xuelin Jiang, Jiubin Tan
To achieve a narrow bandwidth optical filter with a wide swept range for new generation optical spectrum analysis (OSA) of high performance optical sensors, an optoelectronic equivalent narrowband filter (OENF) was investigated and a swept optical filter with bandwidth of several MHz and sweep range of several tens of nanometers was built using electric filters and a sweep laser as local oscillator (LO). The principle of OENF is introduced and analysis of the OENF system is presented. Two electric filters are optimized to be RBW filters for high and medium spectral resolution applications...
February 10, 2017: Sensors
Zhihao Yu, Zhipeng Tian, Anbo Wang
In this Letter, we present the design of a simple signal interrogator for optical fiber-based white light Fabry-Perot (F-P) interferometers. With the hardware being composed of only a flat fused silica wafer and a CCD camera, this interrogator translates the spectral interference into a spatial interference pattern, and then demodulates the F-P cavity length with the use of a relatively simple demodulation algorithm. The concept is demonstrated experimentally in a fiber optic sensor with a sapphire wafer as the F-P cavity...
February 15, 2017: Optics Letters
Aida Oliván-Viguera, Javier Lozano-Gerona, Laura López de Frutos, Jorge J Cebolla, Pilar Irún, Edgar Abarca-Lachen, Ana J García-Malinis, Ángel Luis García-Otín, Yolanda Gilaberte, Pilar Giraldo, Ralf Köhler
The calcium/calmodulin-gated KCa3.1 channel regulates normal and abnormal mitogenesis by controlling K(+)-efflux, cell volume, and membrane hyperpolarization-driven calcium-entry. Recent studies suggest modulation of KCa3.1 by omega-3 fatty acids as negative modulators and impaired KCa3.1 functions in the inherited lysosomal storage disorder (LSD), Fabry disease (FD). In the first part of present study, we characterize KCa3.1 in murine and human fibroblasts and test the impact of omega-3 fatty acids on fibroblast proliferation...
2017: Frontiers in Physiology
Changfu Feng, Zhenzhen Xu, Xu Wang, Hongjuan Yang, Lemin Zheng, Hongbing Fu
Development of luminescence probes with polarized and narrow emissions simultaneously are helpful to remove multiply scattered light and enable multiplexing detection, but remain challengeable by using conventional organic dyes, fluorescence proteins and quantum dots. Here, we demonstrated a smart one-dimensional microlaser probes (MLPs) by coating a thin layer of silica shell on the surface of organic nanowires (ONWs) of 1,4-dimethoxy-2,5-di[4'-(methylthio)styryl]benzene (TDSB), namely, ONW@SiO2 core-shell nanostructures...
February 10, 2017: ACS Applied Materials & Interfaces
Eungu Kang, Yoon-Myung Kim, Dae-Hee Kim, Han-Wook Yoo, Beom Hee Lee
RATIONALE: Angiokeratomas are the earliest manifestation of Fabry disease (FD), and the extent of their appearance is related to disease severity. Angiokeratomas are mostly found on cutaneous regions. PATIENT CONCERNS, DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Here we report an FD patient with widespread gastrointestinal angiokeratomas who developed life-threatening bleeding following anticoagulation for atrial fibrillation. LESSONS: Careful observation for gastrointestinal bleeding is warranted for patients on anticoagulation with extensive cutaneous angiokeratomas...
February 2017: Medicine (Baltimore)
Alessandra Mauri, Susanna Schmidt, Valentina Sosero, Maria Sambataro, Laura Nollino, Francesco Fabris, Anna Corò, Antonella Scantamburlo, Michela Cazziola-Merlotto, Tania Ciani, Michele Tessarin, Agostino Paccagnella
BACKGROUND: Therapeutic education for Type 1 Diabetes involves the process of transmitting knowledge and developing the skills and behavior required to treat the disease. guidelines agree on stressing the importance of therapeutic educational intervention in teaching self-management skills to children and adolescents with Type 1 Diabetes (T1D). This study presents the results of the "Pediatric Education for Type 1 Diabetes (T1D)" (PED) project, specifically designed for children and adolescents aged 6 to 16, and structured on guidelines indications, as part of a broader clinical-educational intervention for Type 1 diabetes...
February 7, 2017: Minerva Pediatrica
Gianfranco Sinagra, Enrico Fabris, Luigi Tavazzi
The field of cardiovascular medicine continues to rapidly evolve in both diagnostic and therapeutic arenas. Subspecialization is required to keep pace with evolving practice; however, a multidisciplinary approach in a framework of cost-benefit care will be equally crucial in the management of increasing numbers of complex patients. Information technology and translational medicine may radically change clinical research and practice. It is important to consider the evolution of medicine and reflect on it in order to set the right course to achieve patient-centered goals...
