TY - JOUR
T1 - Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis
AU - Rodrigues, Catarina
AU - Pinto, Alex
AU - Faria, Ana
AU - Teixeira, Diana
AU - van Wegberg, Annemiek M.J.
AU - Ahring, Kirsten
AU - Feillet, François
AU - Calhau, Conceição
AU - MacDonald, Anita
AU - Moreira-Rosário, André
AU - Rocha, Júlio César
N1 - Funding: CINTESIS—UIDB/4255/2020 a program financially supported by Fundação para a Ciência e Tecnologia/Ministério da Educação e Ciência, through national funds is acknowledged. Support from Human Nutrition and Metabolism Master Program from NOVA Medical School, Universidade Nova de Lisboa is ackowledged.
PY - 2021/9/28
Y1 - 2021/9/28
N2 - 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.
AB - 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.
KW - body mass index
KW - obesity
KW - overweight
KW - phenylalanine restriction
KW - phenylalanine-restricted diet
KW - phenylketonuria
UR - http://www.scopus.com/inward/record.url?scp=85120720507&partnerID=8YFLogxK
U2 - 10.3390/nu13103443
DO - 10.3390/nu13103443
M3 - Review article
C2 - 34684443
AN - SCOPUS:85120720507
SN - 1422-8599
VL - 13
JO - Nutrients
JF - Nutrients
IS - 10
ER -