Strategies Used in Production of Phenylalanine-Free Foods for PKU Management.

Phenylketonuria (PKU) is one of the most widespread dysfunctions caused by an inheritable problem in the phenylalanine metabolism. In this metabolic disease, gene mutations in phenylalanine hydroxylase (PAH) result in phenylalanine accumulation that causes varying degrees of mental retardation. The most effective treatment is restriction of phenylalanine in diet provided through different strategies including combination of low-protein foods, hydrolyzing of protein-rich foods, or use of protein substitutes. Meat and meat products, sea foods, milk and infant formula, cereal products, and beans are among the modified foods for PKU management. Elimination of phenylalanine from food presents major technological, nutritional, and organolleptic challenges because protein, as an essential structure-building and nutritional element, is removed. In addition, following this program is laborious, boring, and restrictive for both patients and their families. This paper reviews the current findings about PKU and the recent developments in the production of phenylalanine-free foods. Also, the nutritional requirements and challenges encountered by PKU individuals and food technologists are finally discussed.