Cardiomyopathy in Pompe's disease.
Level V
Where this comes from
- Record sourced from PubMed, PMID 18206603.
- Also identified by DOI 10.1016/j.ejim.2007.09.018.
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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.