Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria
Abstract
:1. Introduction
2. Materials and Methods
2.1. Project Design
2.2. Data Collection
2.3. Anthropometry
2.4. Dietary Assessment
2.5. Blood Phe Levels
2.6. Reassessment of NP Tolerance
2.7. Ethical Statement
2.8. Statistics
3. Results
3.1. Subjects
3.2. Change in Natural Protein Intake
3.3. Source of Natural Protein
3.4. Protein Substitute
3.5. Energy Intake
3.6. Blood Phe Control
3.7. Changes in Anthropometric Measurements
4. Discussion
5. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Covariate | Level | Classical PKU | HPA | Mild PKU | Total |
---|---|---|---|---|---|
Total | 7 | 10 | 23 | 40 | |
Gender | F | 2 (12%) | 3 (19%) | 11 (69%) | 16 |
M | 5 (21%) | 7 (29%) | 12 (50%) | 24 | |
Age (months) | median (IQR) | 190 (169.0, 243.5) | 185.5 (168.3, 214.5) | 190 (179.5, 270.5) | 190 (175.5, 256.0) |
Diagnostic level (blood Phe in mg/dl) | median (IQR) | 25.2 (21.65, 27.75) | 4.65 (4.05, 5.18) | 11 (7.65, 15.00) | 9.95 (5.88, 16.00) |
Months between assessments | median (IQR) | 6 (5.5, 8.0) | 6.5 (5.3, 7.0) | 7 (5.5, 7.0) | 6.5 (5, 7.3) |
Covariate | Level | Visit 1 | Visit 2 | Total |
---|---|---|---|---|
Weight (kg) | median (IQR) | 52.50 (44.86, 68.63) | 58 (47.75, 69.88) | 54.25 (45.38, 69.00) |
Height (cm) | median (IQR) | 161.0 (155.5, 167.8) | 163.1 (157.6, 169.1) | 162 (156.0, 169.0) |
Height Z score | median (IQR) | −0.33 (−0.84, 0.29) | −0.07 (−0.88, 0.2) | −0.27 (−0.88, 0.27) |
BMI (kg/m2) | median (IQR) | 21.05 (17.98, 23.63) | 21.60 (18.28, 24.23) | 21.30 (18.15, 24.03) |
BMI Z score | median (IQR) | 0.00 (−0.74, 1.05) | 0.37 (−0.91, 1.43) | 0.28 (−0.76, 1.20) |
Natural Protein (g/day) | median (IQR) | 35.0 (23.5, 60.0) | 40.0 (25.5, 74.4) | 35.0 (24.0, 70.3) |
Natural Protein (g/kg/day) | median (IQR) | 0.66 (0.44, 1.04) | 0.71 (0.40, 1.14) | 0.70 (0.42, 1.06) |
Phe * (mg/kg/day) | median (IQR) | 32.8 (21.9, 51.9) | 35.3 (20.0, 57.1) | 35.0 (20.9, 53.1) |
Protein equivalent intake from low-Phe/Phe-free protein substitute (g/day) | median (IQR) | 45.0 (27.0, 55.4) | 41.5 (19.5, 54.8) | 44.8 (24.0, 55.1) |
Protein equivalent intake from low-Phe/Phe-free protein substitute (g/kg/day) | median (IQR) | 0.82 (0.51, 1.07) | 0.75 (0.34, 0.99) | 0.82 (0.48, 1.04) |
Energy intake (Kcal/day) | median (IQR) | 2371 (2200, 2536) | 2365 (2210, 2603) | 2365 (2202, 2575) |
Blood Phe Levels (μmol/L) | median (IQR) | 279 (194, 328) | 284 (210, 375) | 279 (203, 360) |
Number of blood spots | median (IQR) | 6 (4, 9) | 11 (7, 14) | 9 (5, 12) |
% Nutritional Protein Increase | Total | HPA | Mild PKU | Classical PKU |
Minimum 20% natural protein increase | 20/40 (50%) | 5/10 (50%) | 11/23 (48%) | 4/7 (57%) |
Minimum 50% natural protein increase | 6/40 (15%) | 3/10 (30%) | 1/23 (4%) | 2/7 (29%) |
Source of extra natural protein | Total | HPA | Mild PKU | Classical PKU |
Animal foods: Dairy products, fish and meat | 20/26 (77%) | 8/8 (100%) | 11/14 (79%) | 1/4(25%) |
Plant foods: Bread, pasta and potatoes | 6/26 (23%) | 0 | 3/14 (21%) | 3/4 (75%) |
Sample | Height (cm) (1st vs. 2nd Assessment, p-Value) | Height z-Scores (1st vs. 2nd Assessment, p-Value) | BMI (kg/m2) (1st vs. 2nd Assessment, p-Value) | BMI z-Scores (1st vs. 2nd Assessment, p-Value) |
---|---|---|---|---|
Patients <19 years (n = 29) | 159.2 vs. 163.0, p < 0.001 | −0.33 vs. −0.07, p = 0.933 | 19.6 vs. 21.3, p = 0.06 | 0.00 vs. 0.37, p = 0.405 |
Patients <19 years that increased natural protein intake (n = 19) | 161.0 vs. 163.0, p = 0.001 | 0.02 vs. 0.06, p = 0.469 | 20.2 vs. 21.3, p = 0.034 | 0.14 vs. 0.37, p = 0.920 |
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Pinto, A.; Ferreira Almeida, M.; MacDonald, A.; Cristina Ramos, P.; Rocha, S.; Guimas, A.; Ribeiro, R.; Martins, E.; Bandeira, A.; Jackson, R.; et al. Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria. Nutrients 2019, 11, 995. https://doi.org/10.3390/nu11050995
Pinto A, Ferreira Almeida M, MacDonald A, Cristina Ramos P, Rocha S, Guimas A, Ribeiro R, Martins E, Bandeira A, Jackson R, et al. Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria. Nutrients. 2019; 11(5):995. https://doi.org/10.3390/nu11050995
Chicago/Turabian StylePinto, Alex, Manuela Ferreira Almeida, Anita MacDonald, Paula Cristina Ramos, Sara Rocha, Arlindo Guimas, Rosa Ribeiro, Esmeralda Martins, Anabela Bandeira, Richard Jackson, and et al. 2019. "Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria" Nutrients 11, no. 5: 995. https://doi.org/10.3390/nu11050995
APA StylePinto, A., Ferreira Almeida, M., MacDonald, A., Cristina Ramos, P., Rocha, S., Guimas, A., Ribeiro, R., Martins, E., Bandeira, A., Jackson, R., van Spronsen, F., Payne, A., & César Rocha, J. (2019). Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria. Nutrients, 11(5), 995. https://doi.org/10.3390/nu11050995