Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial

• Published in: The American journal of clinical nutrition Vol. 104; no. 2; pp. 334 - 345
• Main Authors: Ney, Denise M, Stroup, Bridget M, Clayton, Murray K, Murali, Sangita G, Rice, Gregory M, Rohr, Frances, Levy, Harvey L
• Format: Journal Article
• Language: English
• Published: United States American Society for Clinical Nutrition, Inc 01.08.2016; American Society for Nutrition
• Subjects: Adolescent; Adult; adverse effects; amino acid composition; Amino acids; analysis of covariance; Analysis of Variance; blood; Caseins - chemistry; Caseins - therapeutic use; Clinical trials; cognitive disorders; Cross-Over Studies; Dietary Proteins - chemistry; Dietary Proteins - therapeutic use; Energy and Protein Metabolism; Feeding Behavior; Female; food intake; food records; Foods, Specialized; Gastrointestinal Diseases - etiology; Gastrointestinal Diseases - prevention & control; gastrointestinal system; Humans; Hunger; inventories; Male; medical foods; Metabolism; Middle Aged; Nutrition; Patient Satisfaction; Peptide Fragments - chemistry; Peptide Fragments - therapeutic use; Peptides; phenylalanine; Phenylalanine - administration & dosage; Phenylalanine - blood; phenylketonuria; Phenylketonurias - blood; Phenylketonurias - diet therapy; proteins; Young Adult; medical food; inborn errors of amino acid metabolism; phenylalanine; threonine; tyrosine; executive function; sapropterin dihydrochloride

To prevent cognitive impairment, phenylketonuria requires lifelong management of blood phenylalanine (Phe) concentration with a low-Phe diet. The diet restricts intake of Phe from natural proteins in combination with traditional amino acid medical foods (AA-MFs) or glycomacropeptide medical foods (G...