Skip to main content

Table 5 Predicted time for normalisation of leucine (efficacy 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 = 18)

Adults (N = 67)

All (N = 85)

Leucine at admission

Value (μmol/L)

Mean (SD)

863 (266)

964 (242)

943 (249)

Median [min; max]

869 [396; 1243]

968 [518; 1685]

961 [396; 1685]

Category

381–762 (µmol/L)

7 (38.9%)

11 (16.4%)

18 (21.2%)

 > 762 µmol/L

11 (61.1%)

56 (83.6%)

67 (78.8%)

Predicted time of leucine normalisation (in days)

Based on average leucine tolerance

Mean (SD)

3.6 (1.6)

5.5 (1.9)

5.1 (2.0)

Median [min; max]

3.6 [1.3; 6.3]

5.0 [3.2; 12.4]

4.8 [1.3; 12.4]

Observed time of leucine normalisation (in days)

For [Leu] < 381 μmol/L during episode

Mean (SD)

2.7 (1.1)

3.0 (1.3)

3.0 (1.2)

Median [min; max]

2.0 [1.0; 6.0]

3.0 [1.0; 7.0]

3.0 [1.0; 7.0]

Reduction in time compared with predicted time to leucine normalisation (%)

 

41%

25%

45%

  1. HF/HD haemofiltration/haemodialysis; SD standard deviation