Table 1.
Patients characteristics
| 1. Children | N = 21 | N (%) |
|---|---|---|
| Sex |
Boys |
10 (47.6) |
| Girls |
11 (52.4) |
|
| Age (years) |
Mean ± SD |
8.25 ± 5.60 |
| M (min-max) |
8.6 (1–18) |
|
| Nature of IEMRD |
Organic aciduria |
10 (47.6) |
| Urea cycle defect |
6 (28.6) |
|
| MSUD |
5 (23.8) |
|
| Feeding modality at inclusion |
Exclusively oral |
12 (57.1) |
| Oral and gastrostomy |
9 (42.9) |
|
| Eating disorders at inclusion |
|
6 (28.5) |
| Neurologic disorder at inclusion |
|
10 (47.6) |
| Renal/cardiac/hepatic disorders at inclusion |
|
9 (42.9) |
| Enteral nutrition |
|
15 (71.4) |
| Current gastrostomy |
|
8 (38.1) |
| Protein (Gr/kg/j) |
Mean ± SD |
1.07 ± 0.32 |
| M (min-max) |
1.14 (0.18-1.49) |
|
| Natural protein (Gr/kg/j) |
Mean ± SD |
0.67 ± 0.29 |
| |
M (min-max) |
0.71 (0.17-1.16) |
|
2. Parents |
N = 21 |
|
| Age (years) |
Mean ± SD |
39.14 ± 6.64 |
| M (min-max) |
39 (30–58) |
|
| Occupational status |
Yes |
11 (52.4) |
| No | 10 (47.6) |
IEMRD: Inborn errors of metabolism with restricting dietary.
MSUD: maple syrup urine disease.
SD standard deviation, M median, min-max minimum and maximum.