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A common feature of the two reported cases is nerve deafness. Galactose\u20101\u2010phosphate uridyl transferase deficiency poses the greatest problems because of the poor long\u2010term outcome in spite of a galactose\u2010restricted diet, and with no clear indications of how and when the underlying damage occurs. Recent evidence of low erythrocyte and tissue UDPgal levels, associated with ovarian dysfunction, may indicate impaired galactoside synthesis. Administration of uridine corrects the UDPgal depletion and trials in which it is added to the galactose\u2010restricted diet have begun.<\/jats:p>","DOI":"10.1007\/bf01799505","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T14:02:35Z","timestamp":1119362555000},"page":"476-486","source":"Crossref","is-referenced-by-count":19,"title":["Galactose disorders: An overview"],"prefix":"10.1002","volume":"13","author":[{"given":"J. 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