Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study
Abstract
:1. Introduction
2. Materials and Methods
2.1. Project Design
2.2. NTBC and succinylacetone concentrations
2.3. Dietary prescriptions
2.4. Anthropometry
2.5. Ethical statement
2.6. Statistics
3. Results
3.1. Patients
3.2. Dietary prescription
3.3. Anthropometric Parameters
3.4. Overweight and obesity by age
3.5. Serum NTBC and plasma succinylacetone concentrations
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Definitions | BMI-for-Age z-Score | |
---|---|---|
<5 Years | 5–19 Years | |
Overweight | ≥ +2 SD | ≥ +1 SD |
Obesity | ≥ +3 SD | ≥ +2 SD |
Age (years) | Number of patients | Height (cm) | Height-for-age z-score | Weight (kg) | Weight-for-age z-score | BMI (kg/m2) | BMI-for-age z-score | Protein (g/kg/day) | ||
---|---|---|---|---|---|---|---|---|---|---|
Total | NP | PE from PS | ||||||||
Median (Q1, Q3) | ||||||||||
1 | 16 | 73.4 (71.6, 75.8) | −1.0 (−1.7, −0.3) | 8.9 (8.8, 10.2) | −0.8 (−1.4, −0.2) | 16.8 (16.2, 18.6) | −0.2 (−1.0, 0.8) | 3.4 (2.9, 3.6) | 0.5 (0.3, 0.7) | 2.8 (2.3, 3.1) |
2 | 17 | 84.0 (80.7, 87.0) | −1.0 (−2.0, −0.6) | 11.7 (11.2, 12.3) | −0.5 (−0.9, −0.2) | 16.6 (15.8, 17.5) | 0.2 (−0.6, 0.8) | 2.8 (2.7, 3.1) | 0.5 (0.3, 0.7) | 2.4 (2.3, 2.6) |
3 | 17 | 91.7 (90.3, 92.5) | −1.3 (−1.5, −1.0) | 13.7 (12.7, 15.0) | −0.7 (−1.1, 0.1) | 16.7 (15.8, 17.7) | 0.5 (−0.2, 1.2) | 2.7 (2.5, 2.9) | 0.4 (0.3, 0.5) | 2.2 (1.9, 2.6) |
4 | 17 | 99.3 (96.1, 100.3) | −1.3 (−1.5, −0.5) | 16.1 (14.4, 17.4) | −0.4 (−1.2, 0.4) | 16.6 (16.0, 17.5) | 0.7 (0.2, 1.2) | 2.5 (2.3, 3.0) | 0.4 (0.3, 0.5) | 2.1 (1.9, 2.7) |
5 | 17 | 104.9 (102.3, 109.3) | −1.2 (−2.0, −0.3) | 17.1 (15.8, 19.4) | −0.6 (−1.5, 0.1) | 16.3 (14.4, 17.5) | 0.6 (−1.0, 1.5) | 2.5 (2.4, 2.9) | 0.5 (0.3, 0.6) | 2.2 (1.9, 2.4) |
6 | 16 | 111.9 (109.5, 114.9) | −1.1 (−1.5, −0.4) | 20.1 (18.5, 22.3) | −0.3 (−1.1, 0.3) | 15.9 (15.0, 18.0) | 0.3 (−0.5, 1.5) | 2.3 (2.0, 2.7) | 0.4 (0.4, 0.5) | 2.0 (1.6, 2.2) |
7 | 16 | 118.8 (115.8, 121.5) | −0.9 (−1.3, −0.2) | 22.9 (19.9, 26.3) | −0.2 (−1.1, 0.7) | 15.8 (14.0, 18.4) | 0.2 (−1.3, 1.5) | 2.3 (2.0, 2.6) | 0.4 (0.3, 0.5) | 1.9 (1.6, 2.2) |
8 | 16 | 123.5 (121.4, 127.5) | −0.8 (−1.3, −0.2) | 25.8 (23.2, 31.4) | 0.0 (−0.9, 1.1) | 16.5 (14.9, 20.0) | 0.4 (−0.6, 1.9) | 2.2 (1.9, 2.2) | 0.4 (0.3, 0.5) | 1.8 (1.5, (1.9) |
9 | 12 | 133.0 (127.0, 134.0) | 0.0 (−1.1, 0.0) | 32.0 (27.1, 37.1) | 0.6 (−0.5, 1.4) | 17.2 (15.1, 20.1) | 1.1 (−0.1, 1.9) | 1.9 (1.7, 2.1) | 0.4 (0.3, 0.5) | 1.6 (1.4, 1.7) |
