Metabolic syndrome in children and adolescents with phenylketonuria

Kanufre, Viviane C.; Soares, Rosângelis D.L.; Alves, Michelle Rosa A.; Aguiar, Marcos J.B.; Starling, Ana Lúcia P.; Norton, Rocksane C.

doi:10.1016/j.jped.2014.06.006

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Original Articles • J. Pediatr. (Rio J.) 91 (1) • Jan-Feb 2015 • https://doi.org/10.1016/j.jped.2014.06.006 copy

Metabolic syndrome in children and adolescents with phenylketonuria^☆ ☆ Please cite this article as: Kanufre VC, Soares RD, Alves MR, Aguiar MJ, Starling AL, Norton RC. Metabolic syndrome in children and adolescents with phenylketonuria. J Pediatr (Rio J). 2015;91:98-103. ^☆☆ ☆☆ Study conducted at Núcleo de Ações e Pesquisa em Apoio Diagnóstico (NUPAD), Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.

Authorship SCIMAGO INSTITUTIONS RANKINGS

OBJECTIVE:

This study aimed to identify markers of metabolic syndrome (MS) in patients with phenylketonuria (PKU).

METHODS:

This was a cross-sectional study consisting of 58 PKU patients (ages of 4-15 years): 29 patients with excess weight, and 29 with normal weight. The biochemical variables assessed were phenylalanine (phe), total cholesterol, HDL-c, triglycerides, glucose, and basal insulin. The patients had Homeostasis Model Assessment (HOMA) and waist circumference assessed.

RESULTS:

No inter-group difference was found for phe. Overweight patients had higher levels of triglycerides, basal insulin, and HOMA, but lower concentrations of HDL-cholesterol, when compared to the eutrophic patients. Total cholesterol/HDL-c was significantly higher in the overweight group. A positive correlation between basal insulin level and HOMA with waist circumference was found only in the overweight group.

CONCLUSION:

The results of this study suggest that patients with PKU and excess weight are potentially vulnerable to the development of metabolic syndrome. Therefore, it is necessary to conduct clinical and laboratory monitoring, aiming to prevent metabolic changes, as well as excessive weight gain and its consequences, particularly cardiovascular risk.

Phenylketonuria; Metabolic syndrome; Children and adolescents; Overweight; Diet

Variables	Study Groups
	Excess weight (n = 29)	Normal weight (n = 29)	p-value
Age (years)	9.3 (± 3.1)	9 (± 2.4)	0.66^a
Weight (kg)	35.8 (± 24.0)	28.0 (± 10.6)	0.01^b
Height (cm)	134.7 (± 20.2)	132.1 (± 13.3)	0.67^a

Gender, n (%)
Female	18 (62.1)	10 (34.5)	0.04^c
Male	11 (37.9)	19 (65.5)

Pubertal stage, n (%)
Prepubertal	17 (58.6)	21 (72.4)	0.27^c
Pubertal	12 (42.8)	8 (28.5)

Variables	Study groups
	Excess weight (n = 29)	Normal weight (n = 29)	p-value
Cholesterol (mg/dL)
Total	136.2 (± 22.2)	130.7 (± 20.2)	0.32^a
HDL-c	34.0 (± 7.5)	37.0 (± 8.0)	0.00^b

Triglycerides (mg/dL)	109.0 (± 43.0)	74.0 (± 40.5)	0.00^b
Glycemia (mg/dL)	73.8 (± 6.4)	76.3 (± 6.3)	0.13^a
Basal insulin (uUI/mL)	8.4 (± 8.95)	3.8 (± 5.15)	0.02^b

Pubertal stage	Variable	Nutritional status	Values mg/dL	p
Prepubertal (n = 38)	Trig	Excess weight	33.7 ± 6.4	0.00^a
		Normal weight	40.1 ± 8.1
	TC	Excess weight	140.1 ± 23.5	0.24^b
		Normal weight	131.1 ± 22.9
	HDL-c	Excess weight	33.7 ± 6.4	0.01^b
		Normal weight	40.1 ± 8.1
Pubertal (n = 20)	Trig	Excess weight	105.5 ± 57.7	0.4^a
		Normal weight	83.0 ± 53.7
	TC	Excess weight	131.0 ± 36.2	0.9^a
		Normal weight	132.0 ± 4.7
	HDL-c	Excess weight	36.5 ± 10.7	0.52^a
		Normal weight	37.0 ± 4.0

	Study groups
	Excess weight	Normal weight	p^a
Total cholesterol/HDL-c	4.00 (± 0.84)	3.33 (± 0.81)	0.00
Insulin (uUI/mL)	8.40 (± 8.95)	3.8 (± 5.15)	0.01
HOMA	1.64 (± 1.59)	0.70 (± 1.10)	0.03

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[1] * Corresponding author. E-mail: vikanufre@gmail.com.br (V.C. Kanufre).