Treatment of
vitamin E deficiency during chronic childhood
cholestasis is hampered by the poor intestinal absorption of available oral preparations of
vitamin E when bile flow is severely impaired; thus parenteral
vitamin E has been the only effective
therapy for many children with this problem. We studied the intestinal absorption, efficacy, and safety of a water-soluble oral form of
vitamin E, d-
alpha-tocopheryl polyethylene glycol 1000 succinate (
TPGS), in 22 children (7 mo to 19 yr old) with severe
cholestasis and
vitamin E deficiency who were unresponsive to massive oral doses (100-200 IU/kg.day) of dl-
alpha-tocopherol. The results of oral
vitamin E tolerance tests showed that
TPGS was well absorbed in virtually all study subjects, that
TPGS intestinal absorption was superior to that of dl-
alpha-tocopherol, and that
TPGS absorption in teenage children with chronic
cholestasis was similar to that of normal adults. In addition, 1.7% +/- 1.6% (mean +/- SD) of the administered
polyethylene glycol 1000 contained in the
TPGS was absorbed and excreted in the urine of the 13 subjects analyzed, compared with 3.0% +/- 1.3% in 4 normal adults. A chronic oral dose of 15-25 IU/kg.day of
TPGS corrected the biochemical
vitamin E deficiency state over 1-19 mo (mean, 10.6 mo) of
TPGS therapy. No clinical or biochemical evidence of gastrointestinal, renal, hepatic, or hematologic toxicity was demonstrated. This study suggests that
TPGS administered orally in a dose of 15-25 IU/kg.day may be a safe and effective form of
vitamin E for prevention and correction of
vitamin E deficiency during severe childhood
cholestasis.