OVERWEIGHT AND ASSOCIATED FACTORS IN CHILDREN AND ADOLESCENTS WITH PHENYLKETONURIA: A SYSTEMATIC REVIEW

Sena, Berilany dos Santos; Andrade, Maria Izabel Siqueira de; Silva, Ana Paula Ferreira da; Dourado, Keila Fernandes; Silva, Andressa Laís Ferreira

doi:10.1590/1984-0462/2020/38/2018201

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Brasil

Revista Paulista de Pediatria

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Brasil

Español English

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Sumário

REVIEW ARTICLE • Rev. paul. pediatr. 38 • 2020 • https://doi.org/10.1590/1984-0462/2020/38/2018201 copy

OVERWEIGHT AND ASSOCIATED FACTORS IN CHILDREN AND ADOLESCENTS WITH PHENYLKETONURIA: A SYSTEMATIC REVIEW

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

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.

Keywords:
Phenylketonurias; Overweight; Child, Adolescent; Review

Reference	Location, year	Design	Population (n)	Population characteristics	Score	%
Aldámiz-Echevarría et al.²⁸28. Aldámiz-Echevarría L, Bueno MA, Couce ML, Lage S, Dalmau J, Vitoria I, et al. Anthropometric characteristics and nutrition in a cohort of PAH-deficient patients. Clin Nutr. 2014;33:702-17. https://doi.org/10.1016/j.clnu.2013.09.011 https://doi.org/https://doi.org/10.1016/...	Spain, 2013	Retrospective cohort	505	Age range from 1 to 36 years old 46.7% M; 53.3% F	17.1	77.7
Bélanger-Quintana and Martínez-Pardo ²⁵25. Bélanger-Quintana A, Martínez-Pardo M. Physical development in patients with phenylketonuria on dietary treatment: a retrospective study. Mol Genet Metab. 2011;104:480-4. https://doi.org/10.1016/j.ymgme.2011.08.002 https://doi.org/https://doi.org/10.1016/...	Spain, 2011	Retrospective cohort	160	Age range from 12 months to 28 years old. 46.9% M; 53.1% F	16.7	75.9
Burrage et al.²⁷27. Burrage RC, McConnell J, Haesler R, O’Riordan MA, Sutton VR, Kerr DS, et al. High prevalence of overweight and obesity in females with phenylketonuria. Mol Genet Metab. 2012;107:43-8. https://doi.org/10.1016/j.ymgme.2012.07.006 https://doi.org/https://doi.org/10.1016/...	United States, 2012	Retrospective Study	87	Age range: 2.1 to 20.5 years old 51.7% M; 48.3% F	16.6	75.4
Albersen et al.¹⁰10. Albersen M, Bonthuis M, de Roos NM, van der Hurk DA, Weber EC, Hendriks MM, et al. Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage. J Inherit Metab Dis. 2010; (Suppl 3):S283-8. https://doi.org/10.1007/s10545-010-9149-8 https://doi.org/https://doi.org/10.1007/...	Holland, 2010	Cross-sectional	20	Age range: 6 to years old 35% M; 65% F	15.8	71.8
Doulgeraki et al.²⁹29. Doulgeraki A, Skarpalezou A, Theodosiadou A, Monopolis I, Schulpis K. Body composition profile of young patients with phenylketonuria and mild hyperphenylalaninemia. Int J Endocrinol Metab. 2014;12:160-1. https://doi.org/10.5812/ijem.16061 https://doi.org/https://doi.org/10.5812/...	Greece, 2014	Cross-sectional	80	Age range: 7 to 10 years old 46.3% M; 53.7% F	15.4	70.0
Rocha et al.²⁶26. Rocha JC, van Spronsen FJ, Almeida MF, Soares G, Quelhas D, Ramos E, et al. Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome. Mol Genet Metab. 2012;107:659-63. https://doi.org/10.1016/j.ymgme.2012.10.006 https://doi.org/https://doi.org/10.1016/...	Portugal, 2012	Cross-sectional	89	Age range: 3 to 30 years old 54% M; 46% F	14.6	66.4

