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Table 3 Treatment during hospitalisation; n = number of episodes (safety set)

From: Intravenous administration of a branched-chain amino-acid-free solution in children and adults with acute decompensation of maple syrup urine disease: a prospective multicentre observational study

 

Children (N = 39)

Adults (N = 87)

All (N = 126)

All episodes

IV BCAA-free solution treatment duration, days*

N

30

75

105

Mean (SD)

4.8 (5.4)

3.8 (1.4)

4.1 (3.1)

Median [min; max]

3.0 [1.0; 30.0]

4.0 [1.0; 8.0]

4.0 [1.0; 30.0]

Dose (g/kg/day)

N

26

40

66

Mean (SD)

1.9 (0.3)

1.3 (0.5)

1.5 (0.5)

Median [min; max]

2.0 [0.8; 2.0]

1.1 [0.5; 2.6]

1.5 [0.5; 2.6]

Hospitalisation duration, days

N

39

86

125

Mean (SD)

7.1 (4.9)

5.2 (3.0)

5.8 (3.8)

Median [min; max]

6.0 [1.0; 30.0]

5.0 [2.0; 27.0]

5.0 [1.0; 30.0]

N (%) of episodes not treated with HF/HD**

32 (82.1%)

86 (98.9)

118 (93.7%)

IV BCAA-free solution treatment duration in episodes not treated with HF/HD, days

N

23

74

97

Mean (SD)

3.4 (2.0)

3.8 (1.4)

3.7 (1.6)

Median [min; max]

3.0 [1.0; 8.0]

4.0 [1.0; 8.0]

4.0 [1.0; 8.0]

Hospitalisation duration without HF/HD, days

N

32

85

117

Mean (SD)

6.0 (3.1)

5.2 (3.0)

5.4 (3.0)

Median [min; max]

5.0 [1.0; 13.0]

5.0 [2.0; 27.0]

5.0 [1.0; 27.0]

N (%) of episodes treated with HF/HD**

7 (17.9%)

1 (1.1%)

8 (6.3%)

Mean (SD) IV BCAA-free solution treatment duration in episodes treated with HF/HD, days**

9.3 (9.6)

2.0 (-)

8.4 (9.3)

Mean (SD) hospitalisation duration with HF/HD, days

12.0 (8.3)

3.0 (-)

10.9 (8.3)

  1. HF/HD haemofiltration/haemodialysis; SD standard deviation.
  2. *Data from 21 episodes were missing, 9 in children and 12 in adults.
  3. **N for episodes that included HF/HD were 8 in total, 7 in children and 1 adult. A total of 21 episodes were missing HF/HD treatment data, 9 in children and 12 in adults.