High tolerance for oral galactose in classical galactosaemia: dietary implications.
OBJECTIVE
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.
Bosch
A M
AM
Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands.
Bakker
H D
HD
Wenniger-Prick
L J M de B
LJ
Wanders
R J A
RJ
Wijburg
F A
FA
D000284:Administration, Oral; D000293:Adolescent; D004361:Drug Tolerance; D005260:Female; D005638:Fruit / Q000737:chemistry; D004376:Galactitol / Q000652:urine; D005690:Galactose / Q000008:administration & dosage*; D005693:Galactosemias / Q000097:blood / Q000178:diet therapy* / Q000652:urine; D005695:Galactosephosphates / Q000097:blood; D006801:Humans; D008297:Male; D014675:Vegetables / Q000737:chemistry
D005695:Galactosephosphates; D004376:Galactitol; C029973:galactose-1-phosphate; D005690:Galactose