Table 2.

Leucine plasma levels, treatments, and outcomes.

Patient	Case	Reason for treatment	Leucine levels (μmol/L)			IV BCAA-free treatment duration (days)	IV BCAA-free dose (g/kg/day)	Hospital ization (days)	Concomitant treatments during DE	Clinical notes
			Admission	End of IV BCAA-free treatment	Discharge
1	1	High leucine Plasma level, enteral feeding not possible	3296	199	38	8	2.75	30	– IV dextrose and lipids - Isoleucine - Valine - Thiamine - Alanine - HD/HF - Hypertonic saline and furosemide	Intestinal perforation before IV BCAA-free treatment. Perforation was resolved at discharge, with no sequelae.
	2	High leucine plasma level, surgery (2nd admission)	167	29	82	7	2.75	11	- IV dextrose and lipids - Isoleucine - Valine - Thiamine - Alanine	Metabolic acidosis prior to IV BCAA-free solution, which resolved at discharge, with no ongoing sequelae.
2	3	High leucine level	2218	198	236	3	0.5-1	42	- IV dextrose and lipids – Isoleucine - Valine - Thiamine - Alanine - HD/HF - Hypertonic saline and furosemide	Hospital discharge was delayed because of poor enteral tolerance and frequent vomiting despite good metabolic control; no ongoing sequelae at discharge.
3	4	Clinical symptoms	699	46	23	20	1.5	65	- IV dextrose and lipids - Isoleucine - Valine - Thiamine - Alanine - HD/HF. - Hypertonic saline and furosemide - Mannitol.	Persistent oral intolerance after DE with frequent vomiting, forcing repeated stopping of enteral feeding. Discharged on physician's recommendation with ongoing sequelae: slow normalization of neurological symptoms.
4	5	Clinical symptoms and high leucine levels	938	320	282	7	0.5	13	- IV dextrose and lipids - Isoleucine - Valine Thiamine - Alanine	Resolution of symptoms at discharge and no ongoing sequelae.
5	6	High leucine plasma levels, vomiting	953	343	175	3	0.7	14	- IV dextrose and lipids Isoleucine - Valine -Thiamine - Alanine	Resolution of symptoms at discharge and no ongoing sequelae.

HD/HF, haemodialysis/haemofiltration.