Document Detail


Impact of enzyme replacement therapy on cardiac morphology and function and late enhancement in Fabry's cardiomyopathy.
MedLine Citation:
PMID:  16679096     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The present study evaluated the evolution of cardiac morphology, function, and late enhancement as a noninvasive marker of myocardial fibrosis, and their inter-relation during enzyme replacement therapy in patients with Fabry's disease using magnetic resonance imaging and color Doppler myocardial imaging. Late enhancement, which was present in up to 50% of patients, was associated with increased left ventricular mass, the failure of a significant regression of hypertrophy during enzyme replacement therapy, and worse segmental myocardial function. Late enhancement may predict the effect of enzyme replacement therapy on left ventricular mass and cardiac function.
Authors:
Meinrad Beer; Frank Weidemann; Frank Breunig; Anita Knoll; Sabrina Koeppe; Wolfram Machann; Dietbert Hahn; Christoph Wanner; Jörg Strotmann; Jörn Sandstede
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2006-03-29
Journal Detail:
Title:  The American journal of cardiology     Volume:  97     ISSN:  0002-9149     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  2006 May 
Date Detail:
Created Date:  2006-05-08     Completed Date:  2006-06-23     Revised Date:  2007-02-16    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1515-8     Citation Subset:  AIM; IM    
Affiliation:
Institut für Röntgendiagnostik, Würzburg, Germany. beer@roentgen.uni-wuerzburg.de
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MeSH Terms
Descriptor/Qualifier:
Adult
Contrast Media
Echocardiography, Doppler
Fabry Disease / drug therapy*,  physiopathology,  ultrasonography
Female
Gadolinium DTPA / diagnostic use
Humans
Magnetic Resonance Imaging
Male
Statistics, Nonparametric
Treatment Outcome
alpha-Galactosidase / therapeutic use*
Chemical
Reg. No./Substance:
0/Contrast Media; 80529-93-7/Gadolinium DTPA; EC 3.2.1.22/alpha-Galactosidase
Comments/Corrections
Comment In:
Am J Cardiol. 2006 Dec 1;98(11):1557-8   [PMID:  17126672 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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