The BMI Z-Score and Protein Energy Ratio in Early- and Late-Diagnosed PKU Patients from a Single Reference Center in Mexico
Abstract
:1. Introduction
2. Materials and Methods
2.1. Study Design
2.2. Study Population
2.3. Nutritional Assessment
2.4. Statistical Analysis
2.5. Ethical Considerations
3. Results
3.1. Patient Description
3.2. Nutritional Assessment
3.3. Protein: Energy Ratio
3.4. Protein Intake Proportion between Intact Protein and Metabolic Formula
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
BMI | body mass index |
HPA | hyperphenylalaninemia |
PKU | phenylketonuria |
P:E ratio | protein and energy ratio |
PAH | Phenylalanine hydroxylase |
Phe | phenylalanine |
WHO | World Health Organization |
IEM | inborn error of metabolism |
Tyr | tyrosine |
SERN | Southeast Regional Genetics Network |
GMDI | Genetic Metabolic Dietitians International |
BH4 | tetrahydrobiopterin |
cPKU | classical PKU |
mPKU | mild PKU |
MHP | mild hyperphenylalaninemia |
IQR | interquartile range |
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Assessment from NBS Diagnosed Patients (n = 442) | |||||
---|---|---|---|---|---|
Infants n (%) | Preschoolers n (%) | Schoolchildren n (%) | Teenagers n (%) | TOTAL (%) | |
Underweight | 39 (67.2) | 13 (22.4) | 6 (10.3) | 0 (0) | 58 (13.1) |
Eutrophic | 160 (49.7) | 111 (34.5) | 34 (10.6) | 17 (5.3) | 322 (72.9) |
Overweight | 19 (30.6) | 25 (40.3) | 18 (29) | 0 (0) | 62 (14.0) |
Assessment from late-diagnosed patients (n = 196) | |||||
Infants n (%) | Preschoolers n (%) | Schoolchildren n (%) | Teenagers n (%) | TOTAL (%) | |
Underweight | 3 (24.5) | 18 (37.5) | 13 (27.1) | 14 (29.2) | 48 (24.5) |
Eutrophic | 28 (61.7) | 43 (35.5) | 43 (35.5) | 7 (5.8) | 121 (61.7) |
Overweight | 8 (13.8) | 13 (48.1) | 0 (0) | 6 (22.2) | 27 (13.9) |
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López-Mejía, L.A.; Fernández-Lainez, C.; Vela-Amieva, M.; Ibarra-González, I.; Guillén-López, S. The BMI Z-Score and Protein Energy Ratio in Early- and Late-Diagnosed PKU Patients from a Single Reference Center in Mexico. Nutrients 2023, 15, 957. https://doi.org/10.3390/nu15040957
López-Mejía LA, Fernández-Lainez C, Vela-Amieva M, Ibarra-González I, Guillén-López S. The BMI Z-Score and Protein Energy Ratio in Early- and Late-Diagnosed PKU Patients from a Single Reference Center in Mexico. Nutrients. 2023; 15(4):957. https://doi.org/10.3390/nu15040957
Chicago/Turabian StyleLópez-Mejía, Lizbeth Alejandra, Cynthia Fernández-Lainez, Marcela Vela-Amieva, Isabel Ibarra-González, and Sara Guillén-López. 2023. "The BMI Z-Score and Protein Energy Ratio in Early- and Late-Diagnosed PKU Patients from a Single Reference Center in Mexico" Nutrients 15, no. 4: 957. https://doi.org/10.3390/nu15040957