Results 31 to 40 of about 136,715 (267)

Fabry Nephropathy

open access: yesArchives of Pathology & Laboratory Medicine, 2017
Fabry disease is a rare X-linked recessive lysosomal storage disease. Multiple mutations of the GLA gene lead to a deficient or absent activity of the lysosomal enzyme α-galactosidase A, resulting in progressive glycotriaosylceramide accumulation in many organs.
Prudence, Colpart, Sophie, Félix
openaire   +2 more sources

Impact of enzyme replacement therapy and migalastat on left atrial strain and cardiomyopathy in patients with Fabry disease

open access: yesFrontiers in Cardiovascular Medicine, 2023
AimsCardiomyopathy in Fabry disease (FD) is a major determinant of morbidity and mortality. This study investigates the effects of FD-specific treatment using enzyme replacement therapy (ERT) and chaperone therapy on left atrial (LA) function using two ...
Christian Pogoda   +7 more
doaj   +1 more source

Study of laser induced temperature variation in silica nanofibers [PDF]

open access: yesEPJ Web of Conferences, 2022
After presenting a theoretical modelling based on the heat equation, we show two different experiments to measure the laser induced temperature variation in silica nanofibers in air, a direct one and an indirect one based on Brillouin scattering, leading
Abdedou Yanis   +10 more
doaj   +1 more source

Input-output relations for a 3-port grating coupled Fabry-Perot cavity [PDF]

open access: yes, 2005
We analyze an optical 3-port reflection grating by means of a scattering matrix formalism. Amplitude and phase relations between the 3 ports, i.e. the 3 orders of diffraction are derived.
A. Bunkowski   +6 more
core   +3 more sources

Synthetic exposure with a CMOS camera for multiple exposure speckle imaging of blood flow

open access: yesScientific Reports, 2022
Speckle contrast imaging is an established technique to obtain relative blood flow maps over wide fields of view. A major improvement of the method relies on the acquisition of raw speckle images at different exposure times but requires simultaneous ...
M. Chammas, F. Pain
doaj   +1 more source

Inverse scattering approach to multiwavelength Fabry-Pérot laser design [PDF]

open access: yes, 2006
A class of multiwavelength Fabry-Pérot lasers is introduced where the spectrum is tailored through a patterning of the cavity effective index. The cavity geometry is obtained using an inverse scattering approach and can be designed such that the spacing ...
Amann, A.   +9 more
core   +3 more sources

Distinct Signatures For Coulomb Blockade and Aharonov-Bohm Interference in Electronic Fabry-Perot Interferometers [PDF]

open access: yes, 2008
Two distinct types of magnetoresistance oscillations are observed in two electronic Fabry-Perot interferometers of different sizes in the integer quantum Hall regime.
C. M. Marcus   +6 more
core   +2 more sources

Enhanced optomechanical readout using optical coalescence [PDF]

open access: yes, 2013
We present a scheme to strongly enhance the readout sensitivity of the squared displacement of a mobile scatterer placed in a Fabry-P\'erot cavity.
Dantan, Aurélien   +3 more
core   +2 more sources

Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial. [PDF]

open access: yes, 2015
Trial designThis analysis characterizes the degree of early organ involvement in a cohort of oligo-symptomatic untreated young patients with Fabry disease enrolled in an ongoing randomized, open-label, parallel-group, phase 3B clinical trial.MethodsMales
An Haack, Kristina   +17 more
core   +8 more sources

A new wavelength calibration for echelle spectrographs using Fabry-Perot etalons

open access: yes, 2019
The study of Earth-mass extrasolar planets via the radial-velocity technique and the measurement of the potential cosmological variability of fundamental constants call for very-high-precision spectroscopy at the level of $\updelta\lambda ...
Cersullo, F.   +4 more
core   +1 more source

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