keyword
MENU ▼
Read by QxMD icon Read
search

Fabry's

keyword
https://www.readbyqxmd.com/read/29341286/test-1-analyzer-and-conventional-westergren-method-for-erythrocyte-sedimentation-rate-a-comparative-study-between-two-laboratories
#1
Cigdem Sonmez, Ozlem Ceylan Dogan, Aysegul Ozturk Kaymak, Nedim Akkaya, Kadir Okhan Akin, Gulcan Guntas
BACKGROUND: Measurement of the length of sedimentation reaction in blood (LSRB), also called erythrocyte sedimentation rate (ESR), is a widely used hematology test. This study intends to compare ESR levels measured by Test-1 method and International Council for Standardization in Hematology's (ICSH) reference method, and analyzes the effect of hematocrit (Hct) on ESR results. MATERIAL AND METHODS: A total of 755 patients from 2 hospitals were included in the study, and samples with EDTA were studied by Test-1 method for ESR measurement and total blood count, whereas citrated samples were studied with reference Westergren method...
January 17, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29340480/bone-repair-access-of-boneceramic%C3%A2-in-5-mm-defects-study-on-rat-calvaria
#2
André Luis da Silva Fabris, Leonardo Perez Faverani, Pedro Henrique Silva Gomes-Ferreira, Tárik Ocon Braga Polo, Joel Ferreira Santiago-Júnior, Roberta Okamoto
OBJECTIVE: The aim of this study was to evaluate the osteoconductive potential of BoneCeramic™ on bone healing in rat calvaria 5-mm defects. MATERIAL AND METHODS: A 5-mm calvaria bone defect was induced in three groups and the defect was not filled with biomaterial [Clot Group (CG)], autogenous bone (AG), or Bone Ceramic Group (BCG). Animals were euthanized after 14 or 28 days and the bone tissue within the central area of the bone defect was evaluated. Results were compared using ANOVA and Tukey test (p<0...
January 15, 2018: Journal of Applied Oral Science: Revista FOB
https://www.readbyqxmd.com/read/29336944/editorial-commentary-newborn-screening-for-fabry-disease-too-much-too-soon
#3
EDITORIAL
M Langeveld
No abstract text is available yet for this article.
January 5, 2018: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29330455/towards-electrotuneable-nanoplasmonic-fabry-perot-interferometer
#4
Hayley Weir, Joshua B Edel, Alexei A Kornyshev, Debabrata Sikdar
Directed voltage-controlled assembly and disassembly of plasmonic nanoparticles (NPs) at electrified solid-electrolyte interfaces (SEI) offer novel opportunities for the creation of tuneable optical devices. We apply this concept to propose a fast electrotuneable, NP-based Fabry-Perot (FP) interferometer, comprising two parallel transparent electrodes in aqueous electrolyte, which form the polarizable SEI for directed assembly-disassembly of negatively charged NPs. An FP cavity between two reflective NP-monolayers assembled at such interfaces can be formed or deconstructed under positive or negative polarization of the electrodes, respectively...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29330335/fabry-disease-prevalence-of-affected-males-and-heterozygotes-with-pathogenic-gla-mutations-identified-by-screening-renal-cardiac-and-stroke-clinics-1995-2017
#5
Dana Doheny, Ram Srinivasan, Silvere Pagant, Brenden Chen, Makiko Yasuda, Robert J Desnick
BACKGROUND: Fabry Disease (FD), an X linked lysosomal storage disease due to pathogenic α-galactosidase A (GLA) mutations, results in two major subtypes, the early-onset Type 1 'Classic' and the Type 2 'Later-Onset' phenotypes. To identify previously unrecognised patients, investigators screened cardiac, renal and stroke clinics by enzyme assays. However, some screening studies did not perform confirmatory GLA mutation analyses, and many included recently recognised 'benign/likely-benign' variants, thereby inflating prevalence estimates...
