Title: Nutrient Status and Intakes of Adults with Phenylketonuria
| Published: | August 15, 2024 |
Phenylketonuria (PKU) is an inherited disorder of amino acid metabolism affecting approximately 1 in 24,000 newborns globally. In PKU, phenylalanine (Phe) is not properly converted into tyrosine and accumulates in the blood and brain. If left untreated, this can cause devastating neurocognitive deficits. Currently, there is no cure. For most patients, management consists of a strict, lifelong, low-protein diet, supplemented with special Phe-free or low-Phe protein substitutes to ensure that infants and children with PKU can meet their nutrient requirements for growth and development.
Little has been published on the nutrient status of adults with PKU. New research investigated blood nutrient concentrations, and nutritional intakes, of early and continuously treated adults with PKU, compared with matched adults without PKU. 1 Nutrient status and intakes of adult patients with PKU using a protein substitute were adequate for most nutrients (DHA, micronutrients). However, compared with controls, patients had lower blood concentrations for selenium, ubiquinone, and some amino acids. The regular use of protein substitutes remains a point of attention in adults with PKU, as they are essential to meet recommended intakes.
Find out more: https://doi.org/10.3390/nu16162724