Abstract
The aim of the present study was to apply the Check-all-that-apply (CATA) method in an ambulatory context involving subjects with phenylketonuria (PKU) to obtain a sensory description and to find the drivers of liking of low-phenylalanine products (Glycomacropeptide vs. L-amino acids formulas). 86 subjects with PKU (age range: 8–55 years) evaluated 8 samples: 4 L-amino acid formulas and 4 Glycomacropeptide (GMP) formulas, flavored with neutral, chocolate, strawberry and tomato aromas. Participants were asked to indicate which sensory attributes characterized each formulations and to score the overall liking. Significant differences were found regarding liking scores (F = 65.29; p < 0.001). GMP samples flavored with chocolate and strawberry, described as sweets, with a mild and natural taste and odor, were the most appreciated. Overall, GMP formulas obtained higher liking scores compared to L-amino acid formulas. Tomato flavored samples, described as bitter, salty, with artificial color, with strong taste and odor, obtained the lowest scores. In conclusion, CATA questionnaire seems to be a suitable method also in ambulatory context since this approach suggested that different foods and beverages with GMP could be developed to improve dietary treatment compliance of subjects with PKU from school age onwards.
Highlights
Phenylketonuria (PKU; Online Mendelian Inheritance in Man, OMIM 261,600) is an autosomal recessive disorder of phenylalanine (Phe) metabolism [1], primarily due to mutations in Phe hydroxylase (PAH) gene, which facilitates conversion of the essential amino acid Phe to tyrosine.Loss of PAH activity results in increased Phe concentrations in the blood and in toxic concentrations in the brain
All participants were following a low-phenylalanine diet (84% of the participants were following a diet mainly based on L-amino acid formulas, 7% of the participants were not using a medical integration, 8% of the participant were following a diet based on both L-amino acid formulas and GMP, only one participant was using GMP)
(M = 2.15 ± 0.26), which were comparable to each other, obtained the lowest hedonic ratings and were not acceptable since they were below the middle of the scale
Summary
Phenylketonuria (PKU; Online Mendelian Inheritance in Man, OMIM 261,600) is an autosomal recessive disorder of phenylalanine (Phe) metabolism [1], primarily due to mutations in Phe hydroxylase (PAH) gene, which facilitates conversion of the essential amino acid Phe to tyrosine.Loss of PAH activity results in increased Phe concentrations in the blood (hyperphenylalaninaemia, HPA) and in toxic concentrations in the brain. The main goal of treatment for PKU is to maintain the blood Phe within safe limits to prevent mental retardation and ensure normal growth and life with good health through adulthood. The dietary treatment usually begins immediately after confirmation of PKU diagnosis in newborns and should be continued throughout their lifetime in patients with untreated phenylalanine levels more than 600 μmol/L. Compliance with treatment is adequate in infancy and childhood, difficulties in maintaining a PKU diet in adolescent and adulthood are reported [2]. Patients with PKU have to avoid foods rich in protein (e.g., meat, fish and dairy products), the diet consists mainly of low-protein natural foods (vegetables, fruits) and Nutrients 2018, 10, 1179; doi:10.3390/nu10091179 www.mdpi.com/journal/nutrients
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
