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


Journal

Nutrients
ISSN: 2072-6643
Titre abrégé: Nutrients
Pays: Switzerland
ID NLM: 101521595

Informations de publication

Date de publication:
28 Sep 2021
Historique:
received: 27 07 2021
revised: 16 09 2021
accepted: 22 09 2021
entrez: 23 10 2021
pubmed: 24 10 2021
medline: 30 11 2021
Statut: epublish

Résumé

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.

Identifiants

pubmed: 34684443
pii: nu13103443
doi: 10.3390/nu13103443
pmc: PMC8538431
pii:
doi:

Substances chimiques

Biomarkers 0
Phenylalanine 47E5O17Y3R

Types de publication

Journal Article Meta-Analysis Systematic Review

Langues

eng

Sous-ensembles de citation

IM

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Auteurs

Catarina Rodrigues (C)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
Comprehensive Health Research Centre, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.

Alex Pinto (A)

Dietetic Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

Ana Faria (A)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
Comprehensive Health Research Centre, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
CINTESIS-Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.

Diana Teixeira (D)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
Comprehensive Health Research Centre, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
CINTESIS-Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.

Annemiek M J van Wegberg (AMJ)

Division of Metabolic Diseases, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands.

Kirsten Ahring (K)

Department of PKU, Copenhagen University Hospital, DK-2600 Glostrup, Denmark.

François Feillet (F)

Department of Paediatrics, Reference Center for Inborn Errors of Metabolism, Hôpital d'Enfants Brabois, CHU Nancy, 54500 Vandoeuvre les Nancy, France.

Conceição Calhau (C)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
CINTESIS-Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.

Anita MacDonald (A)

Dietetic Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.

André Moreira-Rosário (A)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
CINTESIS-Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.

Júlio César Rocha (JC)

Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
CINTESIS-Center for Health Technology and Services Research, NOVA Medical School, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.
Reference Centre of Inherited Metabolic Diseases, Centro Hospitalar Universitário de Lisboa Central, 1169-045 Lisboa, Portugal.

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