keyword
https://read.qxmd.com/read/38535174/the-effect-of-geoclimatic-factors-on-the-distribution-of-paracoccidioidomycosis-in-mato-grosso-do-sul-brazil
#1
JOURNAL ARTICLE
Larissa Rodrigues Fabris, Nathan Guilherme de Oliveira, Bruna Eduarda Bortolomai, Lavínia Cássia Ferreira Batista, Marcos Henrique Sobral, Alisson André Ribeiro, Ursulla Vilella Andrade, Antonio Conceição Paranhos Filho, Lídia Raquel de Carvalho, Ida Maria Foschiani Dias Baptista, Rinaldo Poncio Mendes, Anamaria Mello Miranda Paniago
The incidence of paracoccidioidomycosis (PCM) varies in Latin America, and it is influenced by environmental factors. This study evaluated the distribution of PCM acute/subacute form (AF) cases and their correlation with geoclimatic factors in the Mato Grosso do Sul (MS) state. The study included 81 patients diagnosed with the PCM/AF at the University Hospital of the Federal University of Mato Grosso do Sul between January 1980 and February 2022. Geographic coordinates, health microregion of patient's residence, compensated average temperature, relative air humidity (RH), El Niño Southern Oscillation (ENSO), and average global temperature were analyzed...
February 21, 2024: Journal of Fungi (Basel, Switzerland)
https://read.qxmd.com/read/38533829/reversible-actuation-of-%C3%AE-borophene-nanoscrolls
#2
JOURNAL ARTICLE
Guilherme S L Fabris, Douglas S Galvão, Ricardo Paupitz
In this work, we proposed and investigated the structural and electronic properties of boron-based nanoscrolls (armchair and zigzag) using the DFTB+ method. We also investigated the electroactuation process (injecting and removing charges). A giant electroactuation was observed, but the results show relevant differences between the borophene and carbon nanoscrolls. The molecular dynamics simulations showed that the scrolls are thermally and structurally stable for a large range of temperatures (up to 600 K), and the electroactuation process can be easily tuned and can be entirely reversible for some configurations...
March 27, 2024: Physical Chemistry Chemical Physics: PCCP
https://read.qxmd.com/read/38531466/newborn-genetic-screening-for-fabry-disease-insights-from-a-retrospective-analysis-in-nanjing-china
#3
JOURNAL ARTICLE
Yun Sun, Xian-Wei Guan, Yan-Yun Wang, Dong-Yang Hong, Zhi-Lei Zhang, Ya-Hong Li, Pei-Ying Yang, Xin Wang, Tao Jiang, Xia Chi
Fabry disease (FD), an X-linked disorder resulting from dysfunction of α-galactosidase A, can result in significant complications. Early intervention yields better outcomes, but misdiagnosis or delayed diagnosis is common, impacting prognosis. Thus, early detection is crucial in the clinical diagnosis and treatment of FD. While newborn screening for FD has been implemented in certain regions, challenges persist in enzyme activity detection techniques, particularly for female and late-onset patients. Further exploration of improved screening strategies is warranted...
March 24, 2024: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://read.qxmd.com/read/38527505/-genotype-phenotype-analysis-of-fabry-disease-caused-by-gla-gene-variation-in-a-pedigree
#4
JOURNAL ARTICLE
Z H Ge, Z H Lu, X D Pan, T T Lai, M J Yang, H Q Yang, H B Zhang, G Y Li, Z Q Dai, J H Mao
Objective: To investigate the clinical phenotype and genetic characteristics of patients with Fabry disease caused by a GLA variant, IVS4+919G>A. Methods: It was a prospective study. Fabry disease screening was conducted among high-risk population in Ninghai from October 2021 to August 2023. Those children with decreased α-galactosidase enzyme activity<2.40 μmol/(L·h) or elavated Lyso-GL-3 level>1.10 μg/L in dried blood spot (DBS) method underwent GLA genetic testing for diagnosis confirmation...
March 25, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://read.qxmd.com/read/38525915/acoustic-modulation-and-non-contact-atomization-of-droplets-based-on-the-fabry-p%C3%A3-rot-resonator
#5
JOURNAL ARTICLE
Jingjun Li, Xiukun Wang, Fan Yang, Yadong Sun, Lei Zhang
A non-contact ultrasonic atomization based on the Fabry-Pérot resonator is proposed to obtain atomized droplets with a reduced droplet diameter and concentrated droplet distributions. To better understand the mechanism inside the acoustic chamber, the acoustic-fluid interactions are numerically explored inside the Fabry-Pérot resonator to achieve the precise modulation of droplets. The influence of the acoustic chamber's geometry and the ultrasonic properties on the atomized droplet diameter and distributions is investigated, aiming to establish matching relationships between the atomized droplet diameter and the geometry of the acoustic chamber...
