39 SIGNIFICANCE OF DL-ALPHA-TOCOPHERYL [α-T] ACETATE SUPPLEMENTATION IN PATIENTS WITH CYSTIC FIBROSIS (CF)

Abstract

Vitamin E deficiency [VED] in exocrine pancreatic insufficiency [EPI] and cholestatic liver disease [CLD] is well documented. In the clinical management of CF patients, high dose oral dl-α-T acetate supplements [α-TS] are routinely used. Yet, no data have been available concerning the need and effectiveness of these supplements.

In 52 CF patients (0.8-27.6 yrs), we investigated the significance of oral α-TS and the contribution of EPI (72 hr fecal fat, coefficient of fat absorption, absorbed fat [AF]), associated CLD (sonography, yGT, AP, serum bile acids) and nutritional status (weight, upper arm circumference [UAC], skinfolds) on α-T status. Fat malabsorption [FMA] was found in 48, CLD in 10, poor fat intake in 28, and malnutrition [UAC] in 9 patients. VED defined by plasma-α-T (1), RBC-α-T (2), and plasma-α-T/cholesterol (3) below the cutoff was found in 45% (1), 17% (2), 42% (3) of group A (50-600 mg/d α-TS, n=36) and 87%, 50%, 71% of group B (no α-TS, n=16), resp. 19%. 83%, 58% of group A had normal α-T, as did 13%, 50%, 29% of group B. There was no correlation between α-T and the total daily dose. α-T of 9 matched pairs (daily α-TS, degree of FMA) with and without CLD, revealed no significant difference. En patients with CLD, a multiplicative relationship (y=ax^b) between plasma-α-T/cholesterol and γGT was found (slope sign, at p=0.004). No regression model could be fitted for α-T depending on AF. In 2 out of 4 patients with corrected FMA, α-T deficiency was still present. In CF patients, high dose oral α-TS do not protect against α-T deficiency and do not correlate with α-T status. The prevalence of α-T deficiency varies markedly depending on the parameters used to evaluate α-T status. Indication and effectiveness of α-TS in CF patients should be documented. Special attention has to be paid to concomitant factors such as uncorrected FMA and CLD.

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