Weight Management in Phenylketonuria: What Should Be Monitored?

Rocha, Júlio César; Rijn, M. van; Dam, Esther van; Ahring, Kirsten; Bélanger-Quintana, Amaya; Dokoupil, Katharina; Özel, Hülya Gökmen; Lammardo, A. M.; Robert, M.; Heidenborg, C.; MacDonald, Anita

doi:10.1159/000442304

Cited by 31 publications

(28 citation statements)

References 30 publications

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“…In the PKU group, the fat mass percentage was higher than in the control group without significant difference. Additional evidence supports our finding that PKU does not interfere with growth development nor contribute to obesity [30][31][32][33][34] . In Taiwan, patients with PKU are advised to have plasma Phe levels less than 360 μM before age 3 years, with slight increases up to 600 μM if it is not feasible to keep below 360 μM.…”

Section: Discussionsupporting

confidence: 89%

Dietary intake and nutritional status of patients with phenylketonuria in Taiwan

Weng

Yang

Chen

et al. 2020

Sci Rep

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Phenylalanine hydroxylase (PAH) deficiency leads to phenylalanine accumulation and results in phenylketonuria (PKU). Phenylketonuria can contribute to severe inability such as mental impairment. Early diagnosis and dietary intervention can have beneficial effects on maintaining normal neural and cognitive function in patients with PKU. However, a long-term low phenylalanine diet may put children at risk of malnutrition. A food supplement was therefore used for children with PKU under dietician supervision according to dietary reference intakes (DRIs). In this cross-sectional study, we enrolled patients with PKU and age-matched controls to compare their anthropometry data [weight, height, body mass index (BMI), and body composition using bioelectrical impedance analysis (BIA)], and correlated it with their dietary intake based on 24-h dietary recall. For continuous parameters, the data were expressed as median ± standard deviation (SD), and the Mann-Whitney U test was used to test the difference among the groups. Correlation by natural proteins, body fat, and fat-free mass were evaluated using the Pearson correlation coefficient. Twenty-two participants diagnosed with PKU (ages 8-27 years; mean 15.23 ± 5.23) and a control group of 22 non-PKU participants (ages 8-39 years; mean 19.73 ± 10.6) were recruited for this study. Between the two groups of participants, no significant difference was found in height, weight, BMI, muscle mass, or fat mass. The percentage of natural protein has no effect on body composition. We found a significant positive correlation between the total protein intake percentage of DRIs and muscle mass (r = 0.491, p = 0.020) and a significant negative correlation in the total protein intake percentage of DRIs and fat mass (r =-0.475, p = 0.025) in participants with PKU. There were no significant differences in body composition and nutrition intake between patients with PKU (under metabolic control) and healthy subjects. Thus, giving proper nutrition treatment may have beneficial effects on body growth and nutrition status in patients with pKU in taiwan.

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Section: Discussionsupporting

confidence: 89%

Dietary intake and nutritional status of patients with phenylketonuria in Taiwan

Weng

Yang

Chen

et al. 2020

Sci Rep

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“…Over 30% of SLPF subgroups contained more salt than regular foods, with some containing 100-1050% extra. It is possible that their habitual consumption may contribute to nutritional co-morbidities such as hypertension [32][33][34], vascular stiffness [34,35], overweight/obesity [3,34,[36][37][38][39][40] and an atherogenic lipoprotein profile [34].…”

Section: Discussionmentioning

confidence: 99%

Special Low Protein Foods in the UK: An Examination of Their Macronutrient Composition in Comparison to Regular Foods

et al. 2020

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Special low protein foods (SLPFs) are essential in a low phenylalanine diet for treating phenylketonuria (PKU). With little known about their nutritional composition, all SLPFs on UK prescription were studied (n = 146) and compared to equivalent protein-containing foods (n = 190). SLPF nutritional analysis was obtained from suppliers/manufacturers. Comparable information about regular protein-containing foods was obtained from online UK supermarkets. Similar foods were grouped together, with mean nutritional values calculated for each subgroup (n = 40) and percentage differences determined between SLPFs and regular food subgroups. All SLPF subgroups contained 43–100% less protein than regular foods. Sixty-three percent (n = 25/40) of SLPF subgroups contained less total fat with palm oil (25%, n = 36/146) and hydrogenated vegetable oil (23%, n = 33/146) key fat sources. Sixty-eight percent (n = 27/40) of SLPF subgroups contained more carbohydrate, with 72% (n = 105/146) containing added sugar. Key SLPF starch sources were maize/corn (72%; n = 105/146). Seventy-seven percent (n = 113/146) of SLPFs versus 18% (n = 34/190) of regular foods contained added fibre, predominantly hydrocolloids. Nine percent of SLPFs contained phenylalanine > 25 mg/100 g and sources of phenylalanine/protein in their ingredient lists. Stricter nutritional composition regulations for SLPFs are required, identifying maximum upper limits for macronutrients and phenylalanine, and fat and carbohydrate sources that are associated with healthy outcomes.

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“…Thus, comprehensive nutritional status as well as possible causal factors for endocrinometabolic changes need to be monitored. In their review work, Rocha et al 50 proposed the adoption of standardized procedures for PKU weight control assessments to consider dietary, lifestyle, anthropometric and body composition aspects as well as biochemical markers. Such protocols are useful for standardizing the collection of important screening data, as well as for applying early measures to prevent excessive weight gain in the population with PKU.…”

Section: Indicators Analyzedmentioning

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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Weight Management in Phenylketonuria: What Should Be Monitored?

Cited by 31 publications

References 30 publications

Dietary intake and nutritional status of patients with phenylketonuria in Taiwan

Dietary intake and nutritional status of patients with phenylketonuria in Taiwan

Special Low Protein Foods in the UK: An Examination of Their Macronutrient Composition in Comparison to Regular Foods

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

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