Alkaptonuria (Black Urine Disease)

Urine turns dark when left standing due to oxidation of homogentisic acid 

Clinical 

 • Usually asymptomatic in childhood 

 • Ochronotic arthritis (due to deposits) 

 • Calcification of multiple intervertebral discs 

Management 

 • No effective therapy is available 

 • Diet: limit tyrosine and phenylalanine 



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