Plasma fatty acid composition and lipid peroxide levels in children with paucity of interlobular bile ducts.
The total and free fatty acid composition of plasma and lipid peroxide concentrations was studied in 32 cholestatic children with syndromatic paucity of interlobular bile ducts (Alagille's syndrome). The mean lipid peroxide value in these patients was 8.80 +/- 3.70 nmol/ml, nearly 4 times higher than the mean control value. Compared to the control group, the patients exhibited significant variations in total fatty acids, and in particular a relative decrease in linoleic acid (from 29.5 +/- 6.1% in the controls to 19.1 +/- 8.03% in the patients) compensated by an increase in saturated and monounsaturated fatty acids. The plasma lipid peroxide levels were inversely correlated with the unsaturated/saturated fatty acids ratio in total fatty acids, and with the vitamin E status (vitamin E/total lipids). Most of the total and free fatty acid variations observed were largest in patients with severe jaundice. Dietary fat malabsorption and the increase in lipid peroxidation partly explain these results. Furthermore, in free fatty acids, we observed a marked increase in arachidonic acid (from 1.43 +/- 0.85% in the controls to 4.27 +/- 2.24% in the patients), suggesting abnormal eicosanoid synthesis.
Babin
F
F
Unité de Recherche d'Hépatologie Pédiatrique, INSERM U 56, Hôpital de Bicêtre, France.
Lemonnier
F
F
Goguelin
A
A
Alagille
D
D
Lemonnier
A
A
D000293:Adolescent; D001652:Bile Ducts / Q000002:abnormalities*; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000097:blood* / Q000209:etiology; D005227:Fatty Acids / Q000097:blood*; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005231:Fatty Acids, Unsaturated / Q000097:blood; D006801:Humans; D007223:Infant; D019787:Linoleic Acid; D008041:Linoleic Acids / Q000097:blood; D015227:Lipid Peroxidation; D008054:Lipid Peroxides / Q000097:blood*; D013577:Syndrome; D014810:Vitamin E / Q000097:blood / Q000494:pharmacology
D005227:Fatty Acids; D005230:Fatty Acids, Nonesterified; D005231:Fatty Acids, Unsaturated; D008041:Linoleic Acids; D008054:Lipid Peroxides; D014810:Vitamin E; D019787:Linoleic Acid; D001663:Bilirubin