The effect of diet on Paraoxonase 1/Arylesterase activities in patients with disorders of galactose metabolism.
OBJECTIVE
To investigate the effects of diet on the antiatherogenic enzyme Paraoxonase 1/Arylesterase (PON1/Aryl) activities in patients with disorders of galactose metabolism.


METHODS
Eleven poorly dietary controlled children with classical galactosaemia (GALT deficiency), 7 with epimerase deficiency and 12 with duarte 1 variant 'off diet' underwent clinical and laboratory investigations before and after 10 days on galactose restricted diet whereas controls (N = 20) were examined once. Serum lipids, lipoproteins and apolipoprotein A1 (ApoA1) were measured with routine methods, PON1/Aryl activities and total antioxidant status (TAS) spectrophotometrically, and galactose-1-phosphate (Gal-1-P) enzymatically.


RESULTS
Lipids, lipoproteins, ApoA1, PON1/Aryl, TAS remained unaltered in all groups, except in those with classical galactosaemia pre- versus postdiet. In patients with classical galactosaemia, TAS, PON1, Aryl (0.98 +/- 0.2 mmol/l, 60 +/- 12 U/min/ml, 56 +/- 16 KU/min/ml, respectively) were significantly reduced prediet as compared with those postdiet (1.63 +/- 0.2 mmol/l, 136 +/- 15 U/min/ml, 112 +/- 18 KU/min/ml, respectively; P < 0.001) and controls. The enzyme activities positively correlated with TAS (r = 0.56, P < 0.001) in all groups and negatively with Gal-1-P (r = -0.54, P < 0.001) in group with GALT deficiency.


CONCLUSIONS
Low TAS and high Gal-1-P levels may reduce PON1/Aryl activities. Patients with classical galactosaemia, when on strict diet, may benefit with a generous antiatherogenic capacity.
Schulpis
Kleopatra H
KH
Institute of Child Health, Research Centre, 'Aghia Sophia' Children's Hospital, Athens, Greece.
Karikas
George A
GA
Bartzeliotou
Anastasia
A
Papakonstantinou
Evangelos D
ED
Kalogerakou
Maria
M
Tsakiris
Stylianos
S
D000975:Antioxidants / Q000032:analysis; D016632:Apolipoprotein A-I / Q000097:blood; D043303:Aryldialkylphosphatase / Q000378:metabolism*; D002265:Carboxylic Ester Hydrolases / Q000378:metabolism*; D016022:Case-Control Studies; D002648:Child; D004032:Diet; D005500:Follow-Up Studies; D005690:Galactose / Q000097:blood; D005693:Galactosemias / Q000178:diet therapy* / Q000378:metabolism; D005695:Galactosephosphates / Q000097:blood; D006801:Humans; D008055:Lipids / Q000097:blood; D008074:Lipoproteins / Q000097:blood; D013053:Spectrophotometry
D000975:Antioxidants; D016632:Apolipoprotein A-I; D005695:Galactosephosphates; D008055:Lipids; D008074:Lipoproteins; C029973:galactose-1-phosphate; D002265:Carboxylic Ester Hydrolases; C019734:arylesterase; D043303:Aryldialkylphosphatase; D005690:Galactose