January 12, 2018: Journal of Medical Genetics
https://www.readbyqxmd.com/read/29329225/optical-fiber-demodulation-system-with-high-performance-for-assessing-fretting-damage-of-steam-generator-tubes
#6
Peijian Huang, Ning Wang, Junying Li, Yong Zhu, Jie Zhang, Zhide Xi
In order to access the fretting damage of the steam generator tube (SGT), a fast fiber Fabry-Perot (F-P) non-scanning correlation demodulation system based on a super luminescent light emitting diode (SLED) was performed. By demodulating the light signal coming out from the F-P force sensor, the radial collision force between the SGT and the tube support plate (TSP) was interrogated. For higher demodulation accuracy, the effects of the center wavelength, bandwidth, and spectrum noise of SLED were discussed in detail...
January 12, 2018: Sensors
https://www.readbyqxmd.com/read/29329221/high-temperature-sensor-based-on-fabry-perot-interferometer-in-microfiber-tip
#7
Zhenshi Chen, Songsong Xiong, Shecheng Gao, Hui Zhang, Lei Wan, Xincheng Huang, Bingsen Huang, Yuanhua Feng, Weiping Liu, Zhaohui Li
A miniaturized tip Fabry-Perot interferometer (tip-FPI) is proposed for high-temperature sensing. It is simply fabricated for the first time by splicing a short length of microfiber (MF) to the cleaved end of a standard single mode fiber (SMF) with precise control of the relative cross section position. Such a MF acts as a Fabry-Perot (FP) cavity and serves as a tip sensor. A change in temperature modifies the length and refractive index of the FP cavity, and then a corresponding change in the reflected interference spectrum can be observed...
January 12, 2018: Sensors
https://www.readbyqxmd.com/read/29328225/transversely-coupled-fabry-perot-resonators-with-bragg-grating-reflectors
#8
Md Ghulam Saber, Yun Wang, Eslam El-Fiky, David Patel, Kh Arif Shahriar, Md Samiul Alam, Maxime Jacques, Zhenping Xing, Luhua Xu, Nicolás Abadía, David V Plant
We design and demonstrate Fabry-Perot resonators with transverse coupling using Bragg gratings as reflectors on the silicon-on-insulator (SOI) platform. The effects of tailoring the cavity length and the coupling coefficient of the directional coupler on the spectral characteristics of the device are studied. The fabricated resonators achieved an extinction ratio (ER) of 37.28 dB and a Q-factor of 3356 with an effective cavity length of 110 μm, and an ER of 8.69 dB and a Q-factor of 23642 with a 943 μm effective cavity length...
January 1, 2018: Optics Letters
https://www.readbyqxmd.com/read/29328185/optical-fiber-humidity-sensor-based-on-the-direct-response-of-the-polyimide-film
#9
Ce Bian, Manli Hu, Ruohui Wang, Tingting Gang, Rongxin Tong, Lisong Zhang, Teng Guo, Xiaobo Liu, Xueguang Qiao
An optical fiber humidity sensor based on an optical Fabry-Perot interferometer is proposed and experimentally demonstrated. The sensor is constructed by a short section of hollow-core fiber coated with a polyimide (PI) film. Taking advantage of the direct response of the PI film, a sensitivity of up to 1.309 nm/%RH can be achieved in the humidity change range from 40% RH to 80% RH. The temperature sensitivity is measured to be 43.57 pm/°C when the temperature changes from 25°C to 55°C. Because of its simple structure, fast response time, convenient production, and good reproducibility, the proposed sensor will be competitive in the field of cultural relic humidity monitoring and pharmaceutical storage...
January 10, 2018: Applied Optics
https://www.readbyqxmd.com/read/29326878/ten-year-long-enzyme-replacement-therapy-shows-a-poor-effect-in-alleviating-giant-leg-ulcers-in-a-male-with-fabry-disease
#10
Jun Okada, Mohammad Arif Hossain, Chen Wu, Takashi Miyajima, Hiroko Yanagisawa, Keiko Akiyama, Yoshikatsu Eto
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A (α-gal A), leading to the progressive accumulation of glycosphingolipids. Classical hemizygous males usually present symptoms, including pain and paresthesia in the extremities, angiokeratoma, hypo- or anhidrosis, abdominal pain, cornea verticillata, early stroke, tinnitus, and/or hearing loss, during early childhood or adolescence. Moreover, proteinuria, renal impairment, and cardiac hypertrophy can appear with age...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29326873/motor-involvement-in-fabry-disease
#11
Sirio Cocozza, Lorenzo Ugga, Giuseppe Pontillo, Camilla Russo, Enrico Tedeschi, Antonio Pisani, Arturo Brunetti
No abstract text is available yet for this article.