10 | 14 | 137.0 (133.8, 140.0) | −0.3 (−1.1, 0.2) | 36.6 (29.4, 42.0) | 0.6 (−0.6, 1.4) | 19.3 (15.7, 22.0) | 1.2 (−0.5, 1.9) | 1.8 (1.6, 2.2) | 0.5 (0.4, 0.6) | 1.5 (1.2,1.7) |
11 | 12 | 143.3 (138.9, 146.4) | −0.2 (−1.0, 0.2) | 39.3 (34.1, 46.7) | 0.5 (−0.2, 1.1) | 19.3 (17.8, 21.6) | 0.9 (0.3, 1.7) | 1.6 (1.5, 2.0) | 0.4 (0.3, 0.5) | 1.3 (1.1, 1.5) |
12 | 11 | 150.4 (147.1, 154.1) | 0.0 (−0.7, 0.4) | 47.1 (42.3, 51.0) | 0.6 (0.2, 1.3) | 20.5 (18.7, 22.8) | 1.1 (0.1, 1.8) | 1.5 (1.3, 1.9) | 0.4 (0.3, 0.5) | 1.0 (1.0, 1.4) |
13 | 11 | 154.6 (151.1, 159.7) | 0.0 (−0.9, 0.3) | 52.3 (49.1, 60.1) | 0.6 (0.3, 1.4) | 22.1 (19.3, 24.6) | 1.2 (0.4, 2.0) | 1.4 (1.2, 1.6) | 0.3 (0.3, 0.4) | 1.0 (0.9, 1.2) |
14 | 9 | 161.8 (151.6, 165.3) | 0.1 (−1.6, 0.3) | 62.2 (49.3, 63.1) | 1.1 (−0.3, 1.2) | 22.4 (21.6, 23.1) | 1.3 (0.7, 1.4) | 1.3 (1.2, 1.6) | 0.4 (0.3, 0.4) | 1.0 (0.9, 1.2) |
15 | 7 | 166.2 (162.9, 171.5) | 0.0 (−0.3, 0.3) | 61.9 (53.8, 66.7) | 0.6 (−0.5, 1.2) | 23.8 (18.7, 24.8) | 1.3 (−0.5, 1.7) | 1.5 (1.3, 1.7) | 0.4 (0.3, 0.5) | 1.0 (0.9, 1.2) |
16 | 3 | 172.1 (170.2, 175.6) | −0.2 (−0.4, 0.3) | 71.2 (65.6, 78.0) | 1.0 (0.5, 1.5) | 25.2 (22.7, 25.8) | 1.7 (0.9, 1.8) | 1.3 (1.2, 1.4) | 0.5 (0.3, 0.5) | 0.7 (0.6, 0.9) |
Age (Years) | Number of Patients | Overweight n (%) | Obesity n (%) |
---|---|---|---|
1 | 16 | 0 (0) | 0 (0) |
2 | 17 | 1 (6) | 1 (6) |
3 | 17 | 0 (0) | 1 (6) |
4 | 17 | 2 (12) | 1 (6) |
5 | 17 | 5 (29) | 2 (12) |
6 | 16 | 3 (19) | 2 (13) |
7 | 16 | 6 (38) | 1 (6) |
8 | 16 | 3 (19) | 4 (25) |
9 | 12 | 4 (33) | 2 (17) |
10 | 14 | 5 (36) | 3 (21) |
11 | 12 | 3 (25) | 3 (25) |
12 | 11 | 3 (27) | 3 (27) |
13 | 11 | 5 (46) | 3 (27) |
14 | 9 | 5 (56) | 1 (11) |
15 | 7 | 3 (43) | 1 (14) |
16 | 3 | 2 (67) | 0 (0) |
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Yilmaz, O.; Daly, A.; Pinto, A.; Ashmore, C.; Evans, S.; Gupte, G.; Jackson, R.; Yabanci Ayhan, N.; MacDonald, A. Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study. Nutrients 2021, 13, 3070. https://doi.org/10.3390/nu13093070
Yilmaz O, Daly A, Pinto A, Ashmore C, Evans S, Gupte G, Jackson R, Yabanci Ayhan N, MacDonald A. Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study. Nutrients. 2021; 13(9):3070. https://doi.org/10.3390/nu13093070
Chicago/Turabian StyleYilmaz, Ozlem, Anne Daly, Alex Pinto, Catherine Ashmore, Sharon Evans, Girish Gupte, Richard Jackson, Nurcan Yabanci Ayhan, and Anita MacDonald. 2021. "Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study" Nutrients 13, no. 9: 3070. https://doi.org/10.3390/nu13093070