title: Targeting specific nutrient deficiencies in protein-restricted diets: some practical facts in PKU dietary management.
abstract: Among aminoacidopathies, phenylketonuria (PKU) is the most prevalent one. Early diagnosis in the neonatal period with a prompt nutritional therapy (low natural-protein and phenylalanine diet, supplemented with phenylalanine-free amino acid mixtures and special low-protein foods) remains the mainstay of the treatment. Data considering nutrient contents of cooked dishes is lacking. In this study, fourteen dishes specifically prepared for PKU individuals were analysed, regarding the lipid profile and iron and zinc contents. These dishes are poor sources of essential nutrients like Fe, Zn or n-3 fatty acids, reinforcing the need for adequate supplementation to cover individual patients' needs. This study can contribute to a more accurate adjustment of PKU diets and supplementation in order to prevent eventual nutritional deficiencies. This study contributes to a better understanding of nutrient intake from PKU patients' meals, showing the need for dietary supplementation.
authors.affiliation: REQUIMTE/Departamento de Ciências Químicas, Faculdade de Farmácia da Universidade do Porto, Rua de Jorge Viterbo Ferreira no. 228, 4050-313 Porto, Portugal.
authors.forename: Filipa B
authors.initials: FB
authors.lastname: Pimentel
authors.forename: Rita C
authors.initials: RC
authors.lastname: Alves
authors.forename: M Teresa
authors.initials: MT
authors.lastname: Oliva-Teles
authors.forename: Anabela S G
authors.initials: AS
authors.lastname: Costa
authors.forename: Telmo J R
authors.initials: TJ
authors.lastname: Fernandes
authors.forename: Manuela F
authors.initials: MF
authors.lastname: Almeida
authors.forename: Duarte
authors.initials: D
authors.lastname: Torres
authors.forename: Cristina
authors.initials: C
authors.lastname: Delerue-Matos
authors.forename: M Beatriz P P
authors.initials: MB
authors.lastname: Oliveira
mesh_terms: D003296:Cooking; D018753:Diet, Protein-Restricted / Q000592:standards*; D004044:Dietary Proteins / Q000032:analysis* / Q000378:metabolism / Q000627:therapeutic use; D019587:Dietary Supplements / Q000032:analysis*; D006801:Humans; D015596:Nutrition Assessment; D010649:Phenylalanine / Q000032:analysis; D010661:Phenylketonurias / Q000178:diet therapy* / Q000378:metabolism
chemical_list: D004044:Dietary Proteins; D010649:Phenylalanine