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P Ferenc, J Fabry, T Zatko, P Kunc, J Sojak, M Grendar, R Pecova
The bronchial challenge tests are the most widely used. The aim of this study was to clarify changes of cough reflex sensitivity before and after exercise challenge testing in asthma children.
March 13, 2018: Respiratory Physiology & Neurobiology
Jitka Rybová, Ladislav Kuchar, Helena Hulková, Befekadu Asfaw, Robert Dobrovolný, Jakub Sikora, Vladimír Havlícek, Ludovít Škultéty, Jana Ledvinová
Blood group B glycosphingolipids (B-GSLs) are substrates of the lysosomal alpha-galactosidase A (AGAL). Similar to its major substrate - globotriaosylceramide (Gb3Cer) - B-GSLs are not degraded and accumulate in the cells of patients affected by an inherited defect of AGAL activity (Fabry disease - FD).The pancreas is a secretory organ known to have high biosynthesis of blood group GSLs. Herein, we provide a comprehensive overview of the biochemical and structural abnormalities in pancreatic tissue from two male FD patients with blood group B...
March 14, 2018: Glycobiology
Wanfu Shen, Chunguang Hu, Shuchun Huo, Zhaoyang Sun, Shuangqing Fan, Jing Liu, Xiaotang Hu
We report on both the theoretical and experimental design of a black phosphorus (BP)-based reflective linear polarizer on Si/SiO2 substrate in visible range using the Fabry-Perot cavities method. Thanks to the optical anisotropy of BP, polarization wavelength regulation and a high extinction ratio are achievable via optimizing the thickness of BP. Using azimuth-dependent reflectance difference microscopy, we directly measured a huge optical anisotropy of 1.58, corresponding to an extinction ratio of ∼9  dB, from a 96 nm BP on a silicon substrate capped by 260 nm thermally oxidized silicon at a wavelength of 690 nm for the first time, to the best of our knowledge...
March 15, 2018: Optics Letters
Hiroki Maruyama, Kaori Miyata, Mariko Mikame, Atsumi Taguchi, Chu Guili, Masaru Shimura, Kei Murayama, Takeshi Inoue, Saori Yamamoto, Koichiro Sugimura, Koichi Tamita, Toshihiro Kawasaki, Jun Kajihara, Akifumi Onishi, Hitoshi Sugiyama, Teiko Sakai, Ichijiro Murata, Takamasa Oda, Shigeru Toyoda, Kenichiro Hanawa, Takeo Fujimura, Shigehisa Ura, Mimiko Matsumura, Hideki Takano, Satoshi Yamashita, Gaku Matsukura, Ryushi Tazawa, Tsuyoshi Shiga, Mio Ebato, Hiroshi Satoh, Satoshi Ishii
PurposePlasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females.MethodsBetween 1 July 2014 and 31 December 2015, we screened 2,360 patients (1,324 males) referred from 169 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively...
March 15, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Jinho Park, Jae Hyeong Lee, Gil-Ho Lee, Yositake Takane, Ken-Ichiro Imura, Takashi Taniguchi, Kenji Watanabe, Hu-Jong Lee
We report on short ballistic (SB) Josephson coupling in junctions embedded in a planar heterostructure of graphene. Ballistic Josephson coupling is confirmed by the Fabry-Perot-type interference of the junction critical current I_{c}. The product of I_{c} and the normal-state junction resistance R_{N}, normalized by the zero-temperature gap energy Δ_{0} of the superconducting electrodes, turns out to be exceptionally large close to 2, an indication of strong Josephson coupling in the SB junction limit. However, I_{c} shows a temperature dependence that is inconsistent with the conventional short-junction-like behavior based on the standard Kulik-Omel'yanchuk prediction...
February 16, 2018: Physical Review Letters
Efthymios Maneas, Wenfeng Xia, Olumide Ogunlade, Martina Fonseca, Daniil I Nikitichev, Anna L David, Simeon J West, Sebastien Ourselin, Jeremy C Hebden, Tom Vercauteren, Adrien E Desjardins
Tissue-mimicking phantoms are widely used for the calibration, evaluation and standardisation of medical imaging systems, and for clinical training. For photoacoustic imaging, tissue-mimicking materials (TMMs) that have tuneable optical and acoustic properties, high stability, and mechanical robustness are highly desired. In this study, gel wax is introduced as a TMM that satisfies these criteria for developing photoacoustic imaging phantoms. The reduced scattering and optical absorption coefficients were independently tuned with the addition of TiO2 and oil-based inks...
March 1, 2018: Biomedical Optics Express
Alessandro Di Toro, Valentina Favalli, Eloisa Arbustini
No abstract text is available yet for this article.
