Body Composition in Adolescent PKU Patients: Beyond Fat Mass
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Body Composition
2.3. QUS (Quantitative Ultrasound Scan)
2.4. Statistical Analysis
3. Results
3.1. Comparison of BIA and QUS Parameters in Different Subgroups
3.2. Comparison of BIA and QUS Parameters before and after the Pubertal Spurt
3.3. Comparison of BIA and QUS Parameters According to Type of Protein Substitute
3.4. Correlation of BIA and QUS Parameters with Protein Intake and Compliance to Diet
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Total (n = 36) | p Group (n = 17) | P Group (n = 19) | p-Value | |
---|---|---|---|---|
Males (%) | 16 (44%) | 6 (35%) | 10 (53%) | 0.296 |
Age (years) | 11.4 ± 3.9 | 8.1 ± 2.2 | 14.4 ± 2.4 | <0.001 |
Phe restriction + LAA mixtures + GMP | 5 (14%) | 3 (18%) | 2 (11%) | 0.650 |
Total protein intake (g/Kg/day) | 1.24 ± 0.30 | 1.39 ± 0.31 | 1.10 ± 0.21 | 0.002 |
Natural protein intake (g/kg/day) | 0.39 [0.22–0.75] | 0.41 [0.29–0.79] | 0.32 [0.19–0.70] | 0.288 |
PE intake (g/kg/day) | 0.77 ± 0.32 | 0.86 ± 0.28 | 0.68 ± 0.34 | 0.094 |
GMPs intake (g/day) * | 12.4 ± 2.2 | 12.1 ± 2.9 | 13 ± 1.4 | 0.711 |
GMPs (g/kg/day)/ total PE (g/kg/day) (%) * | 57 ± 32.3 | 54 ± 13.3 | 61 ± 34.9 | 0.473 |
Total caloric intake (Kcal/Kg/day) | 47.1 ± 17.1 | 58.7 ± 17.2 | 36.8 ± 8.0 | <0.001 |
Phe intake (mg/kg/day) | 13.4 [7.6–20.6] | 16.9 [10.76–21] | 9 [5.4–17.7] | 0.034 |
Phe levels | 389.7 ± 199 | 313.5 ± 106.8 | 457.9 ± 237.5 | 0.013 |
Patients non-compliant with diet (%) | 9/36 (25%) | 3/17 (18%) | 6/19 (32%) | 0.451 |
Parameter | p-Group Compliant (a) | p-Group Non-Compliant (b) | P-Group Compliant (c) | P-Group Non-Compliant (d) | (a) vs. (b) | (c) vs. (d) | (a) vs. (c) | (b) vs. (d) |
---|---|---|---|---|---|---|---|---|
LS. Means ± Standard Error | Adjusted p-Value * | |||||||
BMI | 17.4 ± 0.9 | 19.3 ± 1.9 | 21.3 ± 0.8 | 25.2 ± 1.2 | 0.818 | 0.067 | 0.018 | 0.059 |
sds-BMI | −0.1 ± 0.3 | 0.7 ± 0.7 | 0.2 ± 0.3 | 0.8 ± 0.4 | 0.761 | 0.652 | 0.911 | 0.997 |
FM n.v. 5.51–9.51 n.v. 10.01–20.07 | 7.5 ± 2.4 | 12.9 ± 4.6 | 12.2 ± 2.2 | 11.9 ± 3.4 | 0.765 | 1.000 | 0.492 | 0.998 |
PBF% n.v. 11.75–21.75 n.v. 13.89–23.89 | 27.2 ± 4.4 | 28.9 ± 8.5 | 21.8 ± 4 | 13.5 ± 6.2 | 0.998 | 0.687 | 0.798 | 0.463 |
TBW n.v.14.41–17.63 n.v. 35.38–43.26 | 15 ± 2 | 18.3 ± 3.7 | 30.9 ± 1.8 | 45.6 ± 2.7 | 0.881 | 0.001 | <0.001 | <0.001 |
WHR n.v.0.76–0.86 n.v. 0.78–0.88 | 0.74 ± 0.02 | 0.8 ± 0.05 | 0.85 ± 0.02 | 0.83 ± 0.03 | 0.710 | 0.930 | 0.010 | 0.957 |
