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. 2016 Mar 31;11:32. doi: 10.1186/s13023-016-0406-2

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