Literature DB >> 12971420

Free use of fruits and vegetables in phenylketonuria.

A MacDonald1, G Rylance, P Davies, D Asplin, S K Hall, I W Booth.   

Abstract

This study aimed to evaluate systematically the effect of the free use of fruits and vegetables containing an intermediate amount of phenylalanine (51-100 mg/100 g) on the biochemical control in children with phenylketonuria (PKU). Fifteen subjects with PKU, with a median age of 6 years (range 1-24 years) were studied. In a three-part prospective 15-week study, subjects sequentially ate fruits and vegetables containing phenylalanine 0-50 mg/100 g for weeks 1 to 3; 51-75 mg/100 g for weeks 4 to 8; and 76-100 mg/100 g for weeks 9 to 15. Plasma phenylalanine concentrations were measured twice daily for three consecutive days in weeks 1, 3, 6, 8, 11, 13 and 15. A standard menu was followed on the blood sampling days. Daily dietary records of fruits and vegetables under study were kept throughout the trial. Control of phenylalanine concentrations was not adversely affected by the free use of fruits and vegetables containing 51-100 mg/100 g. Pre-breakfast median plasma concentrations were: weeks 1 to 3, 260 micromol/L (range 90-890); weeks 4 to 8, 255 micromol/L (range 130-920); and weeks 9 to 15, 278 micromol/L (range 30-880). Pre-evening meal median plasma phenylalanine concentrations were: weeks 1 to 3, 240 micromol/L (range 30-820); weeks 4 to 8, 210 micromol/L (40-880); and weeks 9 to 15, 238 micromol/L (range 20-880). These data suggest that free use of fruits and vegetables containing 51-75 mg/100 g poses no problem for children with PKU.

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Year:  2003        PMID: 12971420     DOI: 10.1023/a:1025150901439

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.750


  4 in total

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Authors:  A MacDonald; G Rylance; S K Hall; D Asplin; I W Booth
Journal:  Arch Dis Child       Date:  1996-05       Impact factor: 3.791

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Authors:  N D Atherton; A Green
Journal:  Clin Chem       Date:  1988-11       Impact factor: 8.327

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Journal:  Arch Dis Child       Date:  1993-03       Impact factor: 3.791

  4 in total
  14 in total

1.  Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH(4)).

Authors:  A G Thiele; J F Weigel; B Ziesch; C Rohde; U Mütze; U Ceglarek; J Thiery; A S Müller; W Kiess; S Beblo
Journal:  JIMD Rep       Date:  2012-10-17

2.  Nutritional Consequences of Adhering to a Low Phenylalanine Diet for Late-Treated Adults with PKU : Low Phe Diet for Adults with PKU.

Authors:  Ingrid Wiig; Kristina Motzfeldt; Elin Bjørge Løken; Bengt Frode Kase
Journal:  JIMD Rep       Date:  2012-07-01

3.  Dietary protein counting as an alternative way of maintaining metabolic control in phenylketonuria.

Authors:  A L Sweeney; R M Roberts; J M Fletcher
Journal:  JIMD Rep       Date:  2011-09-16

Review 4.  Food products made with glycomacropeptide, a low-phenylalanine whey protein, provide a new alternative to amino Acid-based medical foods for nutrition management of phenylketonuria.

Authors:  Sandra C van Calcar; Denise M Ney
Journal:  J Acad Nutr Diet       Date:  2012-08       Impact factor: 4.910

Review 5.  Oxidative stress in phenylketonuria: future directions.

Authors:  Júlio César Rocha; Maria João Martins
Journal:  J Inherit Metab Dis       Date:  2011-11-25       Impact factor: 4.982

6.  Unrestricted fruits and vegetables in the PKU diet: a 1-year follow-up.

Authors:  C Rohde; U Mütze; S Schulz; A G Thiele; U Ceglarek; J Thiery; A S Mueller; W Kiess; S Beblo
Journal:  Eur J Clin Nutr       Date:  2014-01-08       Impact factor: 4.016

Review 7.  The complete European guidelines on phenylketonuria: diagnosis and treatment.

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

8.  Table of Phenylalanine Content of Foods: Comparative Analysis of Data Compiled in Food Composition Tables.

Authors:  Ana Claudia Marquim F Araújo; Wilma M C Araújo; Ursula M Lanfer Marquez; Rita Akutsu; Eduardo Y Nakano
Journal:  JIMD Rep       Date:  2016-10-08

9.  Effect of dietary regime on metabolic control in phenylketonuria: Is exact calculation of phenylalanine intake really necessary?

Authors:  Carmen Rohde; Alena Gerlinde Thiele; Ulrike Och; Katrin Schönherr; Uta Meyer; Stefanie Rosenbaum-Fabian; Cornelia Maddalon; Sabine Matzken; Holger Blessing; Frauke Lang; Monika Jörg-Streller; Skadi Beblo
Journal:  Mol Genet Metab Rep       Date:  2015-10-22

Review 10.  Multiclinic Observations on the Simplified Diet in PKU.

Authors:  Laurie Bernstein; Casey Burns; Melissa Sailer-Hammons; Angela Kurtz; Frances Rohr
Journal:  J Nutr Metab       Date:  2017-09-13
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