title: Mechanism causing vitamin E deficiency during chronic childhood cholestasis.
abstract: In order to characterize the mechanism(s) causing vitamin E deficiency during chronic childhood cholestasis, we studied 6 vitamin E-deficient cholestatic children with clinical evidence of neurologic dysfunction (group A), 4 vitamin E-sufficient cholestatic children and young adults with normal neurologic status (group B), and 6 vitamin E-sufficient noncholestatic children (group C). Intestinal absorption of dl-alpha-tocopherol (assessed by an oral tolerance test) was markedly impaired (p less than 0.001) in group A compared with groups B and C, which did not differ from each other. Intraluminal total bile acid concentrations were markedly depressed in group A compared with age-matched controls (0.50 vs. 7.00 mM, p less than 0.001), whereas concentrations were low normal in group B. Intramuscular dl-alpha-tocopherol was well absorbed in 4 group A subjects and corrected abnormal hydrogen peroxide hemolysis. Our data suggest that low intraluminal bile acid concentrations result in malabsorption and deficiency of vitamin E in children with prolonged, severe cholestasis. Intact plasma transport and tissue uptake of vitamin E during cholestasis suggest that intramuscular vitamin E should be utilized for prevention and therapy of the neurologic abnormalities caused by vitamin E deficiency.
mesh_terms: D000293:Adolescent; D000328:Adult; D001647:Bile Acids and Salts / Q000378:metabolism; D001692:Biological Transport; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000150:complications*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007273:Injections, Intramuscular; D007408:Intestinal Absorption; D008297:Male; D013997:Time Factors; D014810:Vitamin E / Q000008:administration & dosage / Q000378:metabolism; D014811:Vitamin E Deficiency / Q000209:etiology* / Q000378:metabolism / Q000517:prevention & control
chemical_list: D001647:Bile Acids and Salts; D014810:Vitamin E