March 25, 2024: Lab on a Chip
https://read.qxmd.com/read/38523086/effects-of-topography-and-stand-spatial-structure-on-the-diameter-at-breast-height-growth-of-major-pioneer-tree-species-of-natural-broad-leaved-mixed-forests-in-zhejiang-province-china
#6
JOURNAL ARTICLE
Jian-Wu Wang, Sen Xu, Bi-Yong Ji, Qun DU
Based on the continuous inventory data of forest resources in Zhejiang Province in 2019 and 2021, we used statistical methods such as polynomial regression to analyze the impacts of topography and forest spatial structure on average annual diameter at breast height (DBH) growth of main pioneer tree species in natural broad-leaved mixed forests. The results showed that DBH of Schima superba , Quercus glauca , Quercus fabri , Lithocarpus glaber , Castanopsis eyrei , and Castanopsis sclerophylla were between 5-50...
February 2024: Ying Yong Sheng Tai Xue Bao, the Journal of Applied Ecology
https://read.qxmd.com/read/38522108/grating-assisted-hot-electron-photodetectors-for-s-and-c-band-telecommunication
#7
JOURNAL ARTICLE
Weijia Shao, Weihao Cui, Yixiao Xin, Junhui Hu, Xiaofeng Li
There has been an increasing demand for cryogen-free, cost-effective, and broadband infrared photodetectors in optic fiber communication systems. Here, differing from costly and spectra-limited InGaAs photodetectors, we present a design of hot-electron photodetectors (HE PDs) with attributes of room-temperature operation and strong optical absorption over S and C bands (from 1460 nm to 1565 nm). The designed HE PD consists of a metal-semiconductor-metal hot-electron stack integrated with a front grating. Optical simulations reveal that mode hybridizations between Fabry-Pérot resonance and grating-induced surface plasmon excitation lead to high absorption efficiencies (≥ 0...
March 24, 2024: Nanotechnology
https://read.qxmd.com/read/38520360/assessing-brain-involvement-in-fabry-disease-with-deep-learning-and-the-brain-age-paradigm
#8
JOURNAL ARTICLE
Alfredo Montella, Mario Tranfa, Alessandra Scaravilli, Frederik Barkhof, Arturo Brunetti, James Cole, Michela Gravina, Stefano Marrone, Daniele Riccio, Eleonora Riccio, Carlo Sansone, Letizia Spinelli, Maria Petracca, Antonio Pisani, Sirio Cocozza, Giuseppe Pontillo
While neurological manifestations are core features of Fabry disease (FD), quantitative neuroimaging biomarkers allowing to measure brain involvement are lacking. We used deep learning and the brain-age paradigm to assess whether FD patients' brains appear older than normal and to validate brain-predicted age difference (brain-PAD) as a possible disease severity biomarker. MRI scans of FD patients and healthy controls (HCs) from a single Institution were, retrospectively, studied. The Fabry stabilization index (FASTEX) was recorded as a measure of disease severity...
April 2024: Human Brain Mapping
https://read.qxmd.com/read/38520029/what-should-rheumatologists-know-about-gaucher-disease-and-fabry-disease-connecting-the-dots-for-an-overview
#9
REVIEW
Rafael Alves Cordeiro, Nilton Salles Rosa Neto, Henrique Ayres Mayrink Giardini
Gaucher and Fabry diseases are lysosomal storage disorders in which deficient enzyme activity leads to pathological accumulation of sphingolipids. These diseases have a broad phenotypic presentation. Musculoskeletal symptoms and pain complaints are frequently reported by patients. Thus, rheumatologists can be contacted by these patients, contributing to the correct diagnosis, earlier indication of appropriate treatment and improvement of their prognosis. This review describes important concepts about Gaucher and Fabry diseases that rheumatologists should understand to improve patients' quality of life and change the natural history of these diseases...
March 22, 2024: Advances in Rheumatology
https://read.qxmd.com/read/38512375/pathogenic-pathways-of-renal-damage-in-fabry-nephropathy-interplay-between-immune-cell-infiltration-apoptosis-and-fibrosis
#10
JOURNAL ARTICLE
Constanza Bondar, Maria de Los Angeles de Bolla, Pablo Neumann, Antonio Pisani, Sandro Feriozzi, Paula Adriana Rozenfeld
BACKGROUND: Fabry nephropathy is a consequence of the deposition of globotriaosylceramide, caused by deficient GLA enzyme activity in all types of kidney cells. These deposits are perceived as damage signals leading to activation of inflammation resulting in renal fibrosis. There are few studies related to immunophenotype characterization of the renal infiltrate in kidneys in patients with Fabry disease and its relationship to mechanisms of fibrosis. This work aims to quantify TGF-β1 and active caspase 3 expression and to analyze the profile of cells in inflammatory infiltration in kidney biopsies from Fabry naïve-patients, and to investigate correlations with clinical parameters...
