Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial12
| Autor: | Ney, Denise M, Stroup, Bridget M, Clayton, Murray K, Murali, Sangita G, Rice, Gregory M, Rohr, Frances, Levy, Harvey L |
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| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
musculoskeletal diseases
Adult Male congenital hereditary and neonatal diseases and abnormalities animal structures Adolescent phenylalanine Gastrointestinal Diseases Hunger inborn errors of amino acid metabolism sapropterin dihydrochloride macromolecular substances Young Adult Phenylketonurias medical food Humans skin and connective tissue diseases Energy and Protein Metabolism Foods Specialized Analysis of Variance Cross-Over Studies Caseins Feeding Behavior Middle Aged threonine Peptide Fragments executive function Patient Satisfaction Female Dietary Proteins tyrosine |
| Zdroj: | The American Journal of Clinical Nutrition |
| ISSN: | 1938-3207 0002-9165 |
| Popis: | Background: 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 (GMP-MFs) that contain primarily intact protein and a small amount of Phe. Objective: We investigated the efficacy and safety of a low-Phe diet combined with GMP-MFs or AA-MFs providing the same quantity of protein equivalents in free-living subjects with phenylketonuria. Design: This 2-stage, randomized crossover trial included 30 early-treated phenylketonuria subjects (aged 15–49 y), 20 with classical and 10 with variant phenylketonuria. Subjects consumed, in random order for 3 wk each, their usual low-Phe diet combined with AA-MFs or GMP-MFs. The treatments were separated by a 3-wk washout with AA-MFs. Fasting plasma amino acid profiles, blood Phe concentrations, food records, and neuropsychological tests were obtained. Results: The frequency of medical food intake was higher with GMP-MFs than with AA-MFs. Subjects rated GMP-MFs as more acceptable than AA-MFs and noted improved gastrointestinal symptoms and less hunger with GMP-MFs. ANCOVA indicated no significant mean ± SE increase in plasma Phe (62 ± 40 μmol/L, P = 0.136), despite a significant increase in Phe intake from GMP-MFs (88 ± 6 mg Phe/d, P = 0.026). AA-MFs decreased plasma Phe (−85 ± 40 μmol/L, P = 0.044) with stable Phe intake. Blood concentrations of Phe across time were not significantly different (AA-MFs = 444 ± 34 μmol/L, GMP-MFs = 497 ± 34 μmol/L), suggesting similar Phe control. Results of the Behavior Rating Inventory of Executive Function were not significantly different. Conclusions: GMP-MFs provide a safe and acceptable option for the nutritional management of phenylketonuria. The greater acceptability and fewer side effects noted with GMP-MFs than with AA-MFs may enhance dietary adherence for individuals with phenylketonuria. This trial was registered at www.clinicaltrials.gov as NCT01428258. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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