Cardiomyopathy in Pompe's disease.
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.
Fayssoil
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Cardiologie, CHU Bicêtre, APHP, Le Kremlin Bicetre, France. fayssoil2000@yahoo.fr.
D009202:Cardiomyopathies / Q000209:etiology* / Q000503:physiopathology* / Q000628:therapy; D006009:Glycogen Storage Disease Type II / Q000150:complications* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans