Source:http://linkedlifedata.com/resource/pubmed/id/17483124
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
2
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| pubmed:dateCreated |
2008-1-15
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| pubmed:abstractText |
Anderson-Fabry disease is an X-linked glycosphingolipid storage disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. This leads to a progressive accumulation of globotriaosylceramide (Gb(3)) in the lysosomes of cells throughout the body that ultimately results in premature death from renal, cardiac or cerebrovascular complications. Until recently, there was no effective therapy available for this disease. The present study was designed to assess the safety and efficacy of enzyme replacement therapy with agalsidase alfa on the cardiac manifestations of Anderson-Fabry disease.
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| pubmed:commentsCorrections | |
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
AIM
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| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Feb
|
| pubmed:issn |
1468-201X
|
| pubmed:author | |
| pubmed:issnType |
Electronic
|
| pubmed:volume |
94
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
153-8
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| pubmed:meshHeading |
pubmed-meshheading:17483124-Adult,
pubmed-meshheading:17483124-Aged,
pubmed-meshheading:17483124-Cardiomyopathies,
pubmed-meshheading:17483124-Chromatography, High Pressure Liquid,
pubmed-meshheading:17483124-Double-Blind Method,
pubmed-meshheading:17483124-Echocardiography,
pubmed-meshheading:17483124-Electrocardiography,
pubmed-meshheading:17483124-Fabry Disease,
pubmed-meshheading:17483124-Heart Conduction System,
pubmed-meshheading:17483124-Humans,
pubmed-meshheading:17483124-Hypertrophy, Left Ventricular,
pubmed-meshheading:17483124-Magnetic Resonance Angiography,
pubmed-meshheading:17483124-Male,
pubmed-meshheading:17483124-Middle Aged,
pubmed-meshheading:17483124-Myocardium,
pubmed-meshheading:17483124-Trihexosylceramides,
pubmed-meshheading:17483124-Ventricular Dysfunction, Left,
pubmed-meshheading:17483124-alpha-Galactosidase
|
| pubmed:year |
2008
|
| pubmed:articleTitle |
Effects of enzyme replacement therapy on the cardiomyopathy of Anderson-Fabry disease: a randomised, double-blind, placebo-controlled clinical trial of agalsidase alfa.
|
| pubmed:affiliation |
Department of Academic Haematology, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK. d.hughes@medsch.ucl.ac.uk
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| pubmed:publicationType |
Journal Article,
Randomized Controlled Trial,
Research Support, Non-U.S. Gov't
|