Overweight and obesity in adult patients with phenylketonuria: a systematic review

Abstract Background Excess weight is a rising concern in patients with phenylketonuria (PKU). It is commonly observed in children and adolescents with PKU; but data on adults are inconsistent. This review aims to summarize available data on excess weight in adult PKU individuals. Methods We conducte...

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Main Authors: Aurel T. Tankeu, Despina Christina Pavlidou, Andrea Superti-Furga, Karim Gariani, Christel Tran
Format: Article
Language:English
Published: BMC 2023-02-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:https://doi.org/10.1186/s13023-023-02636-2
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author Aurel T. Tankeu
Despina Christina Pavlidou
Andrea Superti-Furga
Karim Gariani
Christel Tran
author_facet Aurel T. Tankeu
Despina Christina Pavlidou
Andrea Superti-Furga
Karim Gariani
Christel Tran
author_sort Aurel T. Tankeu
collection DOAJ
description Abstract Background Excess weight is a rising concern in patients with phenylketonuria (PKU). It is commonly observed in children and adolescents with PKU; but data on adults are inconsistent. This review aims to summarize available data on excess weight in adult PKU individuals. Methods We conducted a systematic search of literature in English, from inception to October 2021, on PubMed and Embase to identify articles on overweight and obesity in adult PKU patients. Prevalence of overweight and obesity, body mass index (BMI) and gender differences were the outcomes of interest. Results Of 260 articles identified, only 8 fulfilled quality criteria for inclusion after screening of titles, abstracts and full texts. The mean BMI of adult PKU patients in these studies ranged from 26 ± 5.4 to 30.3 ± 1.8 kg/m2. When compared to matched controls, adult PKU patients had higher BMI and higher prevalence of obesity. However, results were inconsistent when PKU adults were compared to the general population. The prevalence of obesity in the included studies varied widely between 4.5% up to 72% in individual studies. Obesity was 2–3 times more frequent in female PKU patients. Conclusions Excess weight is frequent in adult PKU patients, especially in females, even if the difference with the general population is debatable. The heterogeneity of the studies makes it difficult to interpret the results and the factors that contribute to obesity. Content of the diet, psychological status, diet-associated disordered eating, patient’s social environment and lifestyle are listed as potentials contributors to excess weight in PKU adult population. Further studies are needed to better elucidate this question. In the meantime, weight control and healthy eating habits should be considered in the management and follow-up of these patients.
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spelling doaj.art-332dc4c695084e9ea894cfb94a935e062023-03-22T12:21:44ZengBMCOrphanet Journal of Rare Diseases1750-11722023-02-011811910.1186/s13023-023-02636-2Overweight and obesity in adult patients with phenylketonuria: a systematic reviewAurel T. Tankeu0Despina Christina Pavlidou1Andrea Superti-Furga2Karim Gariani3Christel Tran4Division of Genetic Medicine, University of Lausanne and University Hospital of LausanneDivision of Genetic Medicine, University of Lausanne and University Hospital of LausanneDivision of Genetic Medicine, University of Lausanne and University Hospital of LausanneService of Endocrinology, Diabetes, Nutrition and Therapeutic Patient Education, Geneva University Hospitals and Geneva UniversityDivision of Genetic Medicine, University of Lausanne and University Hospital of LausanneAbstract Background Excess weight is a rising concern in patients with phenylketonuria (PKU). It is commonly observed in children and adolescents with PKU; but data on adults are inconsistent. This review aims to summarize available data on excess weight in adult PKU individuals. Methods We conducted a systematic search of literature in English, from inception to October 2021, on PubMed and Embase to identify articles on overweight and obesity in adult PKU patients. Prevalence of overweight and obesity, body mass index (BMI) and gender differences were the outcomes of interest. Results Of 260 articles identified, only 8 fulfilled quality criteria for inclusion after screening of titles, abstracts and full texts. The mean BMI of adult PKU patients in these studies ranged from 26 ± 5.4 to 30.3 ± 1.8 kg/m2. When compared to matched controls, adult PKU patients had higher BMI and higher prevalence of obesity. However, results were inconsistent when PKU adults were compared to the general population. The prevalence of obesity in the included studies varied widely between 4.5% up to 72% in individual studies. Obesity was 2–3 times more frequent in female PKU patients. Conclusions Excess weight is frequent in adult PKU patients, especially in females, even if the difference with the general population is debatable. The heterogeneity of the studies makes it difficult to interpret the results and the factors that contribute to obesity. Content of the diet, psychological status, diet-associated disordered eating, patient’s social environment and lifestyle are listed as potentials contributors to excess weight in PKU adult population. Further studies are needed to better elucidate this question. In the meantime, weight control and healthy eating habits should be considered in the management and follow-up of these patients.https://doi.org/10.1186/s13023-023-02636-2PhenylketonuriaObesityOverweightAdult
spellingShingle Aurel T. Tankeu
Despina Christina Pavlidou
Andrea Superti-Furga
Karim Gariani
Christel Tran
Overweight and obesity in adult patients with phenylketonuria: a systematic review
Orphanet Journal of Rare Diseases
Phenylketonuria
Obesity
Overweight
Adult
title Overweight and obesity in adult patients with phenylketonuria: a systematic review
title_full Overweight and obesity in adult patients with phenylketonuria: a systematic review
title_fullStr Overweight and obesity in adult patients with phenylketonuria: a systematic review
title_full_unstemmed Overweight and obesity in adult patients with phenylketonuria: a systematic review
title_short Overweight and obesity in adult patients with phenylketonuria: a systematic review
title_sort overweight and obesity in adult patients with phenylketonuria a systematic review
topic Phenylketonuria
Obesity
Overweight
Adult
url https://doi.org/10.1186/s13023-023-02636-2
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