Article
New insights in cardiac structural changes in patients with Fabry's disease.
American heart journal - 1 Jun 2000
Linhart A, Palecek T, Bultas J, Ferguson J J, Hrudová J, Karetová D, Zeman J, Ledvinová J, Poupetová H, Elleder M, Aschermann M
Abstract excerpt
BACKGROUND: Fabry's disease is an X-linked recessive genetic deficiency of the enzyme alpha-galactosidase leading to the pathologic intracellular deposition of neutral glycosphingolipids. Although cardiac involvement is frequent, there is controversy regarding the character of the associated left ventricular (LV) changes and the severity of valvular involvement. METHODS: Clinical evaluation (disease severity...
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