Reference	Key Results	Indicators analyzed
Aldámiz-Echevarría et al.²⁸28. Aldámiz-Echevarría L, Bueno MA, Couce ML, Lage S, Dalmau J, Vitoria I, et al. Anthropometric characteristics and nutrition in a cohort of PAH-deficient patients. Clin Nutr. 2014;33:702-17. https://doi.org/10.1016/j.clnu.2013.09.011 https://doi.org/https://doi.org/10.1016/...	Overweight in the PKU group: 9.2% Overweight in the reference population: 17.4% Obesity in the PKU group: 6.5% Obesity in the reference population: 9.8%	Z-scores of weight, height and BMI.
Bélanger-Quintana and Martínez-Pardo²⁵25. Bélanger-Quintana A, Martínez-Pardo M. Physical development in patients with phenylketonuria on dietary treatment: a retrospective study. Mol Genet Metab. 2011;104:480-4. https://doi.org/10.1016/j.ymgme.2011.08.002 https://doi.org/https://doi.org/10.1016/...	Mean Z-scores for height, weight and BMI were approximately zero for all patients with PKU. There were no statistically significant differences in height, weight and BMI Z scores at any age when compared to sex and age reference values.	Z-scores of weight, height and BMI.
Burrage et al.²⁷27. Burrage RC, McConnell J, Haesler R, O’Riordan MA, Sutton VR, Kerr DS, et al. High prevalence of overweight and obesity in females with phenylketonuria. Mol Genet Metab. 2012;107:43-8. https://doi.org/10.1016/j.ymgme.2012.07.006 https://doi.org/https://doi.org/10.1016/...	Overweight in the PKU group: 12% Overweight in the reference population: 14.8% Obesity in the PKU group: 28% Obesity in the reference population: 16.8%	Z-scores of weight, height and BMI.
Albersen et al.¹⁰10. Albersen M, Bonthuis M, de Roos NM, van der Hurk DA, Weber EC, Hendriks MM, et al. Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage. J Inherit Metab Dis. 2010; (Suppl 3):S283-8. https://doi.org/10.1007/s10545-010-9149-8 https://doi.org/https://doi.org/10.1007/...	The mean body fat percentage was significantly higher in patients with PKU compared to healthy controls (25.2 ± 7.3% versus 18.4 ± 5.8%; p=0.002).	Weight, height, BMI and body composition assessment.
Doulgeraki et al.²⁹29. Doulgeraki A, Skarpalezou A, Theodosiadou A, Monopolis I, Schulpis K. Body composition profile of young patients with phenylketonuria and mild hyperphenylalaninemia. Int J Endocrinol Metab. 2014;12:160-1. https://doi.org/10.5812/ijem.16061 https://doi.org/https://doi.org/10.5812/...	Higher mean BMI Z-scores and body weight of individuals with PKU were observed compared to the control group. There was also a significant increase in fat mass in adolescents with PKU compared to prepubescent patients.	Z scores of weight, height, BMI and a body composition assessment.
Rocha et al.²⁶26. Rocha JC, van Spronsen FJ, Almeida MF, Soares G, Quelhas D, Ramos E, et al. Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome. Mol Genet Metab. 2012;107:659-63. https://doi.org/10.1016/j.ymgme.2012.10.006 https://doi.org/https://doi.org/10.1016/...	Overweight in patients with PKU: 32.6% Overweight in the controls: 24.1% Mean body fat (%) in CNS patients and controls were similar: 22.0% (95% CI 14.4-28.9) versus 23.1% (95% CI 16.3-28.9); p = 0.581.	Z scores of weight, height, BMI and a body composition assessment.

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[1] *Corresponding author. E-mail: berilanysena@outlook.com (B.S. Sena).