European Journal of Internal Medicine
Volume 19, Issue 1 , Pages 57-59, January 2008

Cardiomyopathy in Pompe's disease

Cardiologie, CHU Bicêtre, APHP, Le Kremlin Bicetre, France

Received 26 July 2007; accepted 26 September 2007. published online 22 November 2007.

Abstract 

Pompe's disease (glycogen storage disease type II) is a lysosomal storage disorder resulting from a deficiency in alpha 1, 4 glucosidase. Prognosis is poor because of heart involvement. Treatment in adult form relies on supportive therapy. Enzyme replacement therapy with recombinant human alpha glucosidase remains a hope for patients.

Keywords: Pompe's disease, Alpha 1,4 glucosidase, Acid maltase, Glycogen storage disease type II enzyme replacement, Doppler tissue velocity imaging

To access this article, please choose from the options below

Login to an existing account or Register a new account.

  • Purchase this article for 31.50 USD (You must login/register to purchase this article)

    Online access for 24 hours. The PDF version can be downloaded as your permanent record.

  • Subscribe to this title

    Get unlimited online access to this article and all other articles in this title 24/7 for one year.

  • Claim access now

    For current subscribers with Society Membership or Account Number.

  • Visit SciVerse ScienceDirect to see if you have access via your institution.
 

PII: S0953-6205(07)00254-3

doi:10.1016/j.ejim.2007.09.018

European Journal of Internal Medicine
Volume 19, Issue 1 , Pages 57-59, January 2008

Article Tools

* Image Usage & Resolution