MM n.v. 9.71–11.87 n.v. 26.77–32.78 | 10 ± 1.6 | 12.8 ± 3.1 | 23 ± 1.4 | 35.3 ± 2.3 | 0.873 | 0.001 | <0.001 | <0.001 |
sds-BQI | −1.6 ± 0.4 | −1.9 ± 0.9 | −1.8 ± 0.3 | −0.9 ± 0.5 | 0.984 | 0.496 | 0.978 | 0.755 |
BQI | 54.8 ± 4.3 | 50.1 ± 10.6 | 69.6 ± 4 | 75.4 ± 5.9 | 0.978 | 0.850 | 0.081 | 0.182 |
Leucine | 135 ± 11 | 106 ± 27 | 141 ± 10 | 147 ± 15 | 0.770 | 0.991 | 0.980 | 0.573 |
Isoleucine | 69.9 ± 4.7 | 54.6 ± 12 | 76.6 ± 4.7 | 65.9 ± 6.7 | 0.667 | 0.570 | 0.755 | 0.847 |
Valine | 227 ± 12 | 205 ± 30 | 243 ± 12 | 231 ± 17 | 0.908 | 0.940 | 0.795 | 0.874 |
General Characteristics | BIA Parameters | QUS Parameters | |||||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Pts | Sex | Age | Tunner Stage | Phe Values (Mean SD) | BMI | SDS BMI | FM | PBF | TBW | WHR | MM | BQI | SDS-BQI | ||||||||||||
1 | F | 9.3 | 11.5 | I | III | 217 | 148 | 22.15 | 26.21 | 1.31 | 1.62 | 20.70 | 21.80 | 53.70% | 40.60% | 13.20 | 23.50 | 0.75 | 0.87 | 8.60 | 16.80 | 54.26 | 75 | 0.90 | −0.30 |
2 | F | 10.7 | 12.9 | I | III | 312 | 368 | 15.67 | 19.34 | −1.22 | −0.31 | 4.8 | 9.2 | 15.30% | 20.9% | 19.7 | 25.7 | 0.79 | 0.83 | 13.9 | 18.7 | 61.39 | 66.49 | −1.70 | −2.90 |
3 | M | 9.1 | 12.8 | I | III | 364 | 708 | 21.40 | 27.60 | 1.23 | 1.74 | 21.90 | 29.10 | 42.30% | 42.80% | 21.90 | 28.50 | 0.86 | 0.98 | 15.70 | 21.10 | 61.97 | 59.15 | −1.00 | −2.20 |
4 | F | 10.7 | 12.9 | I | II | 355 | 645 | 21.23 | 22.60 | 0.76 | 0.68 | 15.10 | 15.60 | 35.80% | 29.8% | 19.9 | 26.9 | 0.79 | 0.82 | 14 | 19.60 | 71.40 | 81 | −1.40 | −1.30 |
5 | M | 12.1 | 14.8 | I | II | 350 | 487 | 18.43 | 23.4 | −0.39 | 0.66 | 13.5 | 19 | 39.10% | 36.50% | 15.5 | 24.3 | 0.76 | 0.85 | 10.6 | 17.5 | 52 | 65 | −2.4 | −2.3 |
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Tummolo, A.; Carella, R.; Paterno, G.; Bartolomeo, N.; Giotta, M.; Dicintio, A.; De Giovanni, D.; Fischetto, R. Body Composition in Adolescent PKU Patients: Beyond Fat Mass. Children 2022, 9, 1353. https://doi.org/10.3390/children9091353
Tummolo A, Carella R, Paterno G, Bartolomeo N, Giotta M, Dicintio A, De Giovanni D, Fischetto R. Body Composition in Adolescent PKU Patients: Beyond Fat Mass. Children. 2022; 9(9):1353. https://doi.org/10.3390/children9091353
Chicago/Turabian StyleTummolo, Albina, Rosa Carella, Giulia Paterno, Nicola Bartolomeo, Massimo Giotta, Annamaria Dicintio, Donatella De Giovanni, and Rita Fischetto. 2022. "Body Composition in Adolescent PKU Patients: Beyond Fat Mass" Children 9, no. 9: 1353. https://doi.org/10.3390/children9091353