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325843/preoperative-intra-aortic-counterpulsation-in-cardiac-surgery-insights-from-a-retrospective-series-of-588-consecutive-high-risk-patients
#12
Giuseppe Gatti, Laura Morra, Gianluca Castaldi, Luca Maschietto, Florida Gripshi, Enrico Fabris, Andrea Perkan, Bernardo Benussi, Gianfranco Sinagra, Aniello Pappalardo
OBJECTIVE: To support a rational use of preoperative intra-aortic balloon pump (IABP) in cardiac surgery. DESIGN: Retrospective, observational study. SETTING: Single university hospital. PARTICIPANTS: The study included 588 (mean age 68.5 ± 9.6 yr) consecutive patients who received IABP before cardiac surgery from 1999 to 2016. INTERVENTIONS: Coronary surgery was performed in 573 (97.4%) cases...
December 6, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29324715/nitrogen-doped-diamond-film-for-optical-investigation-of-hemoglobin-concentration
#13
Daria Majchrowicz, Monika Kosowska, Kamatchi J Sankaran, Przemysław Struk, Michał Wąsowicz, Michał Sobaszek, Ken Haenen, Małgorzata Jędrzejewska-Szczerska
In this work we present the fabrication and characterization of a diamond film which can be utilized in the construction of optical sensors for the investigation of biological samples. We produced a nitrogen-doped diamond (NDD) film using a microwave plasma enhanced chemical vapor deposition (MWPECVD) system. The NDD film was investigated with the use of scanning electron microscopy (SEM), atomic force microscopy (AFM) and Raman spectroscopy. The NDD film was used in the construction of the fiber optic sensor...
January 11, 2018: Materials
https://www.readbyqxmd.com/read/29324648/lspr-and-interferometric-sensor-modalities-combined-using-a-double-clad-optical-fiber
#14
Harald Ian Muri, Andon Bano, Dag Roar Hjelme
We report on characterization of an optical fiber-based multi-parameter sensor concept combining localized surface plasmon resonance (LSPR) signal and interferometric sensing using a double-clad optical fiber. The sensor consists of a micro-Fabry-Perot in the form of a hemispherical stimuli-responsive hydrogel with immobilized gold nanorods on the facet of a cleaved double-clad optical fiber. The swelling degree of the hydrogel is measured interferometrically using the single-mode inner core, while the LSPR signal is measured using the multi-mode inner cladding...
January 11, 2018: Sensors
https://www.readbyqxmd.com/read/29324338/identification-of-novel-2-benzoxazolinone-derivatives-with-specific-inhibitory-activity-against-the-hiv-1-nucleocapsid-protein
#15
Elia Gamba, Mattia Mori, Lesia Kovalenko, Alessia Giannini, Alice Sosic, Francesco Saladini, Dan Fabris, Yves Mély, Barbara Gatto, Maurizio Botta
In this report, we present a new benzoxazole derivative endowed with inhibitory activity against the HIV-1 nucleocapsid protein (NC). NC is a 55-residue basic protein with nucleic acid chaperone properties, which has emerged as a novel and potential pharmacological target against HIV-1. In the pursuit of novel NC-inhibitor chemotypes, we performed virtual screening and in vitro biological evaluation of a large library of chemical entities. We found that compounds sharing a benzoxazolinone moiety displayed putative inhibitory properties, which we further investigated by considering a series of chemical analogues...
December 24, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29320610/fabry-p%C3%A3-rot-oscillation-and-room-temperature-lasing-in-perovskite-cube-corner-pyramid-cavities
#16
Yang Mi, Zhixiong Liu, Qiuyu Shang, Xinxiang Niu, Jia Shi, Shuai Zhang, Jie Chen, Wenna Du, Zhiyong Wu, Rui Wang, Xiaohui Qiu, Xiaoyong Hu, Qing Zhang, Tom Wu, Xinfeng Liu
Recently, organometal halide perovskite-based optoelectronics, particularly lasers, have attracted intensive attentions because of its outstanding spectral coherence, low threshold, and wideband tunability. In this work, high-quality CH3 NH3 PbBr3 single crystals with a unique shape of cube-corner pyramids are synthesized on mica substrates using chemical vapor deposition method. These micropyramids naturally form cube-corner cavities, which are eminent candidates for small-sized resonators and retroreflectors...