February 7, 2017: Future Cardiology
Han-Jui Lee, Ting-Rong Hsu, Sheng-Che Hung, Wen-Chung Yu, Tzu-Hung Chu, Chia-Feng Yang, Svetlana Bizjajeva, Chui-Mei Tiu, Dau-Ming Niu
BACKGROUND: Patients with the later-onset IVS4+919G>A (IVS4) Fabry mutation are known to have positive central nervous system involvement compared with age- and sex-matched controls. This study compares central nervous system manifestations in patients with the IVS4 mutation or classical Fabry mutations. METHODS: This was a retrospective analysis of magnetic resonance imaging (MRI) data from Taiwanese patients enrolled in the Fabry Outcome Survey (sponsored by Shire; data extracted March 2015)...
February 6, 2017: BMC Neurology
Douglas Fabris, Júlio C M Souza, Filipe S Silva, Márcio Fredel, Joana Mesquita-Guimarães, Yu Zhang, Bruno Henriques
Layered ceramic systems are usually hit by residual thermal stresses created during cooling from high processing temperature. The purpose of this study was to determine the thermal residual stresses at different ceramic multi-layered systems and evaluate their influence on the bending stress distribution. Finite elements method was used to evaluate the residual stresses in zirconia-porcelain and alumina-porcelain multi-layered discs and to simulate the 'piston-on-ring' test. Temperature-dependent material properties were used...
March 2017: Ceramics International
Yohei Sato, Hiroyuki Ida, Toya Ohashi
Formation of antibodies against a therapeutic enzyme is an important complication during enzyme replacement therapy (ERT) for lysosomal storage diseases. Fabry disease (FD) is caused by a deficiency of alpha-galactosidase (GLA), which results in the accumulation of globotriaosylceramide (GL-3). We have shown immune tolerance induction (ITI) during ERT in FD model mice by using an anti-B lymphocyte stimulator (anti-BlyS) antibody (belimumab). A single dose of the anti-BlyS antibody temporarily lowered the percentage of B cells and IgG antibody titer against recombinant human GLA...
February 2, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Jin-Song Shen, Erland Arning, Michael L West, Taniqua S Day, Shuyuan Chen, Xing-Li Meng, Sabrina Forni, Nathan McNeill, Ozlem Goker-Alpan, Xuan Wang, Paula Ashcraft, David F Moore, Seng H Cheng, Raphael Schiffmann, Teodoro Bottiglieri
No abstract text is available yet for this article.
February 2, 2017: Human Molecular Genetics
Christoph Reinhardt, Tina Müller, Jack C Sankey
We present a robust sideband laser locking technique ideally suited for applications requiring low probe power and heterodyne readout. By feeding back to a high-bandwidth voltage-controlled oscillator, we lock a first-order phase-modulation sideband to a high-finesse Fabry-Perot cavity in ambient conditions, achieving a closed-loop bandwidth of 3.5 MHz (with a single integrator) limited fundamentally by the signal delay. The measured transfer function of the closed loop agrees with a simple model based on ideal system components, and from this we suggest a modified design that should achieve a bandwidth exceeding 6 MHz with a near-causally limited feedback gain as high as 4 × 10<sup>7</sup> at 1 kHz...
January 23, 2017: Optics Express
Zhongtao Cheng, Dong Liu, Yupeng Zhang, Chong Liu, Jian Bai, Dan Wang, Nanchao Wang, Yudi Zhou, Jing Luo, Yongying Yang, Yibing Shen, Lin Su, Liming Yang
High-spectral-resolution lidar (HSRL) is a powerful tool for atmospheric aerosol remote sensing. The current HSRL technique often requires a single longitudinal mode laser as the transmitter to accomplish the spectral discrimination of the aerosol and molecular scattering conveniently. However, single-mode laser is cumbersome and has very strict requirements for ambient stability, making the HSRL instrument not so robust in many cases. In this paper, a new HSRL concept, called generalized HSRL technique with a multimode laser (MML-gHSRL), is proposed, which can work using a multimode laser...
