Ochronosis

✍ Scribed by G. K. Rose

• Book ID: 102768609
• Publisher: John Wiley and Sons
• Year: 1957
• Tongue: English
• Weight: 572 KB
• Volume: 44
• Category: Article
• ISSN: 0007-1323
• DOI: 10.1002/bjs.18004418708

✦ Synopsis

OCHRONOSIS BY G. K. ROSE CONSULTING ORTHOPEDIC SURGEON TO THE ROBERT JONES AND AGNES HUNT ORTHOPEDIC HOSPITAL AND THE ROYAL SALOP INFIRMARY ALCAPTONURIA is the manifestation in an individual of failure to break down homogentisic acid, which is one of the intermediate products of tyrosine metabolism, and is presumed to be due to the hereditary deficiency of the enzymes necessary to complete this metabolism. Large quantities of homogentisic acid are therefore excreted in the urine, and this on standing, particularly when alkaline, gradually turns black. An acquired form of the disease occurs following the administration of phenol compounds, and though this is accompanied by pigmentation, it is doubtful whether the pigment in this case is identical with that in alcaptonuric ochronosis. Marcet, in 1823, is credited with noting for the first time a substance in the urine which caused a black stain on cloth. Boedeker in 1861 named this