A Patient with MSUD: Acute Management with Sodium Phenylacetate/Sodium Benzoate and Sodium Phenylbutyrate

Table 1

Values of plasma leucine, isoleucine, valine, alanine, glutamine, glycine, and ammonia during periods of therapy.

	Normal values	0–19 months	NaPB 17th day (discharging)	NaPB 3 months	NaPB 6 months	NaPB 12 months
Leucine (µmol/L)	33–124		108	142	167	138
Isoleucine (µmol/L)	27–110		92	87	52	67
Valine (µmol/L)	30–125		98	101	119	121
Alanine (µmol/L)	54–250		234	207	267	283
Glutamine (µmol/L)	80–450		267	199	301	276
Glycine (µmol/L)	50–230		182	178	167	176
Ammonia (µmol/L)	<70		101	77	63	92