January 10, 2018: Small
https://www.readbyqxmd.com/read/29315984/default-mode-network-modifications-in-fabry-disease-a-resting-state-fmri-study-with-structural-correlations
#17
Sirio Cocozza, Giuseppe Pontillo, Mario Quarantelli, Francesco Saccà, Eleonora Riccio, Teresa Costabile, Gaia Olivo, Vincenzo Brescia Morra, Antonio Pisani, Arturo Brunetti, Enrico Tedeschi
Aim of the study was to evaluate the presence of Default Mode Network (DMN) modifications in Fabry Disease (FD), and their possible correlations with structural alterations and neuropsychological scores. Thirty-two FD patients with a genetically confirmed diagnosis of classical FD (12 males, mean age 43.3 ± 12.2) were enrolled, along with 35 healthy controls (HC) of comparable age and sex (14 males, mean age 42.1 ± 14.5). Resting-State fMRI data were analyzed using a seed-based approach, with six different seeds sampling the main hubs of the DMN...
January 9, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29311596/theory-for-perfect-transmodal-fabry-perot-interferometer
#18
Xiongwei Yang, Joshua M Kweun, Yoon Young Kim
We establish the theory for perfect transmodal Fabry-Perot interferometers that can convert longitudinal modes solely to transverse modes and vice versa, reaching up to 100% efficiency. Two exact conditions are derived for plane mechanical waves: simultaneous constructive interferences of each of two coupled orthogonal modes, and intermodal interference at the entrance and exit sides of the interferometer with specific skew polarizations. Because the multimodal interferences and specific skew motions require unique anisotropic interferometers, they are realized by metamaterials...
January 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29311577/publisher-correction-transcribed-ultraconserved-region-339-promotes-carcinogenesis-by-modulating-tumor-suppressor-micrornas
#19
Ivan Vannini, Petra M Wise, Kishore B Challagundla, Meropi Plousiou, Mirco Raffini, Erika Bandini, Francesca Fanini, Giorgia Paliaga, Melissa Crawford, Manuela Ferracin, Cristina Ivan, Linda Fabris, Ramana V Davuluri, Zhiyi Guo, Maria Angelica Cortez, Xinna Zhang, Lu Chen, Shuxing Zhang, Cecilia Fernandez-Cymering, Leng Han, Silvia Carloni, Samanta Salvi, Hui Ling, Mariam Murtadha, Paolo Neviani, Barbara J Gitlitz, Ite A Laird-Offringa, Patrick Nana-Sinkam, Massimo Negrini, Han Liang, Dino Amadori, Amelia Cimmino, George A Calin, Muller Fabbri
In the originally published version of this Article, the positions of the final two authors in the author list were inadvertently inverted during the production process. This error has now been corrected in both the PDF and HTML versions of the Article.
January 8, 2018: Nature Communications
https://www.readbyqxmd.com/read/29305833/simple-and-efficient-screening-of-patients-with-fabry-disease-with-high-resolution-melting
#20
Gabriela Pasqualim, Bruna Almeida Dos Santos, Roberto Giugliani, Ursula Matte
BACKGROUND: Fabry disease (FD [MIM: 301500]) is a disorder caused by mutations in the alpha-galactosidase gene (GLA), which presents great allelic heterogeneity. The development of fast screening methods may reduce costs and length of diagnosis, being particularly important for screening programs of high-risk female patients. Therefore, the purpose of this study was to develop a pre-sequencing genetic screening method based on high resolution melting (HRM) analysis. METHODS: We performed HRM analysis in one hundred and three individuals, 79 females and 24 males, with a total of 27 different variants in 30 different genotypes...
January 3, 2018: Clinical Biochemistry
keyword
keyword
86731
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"