February 2018: Journal of Cardiovascular Medicine
Aleš Hnízda, Milan Fábry, Takaya Moriyama, Petr Pachl, Michael Kugler, Vítězslav Brinsa, David B Ascher, William L Carroll, Petr Novák, Markéta Žaliová, Jan Trka, Pavlína Řezáčová, Jun J Yang, Václav Veverka
Activating mutations in NT5C2, a gene encoding cytosolic purine 5'-nucleotidase (cN-II), confer chemoresistance in relapsed acute lymphoblastic leukemia. Here we show that all mutants became independent of allosteric effects of ATP and thus constitutively active. Structural mapping of mutations described in patients demonstrates that 90% of leukemia-specific allelles directly affect two regulatory hotspots within the cN-II molecule-the helix A region: residues 355-365, and the intersubunit interface: helix B (232-242) and flexible interhelical loop L (400-418)...
February 25, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Patrício Aguiar, Olga Azevedo, Rui Pinto, Jacira Marino, Carlos Cardoso, Nuno Sousa, Damião Cunha, Derralynn Hughes, José Luís Ducla Soares
BACKGROUND: Cardiomyopathy is a major determinant of overall Fabry disease (FD) prognosis, with the worst outcomes in patients with myocardial fibrosis. Late gadolinium enhancement is currently the gold standard for evaluation of replacement myocardial fibrosis; however, this event is irreversible, thus identification of biomarkers of earlier diffuse fibrosis is paramount. METHODS AND RESULTS: Type I collagen synthesis and degradation biomarkers (PICP [carboxyterminal propeptide of procollagen type I], ICTP [carboxyterminal telopeptide of type I collagen], and MMP1 [matrix metalloproteinase 1] and MMP2) and markers of bone synthesis and degradation were evaluated (to adjust type I collagen metabolism to bone turnover) in FD patients and controls...
March 13, 2018: Journal of the American Heart Association
Silvia Bettini, Emanuel Bordigato, Roberto Fabris, Roberto Serra, Chiara Dal Pra', Anna Belligoli, Marta Sanna, Chiara Compagnin, Mirto Foletto, Luca Prevedello, Paola Fioretto, Roberto Vettor, Luca Busetto
OBJECTIVES: Resting energy expenditure (REE) declines more than what is expected according to body composition changes after caloric restriction. This metabolic adaptation is considered one of the factors favoring weight regain. The aim of this study is to evaluate the changes of REE and calculate the degree of metabolic adaptation occurring after laparoscopic sleeve gastrectomy (LSG). METHODS: REE (by indirect calorimetry) and body composition (fat-free mass or FFM, fat mass or FM by bioelectrical impedance analysis) were determined before and after 12 months in 154 patients with obesity treated with laparoscopic sleeve gastrectomy (LSG)...
March 12, 2018: Obesity Surgery
Rolf Spirig, Ian K Campbell, Sandra Koernig, Chao-Guang Chen, Bonnie J B Lewis, Rebecca Butcher, Ineke Muir, Shirley Taylor, Jenny Chia, David Leong, Jason Simmonds, Pierre Scotney, Peter Schmidt, Louis Fabri, Andreas Hofmann, Monika Jordi, Martin O Spycher, Susann Cattepoel, Jennifer Brasseit, Con Panousis, Tony Rowe, Donald R Branch, Adriana Baz Morelli, Fabian Käsermann, Adrian W Zuercher
Activation of Fc receptors and complement by immune complexes is a common important pathogenic trigger in many autoimmune diseases and so blockade of these innate immune pathways may be an attractive target for treatment of immune complex-mediated pathomechanisms. High-dose IVIG is used to treat autoimmune and inflammatory diseases, and several studies demonstrate that the therapeutic effects of IVIG can be recapitulated with the Fc portion. Further, recent data indicate that recombinant multimerized Fc molecules exhibit potent anti-inflammatory properties...
March 12, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Alberto Ortiz, Dominique P Germain, Robert J Desnick, Juan Politei, Michael Mauer, Alessandro Burlina, Christine Eng, Robert J Hopkin, Dawn Laney, Aleš Linhart, Stephen Waldek, Eric Wallace, Frank Weidemann, William R Wilcox
Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the GLA gene leading to deficient α-galactosidase A activity, glycosphingolipid accumulation, and life-threatening complications. Phenotypes vary from the "classic" phenotype, with pediatric onset and multi-organ involvement, to later-onset, a predominantly cardiac phenotype. Manifestations are diverse in female patients in part due to variations in residual enzyme activity and X chromosome inactivation patterns. Enzyme replacement therapy (ERT) and adjunctive treatments can provide significant clinical benefit...
