Table 1 Patients characteristics

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)