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Teslime Özge Şahin

1 Article

Review Article

Nutrition

Protein substitutions as new-generation pharmanutrition approach to managing phenylketonuria

Fatma Nur Keskin, Teslime Özge Şahin, Raffaele Capasso, Duygu Ağagündüz

Clin Exp Pediatr. 2023;66(8):320-331. Published online November 1, 2022

Web of Science 1 Crossref 2

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.

DOI: https://doi.org/10.3345/cep.2022.00584

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