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High tolerance for oral galactose in classical galactosaemia: dietary implications
  1. A M Bosch,
  2. H D Bakker,
  3. L J M de B Wenniger-Prick,
  4. R J A Wanders,
  5. F A Wijburg
  1. Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands
  1. Correspondence to:
    Dr F A Wijburg
    Paediatrician Metabolic Diseases, Academic Medical Center, University of Amsterdam, Department of Pediatrics (G8-205), PO Box 22660, 1100 DD Amsterdam, Netherlands; F.A.Wijburgamc.uva.nl

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.

  • diet
  • galactitol
  • galactose-1-phosphate
  • galactose-1-phosphate uridyl transferase
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