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
Descripción
Sumario: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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