Overweight and obesity in PKU: The results from 8 centres in Europe and Turkey

Özel, Hülya Gökmen; Ahring, Kirsten; Bélanger-Quintana, Amaya; Dokoupil, Katharina; Lammardo, A. M.; Robert, M.; Rocha, Júlio César; Almeida, Manuela Ferreira de; Rijn, M. van; MacDonald, Anita

doi:10.1016/j.ymgmr.2014.11.003

Cited by 30 publications

(35 citation statements)

References 9 publications

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“…Furthermore, the BMI of parents was not recorded and it is acknowledged that this may have influenced eating habits. However, previous study in this group of PKU children has indicated that the incidence of obesity is no different than in the general population [8] .…”

Section: Discussionmentioning

confidence: 53%

“…It is well established that low Phe diets are high in carbohydrate (59–67% of energy) but lower in fat (23–26%) [1] , [2] , [3] , [4] , [5] than the general populations diet (50–52% carbohydrate and 33% fat) [6] . Eating behaviours evolve in the early years of development, and in PKU, a high carbohydrate diet may influence taste preferences and eating behaviour, potentially influencing the development of obesity in later life [1] , [7] , [8] , [9] . Parents help mould children's early experiences with food, providing both the genes and the environment for children [10] .…”

Section: Introductionmentioning

confidence: 99%

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The influence of parental food preference and neophobia on children with phenylketonuria (PKU)

Evans

Daly

Chahal

et al. 2018

Molecular Genetics and Metabolism Reports

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BackgroundIn a previous case-control study, we demonstrated that children with PKU and non-PKU controls preferred sweet foods. Additionally, children with PKU exhibited food neophobia, with no preference for bitter tasting foods associated with the taste of phenylalanine (Phe)-free L-amino acid supplements.ObjectiveIn an observational extension study, we evaluated the influence of parental food choice and neophobia on their children's taste preferences and food neophobia.MethodsMale and female parents/caregivers of 35 children with PKU and 35 control parents, completed a neophobia and food frequency questionnaire for comparison using the same questionnaires that they completed for their children.ResultsBoth groups of children (PKU and non PKU control) were rated as more food neophobic and exhibited more neophobic behaviour than parents, although children with PKU more so than non-PKU controls (PKU food neophobia p < 0.0001vs control 0.001; PKU general neophobia p = 0.003 vs control p = 0.04). Both groups of children ate significantly more sweets, sweetened drinks and potato fries than their parents but differences were greater for children with PKU who also consumed more high carbohydrate (low protein) staple foods such as bread and pasta, and more sweet snacks such as biscuits than their parents. Non-PKU control children's food choices were closer to their parent's choices.ConclusionsIn PKU, parental food choices and their food neophobia have limited influence on their children's eating habits. Food neophobia in children with PKU may be associated with fear of eating unfamiliar foods potentially containing a source of protein or aspartame. Their preference for sweet foods may be influenced by limited food choices and habitual consumption of artificially sweetened L-amino acid supplements.

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Section: Discussionmentioning

confidence: 53%

Section: Introductionmentioning

confidence: 99%

The influence of parental food preference and neophobia on children with phenylketonuria (PKU)

Evans

Daly

Chahal

et al. 2018

Molecular Genetics and Metabolism Reports

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“…Some studies consider that individuals with PKU are more vulnerable to weight increase, 11,30,31 especially females. 25.27,32 According to data published by Gokmen Ozel et al, 32 the prevalence of being overweight and obese in the general female population can range from 17 to 35%.…”

Section: Discussionmentioning

confidence: 99%

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

Sena

Andrade

Silva

et al. 2020

Rev. paul. pediatr.

