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Black aorta in a patient with alkaptonuria (ochronosis).

Giovanni Concistrè, Brenno Fiorani, Federico Ranocchi, Giovanni Casali, Antonio Loforte, Francesco Musumeci
Journal of Cardiovascular Medicine 2011 June
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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