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Journal of cardiovascular medicine (Hagerstown, Md.)

Black aorta in a patient with alkaptonuria (ochronosis).


PMID 21415764

Abstract

A rare cause of valvular heart disease is the deposition of foreign material in the valvular tissues, including material accumulating as a result of inborn errors of metabolism of the essential amino acids. Alkaptonuria can result in accumulation of homogentisic acid. We report the case of a patient with alkaptonuria undergoing surgery for aortic valve replacement.

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H0751
Homogentisic acid, crystalline
C8H8O4