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Author(s): Filipa B Pimentel
Rita C Alves
Anabela S G Costa
Duarte Torres
Manuela F Almeida
Beatriz B P P Oliveira
Title: Phenylketonuria: Protein content and amino acids profile of dishes for phenylketonuric patients. The relevance of phenylalanine
Issue Date: 2014
Abstract: Phenylketonuria is an inborn error of metabolism, involving, in most cases, a deficient activity of phenylalanine hydroxylase. Neonatal diagnosis and a prompt special diet (low phenylalanine and natural-protein restricted diets) are essential to the treatment. The lack of data concerning phenylalanine contents of processed foodstuffs is an additional limitation for an already very restrictive diet. Our goals were to quantify protein (Kjeldahl method) and amino acid (18) content (HPLC/fluorescence) in 16 dishes specifically conceived for phenylketonuric patients, and compare the most relevant results with those of several international food composition databases. As might be expected, all the meals contained low protein levels (0.67-3.15 g/100 g) with the highest ones occurring in boiled rice and potatoes. These foods also contained the highest amounts of phenylalanine (158.51 and 62.65 mg/100 g, respectively). In contrast to the other amino acids, it was possible to predict phenylalanine content based on protein alone. Slight deviations were observed when comparing results with the different food composition databases.
Subject: Ciências da Saúde, Outras ciências agrárias
Health sciences, Other Agrarian Sciences
Scientific areas: Ciências agrárias::Outras ciências agrárias
Agrarian Sciences::Other Agrarian Sciences
Document Type: Artigo em Revista Científica Internacional
Rights: restrictedAccess
Appears in Collections:FCNAUP - Artigo em Revista Científica Internacional
FFUP - Artigo em Revista Científica Internacional

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