1. Alkaptonuria-associated aortic stenosis.
- Author
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Lok ZS, Goldstein J, and Smith JA
- Subjects
- Aged, Alkaptonuria metabolism, Aortic Valve pathology, Aortic Valve surgery, Aortic Valve Stenosis pathology, Heart Valve Prosthesis, Heart Valve Prosthesis Implantation, Homogentisic Acid metabolism, Humans, Male, Middle Aged, Myocardium metabolism, Treatment Outcome, Tyrosine metabolism, Alkaptonuria complications, Aortic Valve Stenosis etiology, Aortic Valve Stenosis surgery
- Abstract
Alkaptonuria is an autosomal recessive disorder of tyrosine metabolism, which results in accumulation of unmetabolized homogentisic acid and its oxidized product in various tissues, including the heart. Cardiovascular involvement is a rare but serious complication of the disease. We present two patients who have undergone successful aortic valve replacement for alkaptonuria-associated aortic stenosis along with a review of the literature., (© 2013 Wiley Periodicals, Inc.)
- Published
- 2013
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