Literature DB >> 24398645

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

C Rohde1, U Mütze1, S Schulz2, A G Thiele1, U Ceglarek3, J Thiery3, A S Mueller4, W Kiess1, S Beblo1.   

Abstract

Phenylketonuria (PKU) therapy demands phenylalanine (Phe) calculation. In most countries, almost all food is taken into account, even fruits and vegetables. We investigated whether unrestricted consumption of fruits and vegetables negatively influences metabolic control. Nineteen PKU children (2-10 years) started with 2 weeks of free or restricted fruit and vegetable intake. After 2 weeks, the regime changed from free to restricted or restricted to free (cross-over design). Over the first 4 weeks, dried blood Phe concentration was measured, fruit and vegetable consumption recorded and nutrient intake calculated from diet records. Thereafter the diet was changed to free use of fruits and vegetables for all patients. Six and 12 months later, diet and Phe concentrations were monitored. Median Phe intake increased significantly by 65 mg/day (week 4, P<0.001), 68 mg/day (month 6, P<0.001) and 70 mg/day (month 12, P<0.001). Dried blood Phe concentrations remained stable (P=0.894), as did the frequency of Phe concentrations above the recommended range (P=0.592). In conclusion, PKU diet liberalization for fruits and vegetables seems unproblematic.

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Year:  2014        PMID: 24398645     DOI: 10.1038/ejcn.2013.272

Source DB:  PubMed          Journal:  Eur J Clin Nutr        ISSN: 0954-3007            Impact factor:   4.016


  7 in total

1.  Unrestricted consumption of fruits and vegetables in phenylketonuria: no major impact on metabolic control.

Authors:  C Rohde; U Mütze; J F W Weigel; U Ceglarek; J Thiery; W Kiess; S Beblo
Journal:  Eur J Clin Nutr       Date:  2012-02-08       Impact factor: 4.016

2.  Positive effect of a simplified diet on blood phenylalanine control in different phenylketonuria variants, characterized by newborn BH4 loading test and PAH analysis.

Authors:  M Zimmermann; P Jacobs; R Fingerhut; T Torresani; B Thöny; N Blau; M R Baumgartner; M Rohrbach
Journal:  Mol Genet Metab       Date:  2012-04-25       Impact factor: 4.797

Review 3.  The influence of dietary fibre on protein digestion and utilization in monogastrics.

Authors:  B O Eggum
Journal:  Arch Tierernahr       Date:  1995

Review 4.  The reality of dietary compliance in the management of phenylketonuria.

Authors:  Anita MacDonald; Hulya Gokmen-Ozel; Margreet van Rijn; Peter Burgard
Journal:  J Inherit Metab Dis       Date:  2010-04-07       Impact factor: 4.982

5.  Validation of the phenylalanine/tyrosine ratio determined by tandem mass spectrometry: sensitive newborn screening for phenylketonuria.

Authors:  Uta Ceglarek; Peter Müller; Barbara Stach; Peter Bührdel; Joachim Thiery; Wieland Kiess
Journal:  Clin Chem Lab Med       Date:  2002-07       Impact factor: 3.694

6.  Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997.

Authors:  P Burgard; H J Bremer; P Bührdel; P C Clemens; E Mönch; H Przyrembel; F K Trefz; K Ullrich
Journal:  Eur J Pediatr       Date:  1999-01       Impact factor: 3.183

7.  Free use of fruits and vegetables in phenylketonuria.

Authors:  A MacDonald; G Rylance; P Davies; D Asplin; S K Hall; I W Booth
Journal:  J Inherit Metab Dis       Date:  2003       Impact factor: 4.750

  7 in total
  8 in total

1.  Carriership of the rs113883650/rs2287120 haplotype of the SLC7A5 (LAT1) gene increases the risk of obesity in infants with phenylketonuria.

Authors:  Miroslaw Bik-Multanowski; Anna Madetko-Talowska; Iwona Betka; Elzbieta Swieczka; Bozena Didycz; Karolina Orchel-Szastak; Kinga Bik-Multanowska; Ewa Starostecka; Joanna Jaglowska; Renata Mozrzymas; Joanna Zolkowska; Katarzyna Chyz; Dorota Korycinska-Chaaban
Journal:  Mol Genet Metab Rep       Date:  2020-08-21

Review 2.  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

3.  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

4.  Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements.

Authors:  Sharon Evans; Sarah Adam; Sandra Adams; Heather Allen; Catherine Ashmore; Sarah Bailey; Janette Banks; Harriet Churchill; Barbara Cochrane; Jennifer Cook; Clare Dale; Anne Daly; Marjorie Dixon; Carolyn Dunlop; Charlotte Ellerton; Anita Emm; Sarah Firman; Suzanne Ford; Moira French; Joanna Gribben; Anne Grimsley; Ide Herlihy; Melanie Hill; Shirley Judd; Karen Lang; Jo Males; Joy McDonald; Nicola McStravick; Chloe Millington; Camille Newby; Catharine Noble; Rachel Pereira; Alex Pinto; Louise Robertson; Abigail Robotham; Kathleen Ross; Kath Singleton; Rachel Skeath; Allyson Terry; Karen Van Wyk; Fiona White; Lucy White; Jo Wildgoose; Alison Woodall; Anita MacDonald
Journal:  Nutrients       Date:  2020-07-24       Impact factor: 5.717

Review 5.  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

Review 6.  Dietary intervention in the management of phenylketonuria: current perspectives.

Authors:  Júlio César Rocha; Anita MacDonald
Journal:  Pediatric Health Med Ther       Date:  2016-12-01

7.  Simplified Diet for nutrition management of phenylketonuria: A survey of U.S. metabolic dietitians.

Authors:  Joyanna Hansen; Suzanne Hollander; Nicoletta Drilias; Sandra Van Calcar; Fran Rohr; Laurie Bernstein
Journal:  JIMD Rep       Date:  2020-04-08

8.  Weaning practices in phenylketonuria vary between health professionals in Europe.

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
  8 in total

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