High tolerance for oral galactose in classical galactosaemia: dietary implications. Issue 11 (21st October 2004)

• Record Type: Journal Article
• Authors: Bosch, A M; Bakker, H D; de B Wenniger-Prick, L J M; Wanders, R J A; Wijburg, F A
• Is Part Of: Archives of disease in childhood. Volume 89:Issue 11(2004)
• Page Start: 1034
• Page End: 1036
• Publication Date: 2004-10-21
• Subjects: diet -- galactitol -- galactose-1-phosphate -- galactose-1-phosphate uridyl transferase; Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/adc.2003.037671
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Aim: To study the relevance of restricting the exogenous intake of small amounts of galactose, such as from fruit and vegetables, in patients with classical galactosaemia. Methods: For a period of six weeks, increasing doses of oral galactose to a maximum of 600 mg per day, were added to a very strict galactose restricted diet in three adolescent patients homozygous for the Q188R mutation. During the study, physical examination, including an extended ophthalmic examination, and laboratory studies were performed on a weekly basis. Results: No significant change in any of the studied clinical or biochemical parameters was observed. Conclusions: These findings provide further evidence that attempts to exclude trace amounts of galactose from the diet are not justified. Once the diet is made more liberal, a long term follow up study will be necessary.