Abstract
Chronic malabsorption and deficiency of vitamin E (E) during chronic childhood cholestasis (CCC) leads to a progressive degenerative neurologic disorder. The treatment of E deficiency during severe CCC is hampered by the inability of many patients to absorb even massive oral doses of available E preparations, requiring therapy with parenteral forms of E. This study was designed to evaluate intestinal absorption of, and chronic therapy with, a water-soluble form of E (TPGS) during CCC. We studied 6 children (ages 8 mo-14 yr) with well documented E deficiency caused by CCC, each of whom had failed to show increase in serum E levels (3.9-15.5 μg/ml=Nl) or ratios of serum E to total lipids (E/L; >0.6 mg/g=Nl) after 1-3 months of therapy with 60-150 IU/kg/day of oral dl-α-tocopherol. An oral E tolerance test (OVETT) was performed using 100 IU/kg of TPGS. Serum E (fluorometric method) and total lipid levels (colorimetric method) were drawn at 0, +6, +12, +18, +24, +36, and +48 hours. Each child was then treated with 50 IU/kg/day of TPGS; E status monitored after 2 and 4 weeks. Results (x + SEM) in Table.
Conclusion: TPGS is absorbed enterally during severe CCC, corrects the biochemical indices of E deficiency, and, therefore, should be considered in those children with CCC unresponsive to available forms of oral E.
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Sokol, R., Butler-Simon, N. & Silverman, A. 730 TREATMENT OF VITAMIN E DEFICIENCY IN CHRONIC CHILDHOOD CHOLESTASIS WITH ORAL d-α-TOCOPHERYL POLYETHYLENE GLYCOL-1000 SUCCINATE (TPGS). Pediatr Res 19, 232 (1985). https://doi.org/10.1203/00006450-198504000-00760
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DOI: https://doi.org/10.1203/00006450-198504000-00760