January 23, 2017: Optics Express
Wonsuk Lee, Wenjie Wang, Guksik Lee, Seong Ho Ryu, Xudong Fan, Dong Ki Yoon
We demonstrate an electro-tunable laser based on a plano-concave (p-c) Fabry-Pérot (FP) microcavity filled with dye-doped liquid crystal (LC) gain material. Owing to the high Q-factor of the p-c FP microcavity, the lasing threshold of the LC laser is as low as 0.58 µJ/mm<sup>2</sup>. The single-mode emission wavelength can be easily tuned by varying applied voltage, which induces the reconfiguration of LC molecules. Furthermore, this lasing platform operates at room temperature, thanks to the wide temperature range of the nematic LC...
January 23, 2017: Optics Express
Yiping Wang, Xiaoqi Ni, Ming Wang, Yifeng Cui, Qingyun Shi
In this paper, a demodulation method for optic fiber micro-electromechanical systems (MEMS) extrinsic Fabry-Perot interferometer (EFPI) pressure sensor exploiting microwave photonics filter technique is firstly proposed and experimentally demonstrated. A single bandpass microwave photonic filter (MPF) which mainly consists of a spectrum-sliced light source, a pressurized optical fiber MEMS EFPI, a phase modulator (PM) and a length of dispersion compensating fiber (DCF) is demonstrated. The frequency response of the filter with respect to the pressure is studied...
January 23, 2017: Optics Express
Jennifer Juhl Majersik
PURPOSE OF REVIEW: This article is a practical guide to identifying uncommon causes of stroke and offers guidance for evaluation and management, even when large controlled trials are lacking in these rarer forms of stroke. RECENT FINDINGS: Fabry disease causes early-onset stroke, particularly of the vertebrobasilar system; enzyme replacement therapy should be considered in affected patients. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), often misdiagnosed as multiple sclerosis, causes migraines, early-onset lacunar strokes, and dementia...
February 2017: Continuum: Lifelong Learning in Neurology
Nannan Yang, Xia Wang, Feng Xu, Caihong Zeng, Jinquan Wang, Zhihong Liu
AIMS: To improve diagnosis and treatment, we characterized Fabry disease combined with IgA nephropathy and its response to treatment clinically and pathologically in Chinese patients. MATERIALS AND METHODS: Clinical and pathological characteristics of 6 Chinese patients with renal biopsy-proven Fabry disease combined with IgA nephropathy were retrospectively analyzed. RESULTS: There were 4 males and 2 females in this study. All of the 6 patients presented with proteinuria...
February 3, 2017: Clinical Nephrology
Olga Azevedo, Miguel Gago, Gabriel Miltenberger-Miltenyi, Paulo Gaspar, Nuno Sousa, Damião Cunha
We report on the clinical, biochemical, and genetic findings of a large family with the classical phenotype of Fabry disease due to the novel nonsense mutation c.607G>T (p.E203X) of the GLA gene, which occurs in the active site of the α-galactosidase A enzyme. This report highlights that (i) Fabry disease diagnosis should be considered in all cases of unexplained left ventricular hypertrophy (LVH), even in its milder forms; (ii) a complete evaluation of patients with unexplained LVH is important to find diagnostic red flags of treatable causes of LVH, such as Fabry disease; (iii) cascade family screening is paramount to the earlier diagnosis and treatment of other affected family members; and (iv) the Fabry disease phenotype is highly variable in heterozygote females, even within the same family...
February 3, 2017: Cardiology
Juan Politei, Graciela Remondino, Ricardo Heguilen, Eric Wallace, Consuelo Durand, Andrea Schenone
The presence of distal extremity pain in children and adolescents usually triggers the search of rheumatologic diseases without considering non-rheumatologic causes of joint pain. Approaching distal extremity pain with a complete differential diagnosis, including non-rheumatologic entities, may hasten diagnosis, thus decreasing cost and aiding in earlier initiation of appropriate therapy. To present a case of a patient who after years of work up of arthralgia, which was actually attributed to rheumatologic causes, had an inherited metabolic disease...
December 2016: European Journal of Rheumatology
Qi Zhang, Yupeng Zhu, Xiangyu Luo, Guigen Liu, Ming Han
We demonstrate a fiber-optic acoustic emission (AE) sensor system that is capable of performing AE detection, even when the sensor is experiencing large quasi-static strains. The sensor is a Fabry-Perot interferometer formed by cascaded chirped fiber-Bragg gratings (CFBGs). The reflection spectrum of the sensor features a number of narrow spectral notches equally spaced within the reflection bandwidth of the CFBG. A semiconductor laser whose wavelength can be fast tuned through current injection is used to lock the laser line to the center of a slope of a spectral notch...
February 1, 2017: Optics Letters
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