Articles with "anderson fabry" as a keyword



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Cardiac tissue characterization with t1 and t2 mapping in anderson fabry patients: new pathophysiological concepts and early cardiac involvement

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Published in 2020 at "European Heart Journal"

DOI: 10.1093/ehjci/ehaa946.0264

Abstract: Anderson Fabry (AF) disease is a X-linked lysosomal storage disorder with multiorgan involvement. Cardiac disease, mainly represented by left ventricular hypertrophy (LVH) and arrhythmias, is the most frequent cause of premature death. It is well… read more here.

Keywords: early cardiac; tissue; tissue characterization; cardiac involvement ... See more keywords
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Hybrid positron emission tomography-magnetic resonance imaging for assessing different stages of cardiac impairment in patients with Anderson-Fabry disease: AFFINITY study group.

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Published in 2019 at "European heart journal cardiovascular Imaging"

DOI: 10.1093/ehjci/jez039

Abstract: AIMS Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with multi-organ dysfunction. While native myocardial T1 mapping by magnetic resonance (MR) allow non-invasive measurement of myocyte sphingolipid accumulation, 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography… read more here.

Keywords: magnetic resonance; fabry disease; positron emission; anderson fabry ... See more keywords
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Segment-by-segment assessment of left ventricular myocardial affection in Anderson-Fabry disease by non-enhanced T1-mapping

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Published in 2017 at "Acta Radiologica"

DOI: 10.1177/0284185116675657

Abstract: Background Anderson-Fabry disease (AFD) is an X-linked lysosomal enzyme disorder associated with an intracellular accumulation of sphingolipids, which shorten myocardial T1 relaxation times. Myocardial affection, however, varies between different segments. Purpose To evaluate the specific… read more here.

Keywords: segment; fabry disease; affection; non enhanced ... See more keywords
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Anderson–Fabry disease

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Published in 2018 at "Journal of Cardiovascular Medicine"

DOI: 10.2459/jcm.0000000000000637

Abstract: Onset is usually in childhood. Often patients have slight builds with characteristic coarse facial features and delayed puberty. Early symptoms of burning pain and paraesthesia in the extremities (acroparaesthesia) are a major cause of morbidity.… read more here.

Keywords: medicine; fabry disease; disease; decade ... See more keywords

Gastrointestinal Involvement in Anderson-Fabry Disease: A Narrative Review

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Published in 2021 at "International Journal of Environmental Research and Public Health"

DOI: 10.3390/ijerph18063320

Abstract: Anderson-Fabry disease (FD) is an X-linked lysosomal storage disorder leading to a wide array of clinical manifestations. Among these, gastrointestinal (GI) symptoms such as abdominal pain, bloating, and diarrhea affect about half of the FD… read more here.

Keywords: narrative review; fabry disease; anderson fabry; disease ... See more keywords