Adhering to a galactose-free diet by strictly avoiding dairy products
and known hidden sources of galac-tose does not completely normalize g
alactose-1-phosphate (gal-1-P) in erythrocytes from patients with gala
ctosemia. Major neurological complications, even in the best treated p
atients, are threatening a good clinical outcome and dictate a contino
us search for leaks in the dietary regimen. Raffinose and stachyose, p
resent in important amounts in various vegetables, contain alpha-1,4 l
inked galactose which is cleaved only by bacterial alpha-galactosidase
s, presumably in the lower part of the gut. In order to test the hypot
hesis whether galactose released from raffinose and stachyose could be
a source of absorbed galactose and a cause of elevated gal-1-P six pa
tients with galactosemia (aged 6-24 years), underwent a raffinose- and
stachyose-poor dietary regimen for 2 weeks. Before, after, and during
the test period, the daily intake of stachyose and raffinose as well
of protein, carbohydrate, fat and minerals was calculated from food pr
otocols obtained from the patients. Plasma galactose and erythrocyte g
al-1-P were measured at the end of the three test phases. Stachyose an
d raffinose intake was reduced to 5%-10% during the experimental diet,
which was well tolerated, except for constipation in some patients. I
n five of the six patients gal-1-P in erythrocytes was somewhat lower
(statistically not significant) during the test phase than during regu
lar diet while plasma galactose remained unchanged. Galactose released
from raffinose ana stachyose may be absorbed and contribute to elevat
ed gal-1-P values in erythrocytes of galactosemic patients.