February 28, 2018: Molecular Genetics and Metabolism
Amanda Toupin, Pamela Lavoie, Marie-Françoise Arthus, Mona Abaoui, Michel Boutin, Carole Fortier, Claudia Ménard, Daniel G Bichet, Christiane Auray-Blais
Fabry disease is an X-linked lysosomal storage disorder with marked variability in the phenotype and genotype. Glycosphingolipids such as globotriaosylceramide (Gb3 ) isoforms/analogs, globotriaosylsphingosine (lyso-Gb3 ) and analogs, and galabiosylceramide (Ga2 ) isoforms/analogs may accumulate in biological fluids and different organs. The aims of this study were to: 1) develop/validate a novel UHPLC-MS/MS method for relative quantitation of Gb3 in leukocytes (unfractionated white blood cells), B lymphocytes and monocytes; 2) evaluate these biomarkers in a cohort of Fabry patients and healthy controls; and 3) assess correlations between these biomarkers, treatment and genotype...
July 26, 2018: Analytica Chimica Acta
Xiaotian Zhang, Jiteng Sheng, Haibin Wu
We explore theoretically the optomechanical interaction between a light field and a mechanical mode mediated by a Kerr nonlinear medium inside a Fabry-Perot cavity. When the system is driven by a strong and fast amplitude-modulated light field, i.e., in the so-called temporal rocking region, the cavity field and the mechanical oscillator show the characteristics of multistability. The rocking breaks down the continuous phase symmetry of the cavity field to a bistable case of two equivalent states with exact π phase difference...
March 5, 2018: Optics Express
Lei Lei, Shun Li, Haixuan Huang, Keyu Tao, Ping Xu
We propose a design of an ultra-broadband absorber based on a thin metamaterial nanostructure composed of a periodic array of titanium-silica (Ti-SiO2 ) cubes and an aluminum (Al) bottom film. The proposed structure can achieve nearly perfect absorption with an average absorbance of 97% spanning a broad range from visible to near-infrared (i.e., from 354 nm to 1066 nm), showing a 90% absorption bandwidth over 712 nm, and the peak absorption is up to 99.8%. The excitation of superior surface plasmon resonance combined with the resonance induced by the metal-insulator-metal Fabry-Perot (FP) cavity leads to this broadband perfect absorption...
March 5, 2018: Optics Express
Pengcheng Chen, Xuewen Shu
An optical fiber Fabry-Perot probe sensor for high-temperature measurement is proposed and demonstrated, which is fabricated by inducing a refractive-index-modified-dot (RIMD) in the fiber core near the end of a standard single mode fiber (SMF) using a femtosecond laser. The RIMD and the SMF end faces form a Fabry-Perot interferometer (FPI) with a high-quality interference fringe visibility (>20dB). As a high-temperature sensor, such an FPI exhibits a sensitivity of 13.9pm/°C and 18.6pm/°C in the range of 100-500°Cand 500-1000°C,respectively...
March 5, 2018: Optics Express
Bruno Henriques, Douglas Fabris, Júlio C M Souza, Filipe S Silva, Óscar Carvalho, Márcio C Fredel, Joana Mesquita-Guimarães
OBJECTIVES: The aim of this study was to evaluate the effect of laser surface structuring on the bond strength of feldspar-based porcelain to zirconia, as compared to conventional sandblasting treatment. MATERIALS AND METHODS: Thirty cylindrical zirconia substrates, previously sintered, were divided in three groups according to the type of surface conditioning: 1) sandblasting with 50 µm Al2 O3 ; 2) laser structuring (Ø25 µm holes); and 3) laser structuring (Ø50 µm holes)...
February 27, 2018: Journal of the Mechanical Behavior of Biomedical Materials
(no author information available yet)
No abstract text is available yet for this article.
March 8, 2018: BMJ Open
José-Vicente Torregrosa
No abstract text is available yet for this article.
March 6, 2018: Medicina Clínica
Ding Ding, Wenlong Lv, Xueqin Lv, Xiaomei Cai, Yan Zhang, Binbin Xu, Jiangyong Zhang
The influence of grating parameters, including the diffraction efficiency and ruling density, on the performance of high-power blue external-cavity (EC) semiconductor lasers has been studied. We find that for the Littrow-type EC laser with a zeroth-order diffracted beam as a coupling output, the high zeroth-order diffraction efficiency benefits the high external light extraction efficiency, and the high first-order diffraction efficiency contributes to the low threshold current and the wide wavelength tuning bandwidth...
March 1, 2018: Applied Optics
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