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Pediatric Cardiology
Published in: Pediatric Cardiology 8/2013

Open Access 01-12-2013 | Case Report

Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy

Authors: Yohei Sato, Masako Fujiwara, Hiroshi Kobayashi, Hiroyuki Ida

Published in: Pediatric Cardiology | Issue 8/2013

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Abstract

This report describes the pathologic findings for a patient with Hunter syndrome who underwent aortic valve replacement at 10 years of age, 3 years after the initiation of enzyme replacement therapy. Aortic valve pathology showed mild thickening and fibrosis as well as massive glycosaminoglycan accumulation. This suggests that enzyme replacement therapy has limited efficacy for cardiac valve disease both clinically and pathologically.
Literature
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Metadata
Title
Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
Authors
Yohei Sato
Masako Fujiwara
Hiroshi Kobayashi
Hiroyuki Ida
Publication date
01-12-2013
Publisher
Springer US
Published in
Pediatric Cardiology / Issue 8/2013
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-013-0653-0

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