March 21, 2024: Journal of Nephrology
https://read.qxmd.com/read/38512269/development-and-validation-of-staging-systems-for-aa-amyloidosis
#11
JOURNAL ARTICLE
Marco Basset, Stefan O Schönland, Laura Obici, Janine Günther, Eloisa Riva, Tobias Dittrich, Paolo Milani, Virginia Valeria Ferretti, Ettore Pasquinucci, Andrea Foli, Christoph Kimmich, Martina Nanci, Claudia Bellofiore, Francesca Benigna, Jörg Beimler, Pietro Benvenuti, Francesca Fabris, Roberta Mussinelli, Mario Nuvolone, Catherine Klersy, Riccardo Albertini, Giampaolo Merlini, Ute Hegenbart, Giovanni Palladini, Norbert Blank
BACKGROUND: The kidney is involved in almost 100% of cases of AA amyloidosis, a rare disease caused by persistent inflammation with long overall survival but frequent progression to end-stage kidney failure . Identification of patients with advanced disease at diagnosis is difficult, given the absence of validated staging systems. METHODS: Newly diagnosed patients with AA amyloidosis from the Pavia (n=233, testing cohort) and Heidelberg (n=243, validation cohort) centers were included in the study...
March 21, 2024: Journal of the American Society of Nephrology: JASN
https://read.qxmd.com/read/38508062/lessons-learned-from-the-canadian-fabry-disease-initiative-for-future-risk-sharing-and-managed-access-agreements-for-pharmaceutical-and-advanced-therapies-in-canada
#12
JOURNAL ARTICLE
Conor M W Douglas, Shir Grunebaum
Risk sharing agreements (RSAs) and managed access agreements have emerged as tools to overcome evidentiary uncertainty and contain costs of pharmaceuticals; however, Canada has relatively little experience with these health policy instruments. This article describes one of the few examples of national RSAs. Enzyme replacement therapies (ERT) were introduced in Canada to treat Fabry disease in the early 2000s through an RSA. Based on qualitative interviews with key participating actors, this article explains how this RSA ensured continuity of treatment for patients already on ERT, and collected robust real-world evidence to secure treatment for future Fabry patients...
March 13, 2024: Health Policy
https://read.qxmd.com/read/38503034/efficient-testing-of-weld-seam-models-with-radii-of-curvature-in-the-millimeter-range-using-laser-ultrasound
#13
JOURNAL ARTICLE
Markus Saurer, Guenther Paltauf, Robert Nuster
Laser ultrasound is a widely used tool for industrial quality assurance when a contactless and fast method is required. In this work, we used a laboratory setup based on a confocal Fabry-Perot interferometer to examine weld seam models. The focus was placed on small samples with curved surfaces (small in the sense that the radius of curvature is comparable to the largest ultrasonic wavelength) and on efficient ways to detect the presence and volume of process pores, with the goal to transfer this method to industrial applications...
March 16, 2024: Ultrasonics
https://read.qxmd.com/read/38496077/a-case-of-hypofractionated-radiation-therapy-for-early-stage-breast-cancer-in-a-patient-with-fabry-disease
#14
Motoki Honda, Yojiro Ishikawa, Kengo Ito, Satoshi Teramura, Seki Yasuhiro, Takayuki Yamada
Fabry disease is a metabolic disorder caused by a deficiency in lysosomal enzymes and is inherited as an X-chromosomal disorder. Patients with Fabry disease have a low incidence of cancer, and reports of malignant tumors, especially in the thoracic region, are rare. In this case report, we describe our experience with radiation therapy following breast-conserving surgery in a patient with left breast cancer and Fabry disease, and we review the existing literature. The patient, a woman in her 40s, required postoperative irradiation for left breast cancer (pT1N0M0)...
February 2024: Curēus
https://read.qxmd.com/read/38489464/multiwavelength-erbium-doped-fiber-laser-using-the-polarization-hole-burning-effect-for-multichannel-acoustic-detection
#15
JOURNAL ARTICLE
Cheng Zhou, Shengnan Zhou, Jiajun Tian, Yong Yao, Yongkang Dong
We propose and demonstrate a novel, to the best of our knowledge, fiber-optic multipoint acoustic detection system based on a multiwavelength erbium-doped fiber (EDF) laser (MWEDFL) using the polarization-hole-burning effect with Fabry-Perot interferometers as the acoustic cavity-loss modulator. A polarization-wavelength-related filter is designed to assign a distinct polarization state to each laser wavelength. By adjusting the polarization state, the polarization-dependent loss and gain of each laser line are tuned to be equal, effectively suppressing the mode competition of EDF and enabling a stable MWEDFL...
