Alkaptonuria is a rare inherited genetic disorder of tyrosine metabolism that causes the urine to turn black on contact with the air when homogentisic acid is oxidized to form a pigment-like polymer material [1,2]. The earliest sign of the disorder is the tendency for babies’ diapers to stain black; later on in childhood and early adulthood, there is an asymptomatic, progressive deposition of the polymer into collagenous tissues [2–4]. One report suggests an incidence as high as 1 in every 25 000 live births, although worldwide it is certainly far lower [1,4–6]. Our objective in reporting this case study is to highlight the importance of early detection of the disease by paediatricians, to raise awareness and to prevent late complications by simple treatment.
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