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Year : 1968  |  Volume : 2  |  Issue : 1  |  Page : 68-72

Ochronotic Arthropathy

Correspondence Address:
N. D Aggarwal

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The report of this condition in an Egyptian mummy by Simons and Zorab (1961) makes us believe that this disorder is as old as man himself. The passage of dark coloured urine with or without associated symptoms was observed by physicians of the Middle Ages (quoted by O’Brien, La Du and Bunim 1963). The reducing nature of the substance responsible for the colour of urine in these patients was detected by Boedeker in 1961. He named this substance as alkaptone. Virchow in 1866 on the other hand recognized this condition in a post-mortem and named it ochronosis. Albrecht in 1902 was the first to establish the association of alkaptonuria and ochronosis during autopsy of a person known to be suffering from alkaptonuria without any associated symptoms. The first clinical diagnosis of ochronotic arthopathy was made by Sir William Osler in 1904 and since and then three different phases of the disease have become well established. In India this conditions has been reported by Bose and Ghosh (1929), Sarin and Bhargava (1957), Sinha (1930), Natarajan (1960) and Sachdeva and Wahi (1966). Pathogenesis- Alkaptonuria is an inherited error of metabolism, transmitted by a single recessive autosomal gene. Primary lesion is the absence of the enzyme homogentisic acid oxides from the liver and kidneys of the patient. This enzyme plays an important role in tyrosin breakdown. As a result of this the tyrosin breakdown stops short at the level of homogentisic acid which is passed in the urine. This acid has reducing property and on exposure to air gives brownish or black colour to the urine. The metabolic abnormality is present since birth and remains symtomless initially, being discovered only accidentally by the staining of diapers or clothes. In due course of time homogentisic acid gets deposited in the connective tissue of dermis, scleare, articular cartilages, ligaments and tendons and the skeletal symptoms commonly appear between 4th to 6th decades. The purpose of this paper is to present four cases of this rare disease with bilateral spontaneous rupture of tendo-achilles in one. It appears that only two cases of spontaneous rupture of tendo-achilles in ochronosis have been described in the literature so far.

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