March 15, 2024: Optics Letters
https://read.qxmd.com/read/38489440/robust-self-injection-locking-to-a-non-confocal-monolithic-fabry-perot-cavity
#16
JOURNAL ARTICLE
Anatoliy Savchenkov, Wei Zhang, Vladimir Iltchenko, Andrey Matsko
We demonstrate an efficient simultaneous self-injection locking of two semiconductor lasers to high-order modes of a standalone monolithic non-confocal Fabry-Perot cavity. The lasers are used to generate a low-noise microwave signal on a fast photodiode. The overall improvement of the laser spectral purity exceeds 80 dB. The observed single-sideband phase noise of X- to W-band signals is at the -110 dBc/Hz level and is limited by the fundamental thermorefractive noise of the cavity. The demonstrated cavity-laser configuration can be tightly packaged and is promising for the generation of high-frequency RF signals as well as for referencing optical frequency combs...
March 15, 2024: Optics Letters
https://read.qxmd.com/read/38488060/ultrafast-nanodrum-on-chip-pixels
#17
JOURNAL ARTICLE
Jialu Li, James Andell Hutchison, Dan Smith, Hao Wu, Paul Mulvaney
Environmentally friendly, ultrafast display pixels of micrometer sizes are fabricated with nanometer-thick gold films and Si/SiO2 wafers. The color displayed is due to both the plasmon response of the gold film and the optical interference from the Fabry-Peerot cavity formed by the underlying silicon substrate, the semitransparent gold film and the air gap between them. When an electric potential is applied to the gold film, the electrostatic force induces an attraction between the gold film and the silicon wafer...
March 15, 2024: Nano Letters
https://read.qxmd.com/read/38486361/anderson-fabry-disease-management-role-of-the-cardiologist
#18
JOURNAL ARTICLE
Maurizio Pieroni, Mehdi Namdar, Iacopo Olivotto, Robert J Desnick
Anderson-Fabry disease (AFD) is a lysosomal storage disorder characterized by glycolipid accumulation in cardiac cells, associated with a peculiar form of hypertrophic cardiomyopathy (HCM). Up to 1% of patients with a diagnosis of HCM indeed have AFD. With the availability of targeted therapies for sarcomeric HCM and its genocopies, a timely differential diagnosis is essential. Specifically, the therapeutic landscape for AFD is rapidly evolving and offers increasingly effective, disease-modifying treatment options...
March 15, 2024: European Heart Journal
https://read.qxmd.com/read/38485718/mapping-the-habitat-refugia-of-isidella-elongata-under-climate-change-and-trawling-impacts-to-preserve-vulnerable-marine-ecosystems-in-the-mediterranean
#19
JOURNAL ARTICLE
Vincent Georges, Sandrine Vaz, Pierluigi Carbonara, Marie-Claire Fabri, Emanuela Fanelli, Maria Cristina Follesa, Germana Garofalo, Vasilis Gerovasileiou, Angélique Jadaud, Porzia Maiorano, Pilar Marin, Chryssi Mytilineou, Covadonga Orejas, Maria Del Mar Otero, Chris J Smith, Ioannis Thasitis, Valentina Lauria
The bamboo-coral Isidella elongata is a key habitat-forming species in the deep Mediterranean Sea. This alcyonacean is listed as an indicator of Vulnerable Marine Ecosystems (VMEs) and as Critically Endangered due to bottom trawling impacts. In this work, a modeling approach was used to predict and map the habitat suitability of I. elongata in the Mediterranean Sea under current environmental conditions. Occurrence data were modeled as a function of environmental parameters. Using climate change scenarios and fishing effort data, the risk of climate change and fisheries impacts on habitat suitability were estimated, and climate refugia were identified...
March 15, 2024: Scientific Reports
https://read.qxmd.com/read/38483324/triple-para-functionalized-cations-and-neutral-radicals-of-enantiopure-diaza-4-helicenes
#20
JOURNAL ARTICLE
Bibiana Fabri, Tiziana Funaioli, Lucas Frédéric, Christina Elsner, Enrica Bordignon, Francesco Zinna, Lorenzo Di Bari, Gennaro Pescitelli, Jérôme Lacour
Modulation of absorbance and emission is key for the design of chiral chromophores. Accessing a series of compounds absorbing and emitting (circularly polarized) light over a wide spectral window and often toward near-infrared is of practical value in (chir)optical applications. Herein, by late-stage functionalization on derivatives bridging triaryl methyl and helicene domains, we have achieved the regioselective triple introduction of para electron-donating or electron-withdrawing substituents. Extended tuning of electronic (e...
March 14, 2024: Journal of the American Chemical Society
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