Treatment of cholestatic children with water-soluble vitamin E (alpha-tocopheryl polyethylene glycol succinate): effects on serum vitamin E, lipid peroxides, and polyunsaturated fatty acids.

BACKGROUND: Treatment of vitamin E-deficient cholestatic children with water-soluble alpha-tocopherol polyethylene glycol succinate (TPGS) was previously shown to normalize vitamin E status and to improve neurological outcome.

METHODS: Because vitamin E plays an important role as a free-radical scavenger, we studied the effects of long-term TPGS supplementation on lipid peroxidation and polyunsaturated fatty acid status in 15 children ages 9 months-3.4 years (median, 1.3 years) with chronic cholestasis with low serum vitamin E concentrations [1.95 (0.8-3.7) mg/L; median (1st-3rd quartile)]. The previous supplementation of alpha-tocopherol was replaced by a 20% solution of TPGS in one daily dose of 20 IU/kg. Serum alpha-tocopherol, plasma lipid peroxides expressed as thiobarbiturate reactive substance concentration (TBARS) and plasma phospholipid fatty acid profile were estimated at baseline and again after 1 month in all 15 patients, and after 1 year of TPGS therapy in 11 patients.

RESULTS: alpha-Tocopherol was significantly increased after 1 month [6.9 (4.4-8.4) mg/L; p = 0.008] and rose further after 1 year [9.7 (7.2-14.9) mg/L]; similar results were obtained for the ratio vitamin E/total lipids. TBARS concentrations were significantly higher in cholestatic children at baseline [2.9 (1.5-3.32) nmol/ml] than in a control group [1.2 (1.1-1.3) nmol/ml; p = 0.0006], but were not changed significantly during TPGS therapy [after 1 year 2.34 (1.9-3.0) nmol/ml]. Compared with controls, the contributions of polyunsaturated fatty acids to total phospholipid fatty acids were markedly decreased in cholestatic patients at baseline [27.7 (22.4-31.5)% versus 36.9 (34.5-39.0)%; p = 0.001] and did not show major changes after 1 year of TPGS supplementation.

CONCLUSIONS: We conclude that oral TPGS supplementation of cholestatic children can quickly normalize serum vitamin E levels but does not improve the increased lipid peroxidation and poor polyunsaturated fatty acid status.