Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
5604164 | International Journal of Cardiology | 2017 | 6 Pages |
Abstract
Progressive gender-specific cardiac pathology and clinical manifestations were documented in affected Classic family members. Increasing cardiomyocyte diameter was correlated with disease severity, age, and gender. Fibrosis was presumably caused by cell death of enlarged, substrate-engorged cardiomyocytes. These results support early enzyme therapy in Classic males to prevent/minimize irreversible cardiac damage.
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Authors
Andrea Frustaci, Cristina Chimenti, Dana Doheny, Robert J. Desnick,