Plasma micronutrient concentrations in infants undergoing therapy for phenylketonuria.
[classical phenylketonuria]
Twenty-seven infants with classical phenylketonuria were evaluated longitudinally for 6 mo while ingesting Phenex-1 Amino Acid Modified Medical Food With Iron as their primary protein source. Intake of selected nutrients and biochemical indices of trace and ultratrace mineral status and plasma retinol and alpha-tocopherol concentrations were evaluated. The means of iron status indices (complete blood count, plasma ferritin, iron, transferrin saturation, total iron binding capacity) and the plasma concentrations of trace and ultratrace minerals (copper, manganese, molybdenum, selenium, zinc) and plasma retinol and alpha-tocopherol were in the reference ranges. Vitamin A intakes (r = 0.49, p < 0.05) and plasma retinol-binding protein concentrations (r = 0.42, p < 0.05) were positively correlated with plasma retinol concentrations at 3 mo of study. At 6 mo, concentrations of plasma transthyretin (r = 0.72, p < 0.01) and retinol-binding protein (r = 0.48, p < 0.05) were positively correlated with plasma retinol concentrations. At 6 mo, concentrations of plasma transthyretin (r = 0.52, p < 0.05) were positively correlated with retinol-binding protein concentrations. Phenex-1 supports normal mean iron status indices and mean concentrations of trace and ultratrace minerals, retinol, and alpha-tocopherol when fed in adequate amounts.
