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Due to deficiency of Homogentisic acid oxidase which causes large amount of homogentisic acid to accumulate in the body and then to be excreted in urine.


Clinical Feature

  1. Consists of ochronosis and arthritis
  2. These findings may not be come evident until mid adult life. The only sign of the disorder in the Pediatric age group is darkening of the urine to almost black color on standing.
  3. This is caused by oxidation and polymerization of the homogentisic acid and is enhanced with an alkaline pH.
    Ochronosis is a term used to describe the darkening of tissue and is due to a slow accumulation of the black polymer of Homogentisic in cartilage and other mesenchymal tissues. Q

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