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

Table 2

Nutritional treatment plan during periods of therapy.

	After diagnosis	19 mo of age (before attack)	Acute management; 12–72 hours	Acute Management After 72 hours	NaPB 17th day (Discharging)	NaPB 3 months	NaPB 6 months	NaPB 12 months
Leu (mg/day)	336	360	0	360	360	390	420	450
Isoleu (mg/day)	161	298	300	290	290	342	390	410
Valine (mg/day)	158	287	300	280	280	300	320	330
Protein (gr/kg/day)	2,7	2,5	2,5	2,9	2,95	2,8	2,85	2,92
Energy (Kcal/day)	110 kcal/kg/day	1198	1400	1300	1450	1580	1620	1700