Diet Liberalization of Patients with Plasma Phenylalanine Concentrations in the Therapeutic Sample Clauses

Diet Liberalization of Patients with Plasma Phenylalanine Concentrations in the Therapeutic. Range After one month of BH4 therapy, three patients eating completely liberalized diets had plasma phenylalanine concentrations below 360 μmol/L and were classified as definitive responders. All three patients no longer needed medical food to meet their age- and sex- specific RDA for protein or to maintain plasma phenylalanine concentrations in the therapeutic range. Twenty-three patients initiated the milk powder challenge. Of note are two patients who were assessed despite having month one plasma phenylalanine concentrations exceeding 360 μmol/L (439 and 441 μmol/L). Both patients were eating diets high in phenylalanine. To establish their new dietary tolerance they displaced a portion of their intact protein intake with milk powder and proceeded through the challenge. Of the 23 patients who initiated the milk powder challenge, 15 were classified as definitive BH4 responders while the other 8 were classified as provisional responders.
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