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Correlation Between Plasma and Liver Concentrations of Vitamins A and E in Patients with Cystic Fibrosis (CF)

This study was designed to determine if the low plasma concentrations of vitamins A and E often seen in children with CF are a reflection of tissue depletion or of a defect in transport mechanisms. Ten patients with CF, ranging in age from 9 to 23 years, were studied. Plasma concentrations of vitamins A and E and carotene were measured in 7 of the 10 patients during the 18 months prior to death. At autopsy all 10 subjects had repeat measurements of these parameters plus an assessment of liver concentrations of vitamin A and α-tocopherol. All patients had received regular supplementation with vitamin A at twice the recommended daily allowance for several years; however, plasma levels of this vitamin were consistently below normal and carotene values were remarkably low in all subjects. Nine of the 10 CF patients had normal or increased vitamin A concentrations in the liver. Eight of the 10 patients received no supplemental vitamin E and had plasma vitamin E levels than 0.4 mg/100 ml and liver concentrations of α-tocopherol less than half that of the control group. This study has shown that the low plasma concentrations of vitamin E do correlate with decreased liver stores of α-tocopherol. (Supported by grants from the National Cystic Fibrosis Research Foundation and the Nutrition Foundation.)