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Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage.

Albersen M, Bonthuis M, de Roos NM, van den Hurk DA, Carbasius Weber E, Hendriks MM, de Sain-van der Velden MG, de Koning TJ, Visser G - J. Inherit. Metab. Dis. (2010)

Bottom Line: The BodPod whole body air-displacement plethysmography method is a fast, safe and accurate technique to measure body composition.The mean body fat percentage in patients with PKU (n = 20) was significantly higher compared to healthy controls (n = 20) (25.2% vs 18.4%; p = 0.002), especially in girls above 11 years of age (30.1% vs 21.5%; p = 0.027).Body fat percentage increased with rising body weight in patients with PKU only (R = 0.693, p = 0.001), but did not correlate with mean blood PHE level (R = 0.079, p = 0.740).

View Article: PubMed Central - PubMed

Affiliation: Department of Metabolic and Endocrine Diseases, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Huispost KC02.069.1, Lundlaan 6, 3584 EA Utrecht, The Netherlands.

ABSTRACT

Background: Phenylketonuria (PKU) causes irreversible central nervous system damage unless a phenylalanine (PHE) restricted diet with amino acid supplementation is maintained. To prevent growth retardation, a protein/amino acid intake beyond the recommended dietary protein allowance is mandatory. However, data regarding disease and/or diet related changes in body composition are inconclusive and retarded growth and/or adiposity is still reported. The BodPod whole body air-displacement plethysmography method is a fast, safe and accurate technique to measure body composition.

Aim: To gain more insight into the body composition of children with PKU.

Methods: Patients diagnosed with PKU born between 1991 and 2001 were included. Patients were identified by neonatal screening and treated in our centre. Body composition was measured using the BodPod system (Life Measurement Incorporation©). Blood PHE values determined every 1-3 months in the year preceding BodPod analysis were collected. Patients were matched for gender and age with data of healthy control subjects. Independent samples t tests, Mann-Whitney and linear regression were used for statistical analysis.

Results: The mean body fat percentage in patients with PKU (n = 20) was significantly higher compared to healthy controls (n = 20) (25.2% vs 18.4%; p = 0.002), especially in girls above 11 years of age (30.1% vs 21.5%; p = 0.027). Body fat percentage increased with rising body weight in patients with PKU only (R = 0.693, p = 0.001), but did not correlate with mean blood PHE level (R = 0.079, p = 0.740).

Conclusion: Our data show a higher body fat percentage in patients with PKU, especially in girls above 11 years of age.

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Related in: MedlinePlus

Scatterplot with regression lines of body fat percentage against body weight in 20 patients with PKU () (R = 0.693, p = 0.001)*(—) and in 20 healthy control subjects () (R = 0.250, p = 0.287) (------). *p < 0.05
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Fig3: Scatterplot with regression lines of body fat percentage against body weight in 20 patients with PKU () (R = 0.693, p = 0.001)*(—) and in 20 healthy control subjects () (R = 0.250, p = 0.287) (------). *p < 0.05

Mentions: The mean body fat percentage was significantly higher in patients with PKU compared to healthy control subjects (25.2 ± 7.3% vs 18.4 ± 5.8%; p = 0.002) (Fig. 2, Table 2). This difference was most pronounced in girls (24.8 ± 8.2% vs 18.7 ± 4.4%; p = 0.026) and especially above 11 years of age (30.1 ± 5.5% vs 21.5 ± 2.2%; p = 0.027) (Table 2). Furthermore, body fat percentage in patients with PKU increased with body weight (R = 0.693, p = 0.001) (Fig. 3). In the healthy control group, this increase was not observed (R = 0.250, p = 0.287) (Fig. 3).Fig. 2


Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage.

Albersen M, Bonthuis M, de Roos NM, van den Hurk DA, Carbasius Weber E, Hendriks MM, de Sain-van der Velden MG, de Koning TJ, Visser G - J. Inherit. Metab. Dis. (2010)

Scatterplot with regression lines of body fat percentage against body weight in 20 patients with PKU () (R = 0.693, p = 0.001)*(—) and in 20 healthy control subjects () (R = 0.250, p = 0.287) (------). *p < 0.05
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3757265&req=5

Fig3: Scatterplot with regression lines of body fat percentage against body weight in 20 patients with PKU () (R = 0.693, p = 0.001)*(—) and in 20 healthy control subjects () (R = 0.250, p = 0.287) (------). *p < 0.05
Mentions: The mean body fat percentage was significantly higher in patients with PKU compared to healthy control subjects (25.2 ± 7.3% vs 18.4 ± 5.8%; p = 0.002) (Fig. 2, Table 2). This difference was most pronounced in girls (24.8 ± 8.2% vs 18.7 ± 4.4%; p = 0.026) and especially above 11 years of age (30.1 ± 5.5% vs 21.5 ± 2.2%; p = 0.027) (Table 2). Furthermore, body fat percentage in patients with PKU increased with body weight (R = 0.693, p = 0.001) (Fig. 3). In the healthy control group, this increase was not observed (R = 0.250, p = 0.287) (Fig. 3).Fig. 2

Bottom Line: The BodPod whole body air-displacement plethysmography method is a fast, safe and accurate technique to measure body composition.The mean body fat percentage in patients with PKU (n = 20) was significantly higher compared to healthy controls (n = 20) (25.2% vs 18.4%; p = 0.002), especially in girls above 11 years of age (30.1% vs 21.5%; p = 0.027).Body fat percentage increased with rising body weight in patients with PKU only (R = 0.693, p = 0.001), but did not correlate with mean blood PHE level (R = 0.079, p = 0.740).

View Article: PubMed Central - PubMed

Affiliation: Department of Metabolic and Endocrine Diseases, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Huispost KC02.069.1, Lundlaan 6, 3584 EA Utrecht, The Netherlands.

ABSTRACT

Background: Phenylketonuria (PKU) causes irreversible central nervous system damage unless a phenylalanine (PHE) restricted diet with amino acid supplementation is maintained. To prevent growth retardation, a protein/amino acid intake beyond the recommended dietary protein allowance is mandatory. However, data regarding disease and/or diet related changes in body composition are inconclusive and retarded growth and/or adiposity is still reported. The BodPod whole body air-displacement plethysmography method is a fast, safe and accurate technique to measure body composition.

Aim: To gain more insight into the body composition of children with PKU.

Methods: Patients diagnosed with PKU born between 1991 and 2001 were included. Patients were identified by neonatal screening and treated in our centre. Body composition was measured using the BodPod system (Life Measurement Incorporation©). Blood PHE values determined every 1-3 months in the year preceding BodPod analysis were collected. Patients were matched for gender and age with data of healthy control subjects. Independent samples t tests, Mann-Whitney and linear regression were used for statistical analysis.

Results: The mean body fat percentage in patients with PKU (n = 20) was significantly higher compared to healthy controls (n = 20) (25.2% vs 18.4%; p = 0.002), especially in girls above 11 years of age (30.1% vs 21.5%; p = 0.027). Body fat percentage increased with rising body weight in patients with PKU only (R = 0.693, p = 0.001), but did not correlate with mean blood PHE level (R = 0.079, p = 0.740).

Conclusion: Our data show a higher body fat percentage in patients with PKU, especially in girls above 11 years of age.

Show MeSH
Related in: MedlinePlus