The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
Identifikátory výsledku
Kód výsledku v 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>
Nalezeny alternativní kódy
RIV/00216208:11110/19:10399930
Výsledek na webu
<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>
Alternativní jazyky
Jazyk výsledku
angličtina
Název v původním jazyce
The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
Popis výsledku v původním jazyce
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.
Název v anglickém jazyce
The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease
Popis výsledku anglicky
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.
Klasifikace
Druh
J<sub>imp</sub> - Článek v periodiku v databázi Web of Science
CEP obor
—
OECD FORD obor
30201 - Cardiac and Cardiovascular systems
Návaznosti výsledku
Projekt
—
Návaznosti
V - Vyzkumna aktivita podporovana z jinych verejnych zdroju
Ostatní
Rok uplatnění
2019
Kód důvěrnosti údajů
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Údaje specifické pro druh výsledku
Název periodika
JACC: Cardiovascular Imaging
ISSN
1936-878X
e-ISSN
—
Svazek periodika
12
Číslo periodika v rámci svazku
7
Stát vydavatele periodika
US - Spojené státy americké
Počet stran výsledku
13
Strana od-do
1230-1242
Kód UT WoS článku
000473334100016
EID výsledku v databázi Scopus
2-s2.0-85067503375