- Review Article
- Nutrition
- Protein substitutions as new-generation pharmanutrition approach to managing phenylketonuria
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Fatma Nur Keskin, Teslime Özge Şahin, Raffaele Capasso, Duygu Ağagündüz
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Clin Exp Pediatr. 2023;66(8):320-331. Published online November 1, 2022
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Phenylketonuria, an autosomal recessive disease that results from the inability to metabolize phenylalanine, is currently treated with medical nutrition therapy. New treatment approaches such as tetrahydrobiopterin, glycomacropeptide, large neutral amino acids, pegvaliase, and gene therapy significantly impact disease management and dietary enrichment. This article also reviews animal and human studies that have evaluated the efficacy and safety of these new protein substitutes. |
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