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 #Alkaptonuria #BlackUrine #Peds #Pediatrics #Diagnosis #Pathophysiology
