Search results for "Fab"
showing 10 items of 1201 documents
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…
‘Wulfila as the Inventor of the Gothic Alphabet. The Tradition in Late Antiquity and the Middle Ages’
1992
L'histoire en chansons : de la mémorisation à la fabulation poético-narrative
2007
Los cantarcillos tradicionales del repertorio infantil asocian voz, oído, memoria y gesto con modalidades de complejidad variable ; el carácter familiar de tales cantares conocidos de todos explica que hast auna fecha muy reciente los filólogos no hayan prestado atencion a este tipo de composiciones de arte verbal. Sin embargo, tales poemas cantados permiten entrecruzar varios niveles de reflexion en torno a la memoria. Memoria creadora, primero (ya que ésta es el vector que permite fijar y transmitir estos textos abiertos) ; memoria estructurante, luego (en la mendida en que la arquitectura narrativa de estos poemas cantados depende intrinsecamente de las limitaciones que impone la memoriz…
Fabricación, caracterización, modelado y propuesta de aplicabilidad de sensores magnetorresistivos de efecto túnel para medición de potencia y sensor…
2015
En el capítulo 1 se describen los fundamentos teóricos a utilizar para los análisis y estudios a realizar más adelante, así como una breve explicación del estado de la técnica que es el estado actual de la tecnología al momento de redacción de la tesis. La introducción se divide en la tecnología magnetorresistiva, definiendo qué es y los efectos magnetorresistivos que existen así como su aplicación aeroespacial y modelización fraccional. A continuación se presenta el procesamiento digital de datos y las redes de sensores tratando un poco sobre los protocolos de comunicación formatos y aplicación de la transformada digital de Fourier. En el capítulo 2 se aborda la microfabricación de los sen…
Orthogonal switching of AMS axes during type-2 fold interference : Insights from integrated X-ray computed tomography, AMS and 3D petrography
2017
We applied X-ray computed microtomography (μ-CT) in combination with anisotropy of magnetic susceptibility (AMS) analysis to study metamorphic rock fabrics in an oriented drill core sample of pyrite-pyrrhotite-quartz-mica schist. The sample is extracted from the Paleoproterozoic Martimo metasedimentary belt of northern Finland. The μ-CT resolves the spatial distribution, shape and orientation of 25,920 pyrrhotite and 153 pyrite grains localized in mm-thick metapelitic laminae. Together with microstructural analysis, the μ-CT allows us to interpret the prolate symmetry of the AMS ellipsoid and its relationship to the deformation history. AMS of the sample is controlled by pyrrhotite porphyro…
Synthesis of self-assembled mesoporous 3D In2O3 hierarchical micro flowers composed of nanosheets and their electrochemical properties
2018
This report describes the methodology for the fabrication of mesoporous In2O3 microflowers by hydrothermal and calcination procedures in which In(OH)3/In2S3 acts as an intermediate. Both In2O3 and its precursor were analyzed with scanning electron microscopy, energy dispersive X-ray spectrophotometry, transmission electron microscopy and powder X-ray diffraction. BET surface area, pore size and pore volume analyses were also carried out. Electron microscopy images clearly evidence the self-assembly of 2D nanosheets into the micro flower structure. The mechanism of self-assembly and calcination is reported. Electrochemical properties of the synthesized In2O3 micro flowers were studied. peerR…