Search results for "fabry"
showing 10 items of 171 documents
Enzyme replacement therapy in Fabry disease: Comparison of agalsidase alfa and agalsidase beta
2008
The Heart in Fabry Disease – from Pathogenesis to Enzyme Replacement Therapy
2010
The cardiovascular involvement in Fabry disease is progressive, and accounts for one of the major reasons for abbreviated life expectancy and increased morbidity. The majority of patients develop signs and symptoms, related to heart failure and arrhythmias. Hypertrophic cardiomyopathy, in later stages combined with myocardial fibrosis, is one of the leading features. Both genders are affected, and females develop severe cardiac Fabry disease approximately 10–15 years later in life than male patients. Diastolic dysfunction and reduced longitudinal mid-wall related systolic function are the mechanical consequences of the myocardial and epithelial changes related to accumulation of the storage…
Symptomatic and Ancillary Therapy
2010
Although enzyme replacement therapy has had a considerable impact on the management of patients with Fabry disease, it is essential that attention is also given to supportive therapy. In this chapter a general overview about ancillary therapy is given. The great variability of clinical symptoms faced by patients with Fabry disease need the involvement from many different specialists, as well as a wide range of concomitant treatments. Coordinating a good multidisciplinary approach for each patient and ensuring that treatment is made as convenient as possible may offer a more positive impact on the quality of life of those patients affected by Fabry disease.
Enzyme replacement therapy for Fabry's disease – Authors' reply
2010
A study of plasma and urinary sediment globotriaosylceramide levels in females with Fabry disease
2007
Pathophysiologische Aspekte hirnstruktureller Veränderungen bei Morbus Fabry: Literaturübersicht
2006
Fabry Disease (FD) is a rare X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-GAL) enzyme activity. Neutral glycosphingolipides (esp. Gb3) accumulate in lysosomes of several tissues, particularly in vascular endothelium and smooth muscle cells. Cerebral manifestations that might be mainly due to progressive cerebrovascular dysfunction, are one major and often life-threatening burden of the disease. We reviewed the present literature concerning brain structural alterations in FD and discuss the possibly relevant underlying pathophysiological aspects of these disturbances. Cerebrovascular events (TIA, stroke) occur in FD at a rather early age. In female…
Hyperspectral Imaging of Macroinvertebrates : a Pilot Study for Detecting Metal Contamination in Aquatic Ecosystems
2018
The applicability of spectral analysis in detection of freshwater metal contamination was assessed by developing and testing a novel hyperspectral imaging (HSI) application for aquatic insect larvae (Trichoptera: Hydropsychidae). Larvae were first exposed to four different cadmium (Cd) concentrations: 0, 1, 10, and 100 μg L−1 for 96 h. Individual larvae were then preserved in ethanol, inspected with microscopy for the number of anomalies in larval gills, and imaged by hyperspectral camera operating with wavebands between 500 and 850 nm. Three additional larvae from each exposure were analyzed for tissue Cd concentration. Although the larval tissue Cd concentrations correlated positively wit…
High-spectral-resolution Fabry-Pérot interferometers overcome fundamental limitations of present volcanic gas remote sensing techniques
2023
Remote sensing (RS) of volcanic gases has become a central tool for studying volcanic activity. For instance, ultraviolet (UV) skylight spectroscopy with grating spectrographs (GS) enables SO2 (and, under favourable conditions, BrO) quantification in volcanic plumes from autonomous platforms at safe distances. These measurements can serve volcanic monitoring and they cover all stages of volcanic activity in long measurement time series, which substantially contributes to the refinement of theories on volcanic degassing. Infrared (IR) remote sensing techniques are able to measure further volcanic gases (e.g., HF, HCl, CO2, CO). However, the employed Fourier transform spectrometers (FTSs) are…
Biomass estimator for NIR image with a few additional spectral band images taken from light UAS
2012
A novel way to produce biomass estimation will offer possibilities for precision farming. Fertilizer prediction maps can be made based on accurate biomass estimation generated by a novel biomass estimator. By using this knowledge, a variable rate amount of fertilizers can be applied during the growing season. The innovation consists of light UAS, a high spatial resolution camera, and VTT's novel spectral camera. A few properly selected spectral wavelengths with NIR images and point clouds extracted by automatic image matching have been used in the estimation. The spectral wavelengths were chosen from green, red, and NIR channels.
UAV-based hyperspectral monitoring of small freshwater area
2014
Recent development in compact, lightweight hyperspectral imagers have enabled UAV-based remote sensing with reasonable costs. We used small hyperspectral imager based on Fabry-Perot interferometer for monitoring small freshwater area in southern Finland. In this study we shortly describe the utilized technology and the field studies performed. We explain processing pipeline for gathered spectral data and introduce target detection-based algorithm for estimating levels of algae, aquatic chlorophyll and turbidity in freshwater. Certain challenges we faced are pointed out.