The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064165%3A_____%2F19%3A10399930" target="_blank" >RIV/00064165:_____/19:10399930 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11110/19:10399930
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=m.YkVTTpLE" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=m.YkVTTpLE</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1016/j.jcmg.2018.11.039" target="_blank" >10.1016/j.jcmg.2018.11.039</a>
Alternative languages
Result language
angličtina
Original language name
The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
Original language description
Anderson-Fabry disease (AFD) is a rare X-linked inherited metabolic disorder which results in a deficiency or absence of the enzyme alpha-galactosidase A, leading to the accumulation of glycosphingolipids in various cells and organs including the heart. Cardiac involvement is common and results in increased myocardial inflammation, left ventricular hypertrophy (LVH), and myocardial fibrosis. Echocardiography and cardiovascular magnetic resonance (CMR) offer distinctive and often complementary use to assist in the diagnosis and monitoring pharmacologic therapy in AFD, including detection of the AFD cardiac phenotype, differentiation from other forms of LVH, and patient selection for therapeutic intervention. Advanced cardiac imaging holds promise in subclinical detection of AFD-related abnormalities as well as disease staging and prognostication. Crown Copyright (C) 2019 Published by Elsevier on behalf of the American College of Cardiology Foundation. All rights reserved.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30201 - Cardiac and Cardiovascular systems
Result continuities
Project
—
Continuities
V - Vyzkumna aktivita podporovana z jinych verejnych zdroju
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
JACC: Cardiovascular Imaging
ISSN
1936-878X
e-ISSN
—
Volume of the periodical
12
Issue of the periodical within the volume
7
Country of publishing house
US - UNITED STATES
Number of pages
13
Pages from-to
1230-1242
UT code for WoS article
000473334100016
EID of the result in the Scopus database
2-s2.0-85067503375