E Weetch1, A Macdonald. 1. National Society for Phenylketonuria, Hoyland, Nr Barnsley, UK.
Abstract
BACKGROUND: A low phenylalanine diet for people with phenylketonuria (PKU) is very restrictive. Natural foods allowed are based chiefly on fruits and vegetables but there is limited analysis on the phenylalanine content of newer and exotic fruit and vegetables. The assumption that 1 g protein yields 50 mg phenylalanine does not apply to fruits and vegetables. METHODS: The National Society for Phenylketonuria Medical Advisory Panel commissioned the phenylalanine analysis of 172 foods, which emphasis on fruit and vegetables. The foods were purchased from retail outlets and analysed by LGC Limited (67 foods) and the LFI (105 foods). RESULTS: There was a strong correlation between this phenylalanine and UK analyses published in 1980, but previous analysis included only a limited range of fruits and vegetables (r = 0.8746; P < 0.0001). There was also correlation between phenylalanine and UK protein analyses (r = 0.7976; P < 0.0001). CONCLUSION: This new phenylalanine data have enabled a wider range of fruits and vegetables to be safely incorporated into the diet of people with PKU.
BACKGROUND: A low phenylalanine diet for people with phenylketonuria (PKU) is very restrictive. Natural foods allowed are based chiefly on fruits and vegetables but there is limited analysis on the phenylalanine content of newer and exotic fruit and vegetables. The assumption that 1 g protein yields 50 mg phenylalanine does not apply to fruits and vegetables. METHODS: The National Society for Phenylketonuria Medical Advisory Panel commissioned the phenylalanine analysis of 172 foods, which emphasis on fruit and vegetables. The foods were purchased from retail outlets and analysed by LGC Limited (67 foods) and the LFI (105 foods). RESULTS: There was a strong correlation between this phenylalanine and UK analyses published in 1980, but previous analysis included only a limited range of fruits and vegetables (r = 0.8746; P < 0.0001). There was also correlation between phenylalanine and UK protein analyses (r = 0.7976; P < 0.0001). CONCLUSION: This new phenylalanine data have enabled a wider range of fruits and vegetables to be safely incorporated into the diet of people with PKU.
Authors: A M J van Wegberg; A MacDonald; K Ahring; A Bélanger-Quintana; N Blau; A M Bosch; A Burlina; J Campistol; F Feillet; M Giżewska; S C Huijbregts; S Kearney; V Leuzzi; F Maillot; A C Muntau; M van Rijn; F Trefz; J H Walter; F J van Spronsen Journal: Orphanet J Rare Dis Date: 2017-10-12 Impact factor: 4.123
Authors: A MacDonald; A M J van Wegberg; K Ahring; S Beblo; A Bélanger-Quintana; A Burlina; J Campistol; T Coşkun; F Feillet; M Giżewska; S C Huijbregts; V Leuzzi; F Maillot; A C Muntau; J C Rocha; C Romani; F Trefz; F J van Spronsen Journal: Orphanet J Rare Dis Date: 2020-06-30 Impact factor: 4.123
Authors: A Pinto; S Adams; K Ahring; H Allen; M F Almeida; D Garcia-Arenas; N Arslan; M Assoun; Y Atik Altınok; D Barrio-Carreras; A Belanger Quintana; S M Bernabei; C Bontemps; F Boyle; G Bruni; M Bueno-Delgado; G Caine; R Carvalho; A Chrobot; K Chyż; B Cochrane; C Correia; K Corthouts; A Daly; S De Leo; A Desloovere; A De Meyer; A De Theux; B Didycz; M E Dijsselhof; K Dokoupil; J Drabik; C Dunlop; W Eberle-Pelloth; K Eftring; J Ekengren; I Errekalde; S Evans; A Foucart; L Fokkema; L François; M French; E Forssell; C Gingell; C Gonçalves; H Gökmen Özel; A Grimsley; G Gugelmo; E Gyüre; C Heller; R Hensler; I Jardim; C Joost; M Jörg-Streller; C Jouault; A Jung; M Kanthe; N Koç; I L Kok; T Kozanoğlu; B Kumru; F Lang; K Lang; I Liegeois; A Liguori; R Lilje; O Ļubina; P Manta-Vogli; D Mayr; C Meneses; C Newby; U Meyer; S Mexia; C Nicol; U Och; S M Olivas; C Pedrón-Giner; R Pereira; K Plutowska-Hoffmann; J Purves; A Re Dionigi; K Reinson; M Robert; L Robertson; J C Rocha; C Rohde; S Rosenbaum-Fabian; A Rossi; M Ruiz; J Saligova; A Gutiérrez-Sánchez; A Schlune; K Schulpis; J Serrano-Nieto; A Skarpalezou; R Skeath; A Slabbert; K Straczek; M Giżewska; A Terry; R Thom; A Tooke; J Tuokkola; E van Dam; T A M van den Hurk; E M C van der Ploeg; K Vande Kerckhove; M Van Driessche; A M J van Wegberg; K van Wyk; C Vasconcelos; V Velez García; J Wildgoose; T Winkler; J Żółkowska; J Zuvadelli; A MacDonald Journal: Mol Genet Metab Rep Date: 2018-11-25