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Volume 13
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Systematic Review

Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis

by
Catarina Rodrigues
1,2,
Alex Pinto
3,
Ana Faria
1,2,4,
Diana Teixeira
1,2,4,
Annemiek M. J. van Wegberg
5,
Kirsten Ahring
6,
François Feillet
7,
Conceição Calhau
1,4,
Anita MacDonald
3,
André Moreira-Rosário
1,4,*,† and
Júlio César Rocha
1,4,8,*,†
1
Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal
2
Comprehensive Health Research Centre, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal
3
Dietetic Department, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
4
CINTESIS—Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal
5
Division of Metabolic Diseases, Beatrix Children’s Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands
6
Department of PKU, Copenhagen University Hospital, DK-2600 Glostrup, Denmark
7
Department of Paediatrics, Reference Center for Inborn Errors of Metabolism, Hôpital d’Enfants Brabois, CHU Nancy, 54500 Vandoeuvre les Nancy, France
8
Reference Centre of Inherited Metabolic Diseases, Centro Hospitalar Universitário de Lisboa Central, 1169-045 Lisboa, Portugal
*
Authors to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Nutrients 2021, 13(10), 3443; https://doi.org/10.3390/nu13103443
Submission received: 27 July 2021 / Revised: 16 September 2021 / Accepted: 22 September 2021 / Published: 28 September 2021
(This article belongs to the Special Issue Diet Therapy and Nutritional Management of Phenylketonuria)
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Abstract

Although there is a general assumption that a phenylalanine (Phe)-restricted diet promotes overweight in patients with phenylketonuria (PKU), it is unclear if this presumption is supported by scientific evidence. This systematic review aimed to determine if patients with PKU are at a higher risk of overweight compared to healthy individuals. A literature search was carried out on PubMed, Cochrane Library, and Embase databases. Risk of bias of individual studies was assessed using the Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies, and the quality of the evidence for each outcome was assessed using the NutriGrade scoring system. From 829 articles identified, 15 were included in the systematic review and 12 in the meta-analysis. Body mass index (BMI) was similar between patients with PKU and healthy controls, providing no evidence to support the idea that a Phe-restricted diet is a risk factor for the development of overweight. However, a subgroup of patients with classical PKU had a significantly higher BMI than healthy controls. Given the increasing prevalence of overweight in the general population, patients with PKU require lifelong follow-up, receiving personalised nutritional counselling, with methodical nutritional status monitoring from a multidisciplinary team in inherited metabolic disorders.
Keywords: body mass index; obesity; overweight; phenylalanine restriction; phenylalanine-restricted diet; phenylketonuria body mass index; obesity; overweight; phenylalanine restriction; phenylalanine-restricted diet; phenylketonuria

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MDPI and ACS Style

Rodrigues, C.; Pinto, A.; Faria, A.; Teixeira, D.; van Wegberg, A.M.J.; Ahring, K.; Feillet, F.; Calhau, C.; MacDonald, A.; Moreira-Rosário, A.; et al. Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis. Nutrients 2021, 13, 3443. https://doi.org/10.3390/nu13103443

AMA Style

Rodrigues C, Pinto A, Faria A, Teixeira D, van Wegberg AMJ, Ahring K, Feillet F, Calhau C, MacDonald A, Moreira-Rosário A, et al. Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis. Nutrients. 2021; 13(10):3443. https://doi.org/10.3390/nu13103443

Chicago/Turabian Style

Rodrigues, Catarina, Alex Pinto, Ana Faria, Diana Teixeira, Annemiek M. J. van Wegberg, Kirsten Ahring, François Feillet, Conceição Calhau, Anita MacDonald, André Moreira-Rosário, and et al. 2021. "Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis" Nutrients 13, no. 10: 3443. https://doi.org/10.3390/nu13103443

APA Style

Rodrigues, C., Pinto, A., Faria, A., Teixeira, D., van Wegberg, A. M. J., Ahring, K., Feillet, F., Calhau, C., MacDonald, A., Moreira-Rosário, A., & Rocha, J. C. (2021). Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis. Nutrients, 13(10), 3443. https://doi.org/10.3390/nu13103443

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