High tolerance for oral galactose in classical galactosaemia: dietary implications

A.M. Bosch, H.D. Bakker, L.J.J.M. Wenniger-Prick, R.J.A. Wanders, F.A. Wijburg

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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
Original languageUndefined/Unknown
Pages (from-to)1034-1036
JournalArchives of disease in childhood
Volume89
Issue number11
DOIs
Publication statusPublished - 2004

Keywords

  • AMC wi-co

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