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    Eur J Intern Med. 2008 Jan;19(1):57-9. Epub 2007 Nov 26.

    Cardiomyopathy in Pompe's disease.

    Source

    Cardiologie, CHU Bicêtre, APHP, Le Kremlin Bicetre, France. fayssoil2000@yahoo.fr.

    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.

    PMID:
    18206603
    [PubMed - indexed for MEDLINE]

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