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Asymptomatic during childhood, this accumulation will lead from the second decade of life to a progressive and severe spondylo\u2010arthopathy, associated with multisystem involvement: osteoporosis\/fractures, stones (renal, prostatic, gall bladder, salivary glands), ruptures of tendons\/muscle\/ligaments, renal failure and aortic valve disease. The pathophysiological mechanisms of AKU remain poorly understood, but recent advances lead us to reconsider the treatment strategy in AKU patients. Besides the supporting therapies (pain killers, anti\u2010inflammatory drugs, physiotherapy, joints replacements and others), specific therapies have been considered (anti\u2010oxidant, low protein diet, nitisinone), but clinical studies have failed to prove efficiency on the rheumatological lesions of the disease. Here we propose a treatment strategy for children and adults with AKU, based on a review of the latest findings on AKU and lessons from other aminoacipathies, especially tyrosinemias.<\/jats:p>","DOI":"10.1007\/s10545-015-9844-6","type":"journal-article","created":{"date-parts":[[2015,4,9]],"date-time":"2015-04-09T10:17:41Z","timestamp":1428574661000},"page":"791-796","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Old treatments for new insights and strategies: proposed management in adults and children with alkaptonuria"],"prefix":"10.1002","volume":"38","author":[{"given":"Jean\u2010Baptiste","family":"Arnoux","sequence":"first","affiliation":[{"name":"Reference Centre for Inherited Metabolic Diseases Necker\u2010Enfants Malades Hospital Assistance Publique \u2013 H\u00f4pitaux de Paris 149 rue de S\u00e8vres Paris 75015 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