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Objective: To verify the occurrence of overweight in children and adolescents with phenylketonuria and to identify possible causal factors.Data sources: A systematic review was performed in the SciELO, PubMed and VHL databases using the descriptors "Phenylketonurias", "Overweight", "Child" and "Adolescent". Original articles conducted with children and adolescents, published between 2008 and 2018 in Portuguese, English or Spanish languages were included.Data synthesis: A total of 16 articles were identified and, after screening procedures, 6 studies were selected for the review.Overweight in children and adolescents with phenylketonuria was a frequent occurence in the studies included in this review, ranging from 7.8 to 32.6%. The female sex was the most affected by the nutritional disorder. Furthermore, a high caloric intake combined with a lack of stimuli to practice physical activities were main factors associated with the excessive weight in the population of interest.Conclusions: Excess weight can be considered a common outcome in children and adolescents with phenylketonuria. It is mainly caused by inadequate food consumption and sedentary lifestyle.The importance of early identification of nutritional disturbances in children and adolescents with phenylketonuria should be emphasized, in order to prevent associated chronic diseases and to promote health by encouraging continued healthy eating habits and the regular practice of physical exercises.Objetivo: Verificar a ocorrência de excesso de peso em crianças e adolescentes com fenilcetonúria e identificar possíveis fatores causais. Fontes de dados: Revisão sistemática realizada nas bases de dados Scientific Eletronic Library Online (SciELO), Publisher Medline (PubMed) e Biblioteca Virtual em Saúde (BVS) com os descritores "Phenylketonurias", "Overweight", "Child" e "Adolescent". Foram incluídos artigos originais sobre crianças e adolescentes, publicados entre 2008 e 2018 nos idiomas português, inglês ou espanhol. Síntese dos dados: Foram identificados 16 artigos e, após aplicação dos procedimentos de seleção, 6 estudos foram selecionados para compor a revisão. O excesso de peso em crianças e adolescentes com fenilcetonúria foi evento frequente nos estudos incluídos na presente revisão, variando de 7,8 a 32,6%. Aponta-se o sexo feminino como o grupo mais acometido pelo distúrbio nutricional.O principal fator associado ao excesso de peso na população de interesse na população de interesse foi o consumo calórico elevado aliado à falta de estímulos para a prática de atividades físicas.Conclusões: O excesso de peso pode ser considerado um desfecho comum em crianças e adolescentes com fenilcetonúria, sendo ocasionado principalmente pelo consumo alimentar inadequado e pelo sedentarismo. Salienta-se a importância da identificação precoce de agravos nutricionais em crianças e adolescentes fenilcetonúricos, a fim de prevenir doenças crônicas associadas e promover a saúde, com incentivo à manutenção de hábitos alimentares saudáveis e à prática regular de exercícios fí...

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“…Many adults with PKU have a vegan-like diet but may not take Phe-free L-amino acid supplements [ 74 ] and consequently may be at risk of micronutrient deficiencies [ 75 ]. There is increasing reports of females (and not males) with PKU being overweight and obese [ 76 , 77 ]. The risk of comorbidities makes dietary management more complex [ 78 ].…”

Section: Initiation Of Treatment and Treatment For Lifementioning

confidence: 99%

The complete European guidelines on phenylketonuria: diagnosis and treatment

Wegberg

MacDonald

Ahring

et al. 2017

Orphanet J Rare Dis

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557

885

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Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine. If left untreated, PKU results in increased phenylalanine concentrations in blood and brain, which cause severe intellectual disability, epilepsy and behavioural problems. PKU management differs widely across Europe and therefore these guidelines have been developed aiming to optimize and standardize PKU care. Professionals from 10 different European countries developed the guidelines according to the AGREE (Appraisal of Guidelines for Research and Evaluation) method. Literature search, critical appraisal and evidence grading were conducted according to the SIGN (Scottish Intercollegiate Guidelines Network) method. The Delphi-method was used when there was no or little evidence available. External consultants reviewed the guidelines. Using these methods 70 statements were formulated based on the highest quality evidence available. The level of evidence of most recommendations is C or D. Although study designs and patient numbers are sub-optimal, many statements are convincing, important and relevant. In addition, knowledge gaps are identified which require further research in order to direct better care for the future.

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Overweight and obesity in PKU: The results from 8 centres in Europe and Turkey

Cited by 30 publications

References 9 publications

The influence of parental food preference and neophobia on children with phenylketonuria (PKU)

The influence of parental food preference and neophobia on children with phenylketonuria (PKU)

Overweight and Associated Factors in Children and Adolescents With Phenylketonuria: A Systematic Review

The complete European guidelines on phenylketonuria: diagnosis and treatment

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