Aortic Valve Replacement In Alkaptonuric Ochronosis.

Autor:	Rey EL; Service of Cardiac Surgery. University Hospital. Department of Surgery and Medico-Surgical Specialities. School of Medicine. University of Santiago de Compostela, Spain., Monzonís AM; Service of Cardiology. University Hospital, Santiago de Compostela, Spain., Bajula A; Service of Anesthesiology. University Hospital, Santiago de Compostela, Spain., Peñaranda JMS; Service of Pathology. University Hospital Santiago de Compostela, Spain., Fernández AL; Service of Cardiac Surgery. University Hospital. Department of Surgery and Medico-Surgical Specialities School of Medicine. University of Santiago de Compostela, Spain.
Jazyk:	angličtina
Zdroj:	Revista portuguesa de cirurgia cardio-toracica e vascular : orgao oficial da Sociedade Portuguesa de Cirurgia Cardio-Toracica e Vascular [Rev Port Cir Cardiotorac Vasc] 2020 Apr-Jun; Vol. 27 (2), pp. 121-123.
Abstrakt:	Alkaptonuria is an autosomal recessive inborn error of metabolism of the aromatic amino acids. Deficiency of the homogentisate1,2-dioxygenase leads to an increased blood and urinary concentration of homogentisc acid resulting in a slow accumulation of its oxidation products in the connective tissues (ochronosis). The most common clinical manifestation of ochronosis is arthropathy whereas cardiac involvement is very infrequent. We report the case of a patient with ochronotic involvement of the aortic valve who underwent a valve replacement. Some aspects of pathogenesis, and treatment are discussed.
Databáze:	MEDLINE
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