Table 3.
Change in plasma ammonia concentration from baseline to achievement of normalised plasma ammoniaab
| Episodes n | Mean change at NH3 ≤ 60 μmol/L | Mean time to achieve NH3 ≤ 60 μmol/L, days | |
|---|---|---|---|
| Total | 42 | -272.8 | 2.4 |
| Isovaleric aciduria | 3 | -299.0 | 2.3 |
| Methylmalonic aciduria | 20 | -233.1 | 1.9 |
| Propionic aciduria | 19 | -310.5 | 3.0 |
| Neonates | 24 | -392.6 | 2.0 |
| Non-neonates | 18 | -113.2 | 3.1 |
| With concomitant ammonia scavengers | 14 | -344.7 | 2.3 |
| Without concomitant ammonia scavengers | 22 | -157.2 | 2.8 |
| With haemofiltration (with or without ammonia scavengers) | 6 | -529.3 | 1.5 |
aNormalised plasma ammonia defined as NH3 ≤ 60 μmol/L
bEfficacy population
NH3, ammonia