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

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 accu...

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Detalles Bibliográficos
Autores principales: Sato, Yohei, Fujiwara, Masako, Kobayashi, Hiroshi, Ida, Hiroyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2013
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3825558/
https://www.ncbi.nlm.nih.gov/pubmed/23404265
http://dx.doi.org/10.1007/s00246-013-0653-0
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author Sato, Yohei
Fujiwara, Masako
Kobayashi, Hiroshi
Ida, Hiroyuki
author_facet Sato, Yohei
Fujiwara, Masako
Kobayashi, Hiroshi
Ida, Hiroyuki
author_sort Sato, Yohei
collection PubMed
description 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.
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spelling pubmed-38255582013-11-21 Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy Sato, Yohei Fujiwara, Masako Kobayashi, Hiroshi Ida, Hiroyuki Pediatr Cardiol Case Report 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. Springer US 2013-02-13 2013 /pmc/articles/PMC3825558/ /pubmed/23404265 http://dx.doi.org/10.1007/s00246-013-0653-0 Text en © The Author(s) 2013 https://creativecommons.org/licenses/by/2.0/ Open AccessThis article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.
spellingShingle Case Report
Sato, Yohei
Fujiwara, Masako
Kobayashi, Hiroshi
Ida, Hiroyuki
Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title_full Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title_fullStr Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title_full_unstemmed Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title_short Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy
title_sort massive accumulation of glycosaminoglycans in the aortic valve of a patient with hunter syndrome during enzyme replacement therapy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3825558/
https://www.ncbi.nlm.nih.gov/pubmed/23404265
http://dx.doi.org/10.1007/s00246-013-0653-0
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