Table 1.

The characteristics of liver transplant recipients with ethylmalonic encephalopathy before and after liver transplantation

Ref.	Case number	Gender	Age at diagnosis	Pre-LT dietary restriction	Pre-LT medical treatment	Age at LT in mo	Type of graft	Post-LT dietary restriction	Post-LT medical treatment	Follow-up in mo	Neurodevelopmental outcomes
Dionisi-Vici et al[3] 2016	1	Female	7 mo	N/A	Metronidazole, N-acetylcysteine	9	LDLT	N/A	Carnitine, metronidazole	12	Striking neurological improvement, remarkable achievements in psychomotor development.
Tam et al[11] 2019	2	Female	13 mo	N/A	Metronidazole, N-acetylcysteine	19	DDLT	N/A	Carnitine, metronidazole, N-acetylcysteine	9	Drastically improved social interaction and language.
	3	Male	8 wk	N/A	Metronidazole, N-acetylcysteine	13	OLT	N/A	Metronidazole, N-acetylcysteine	22	No other encephalopathic episodes and slow developmental progress.
Present case	4	Male	9 mo	Yes (start from 15 mo)	Carnitine, metronidazole, (start from 15 m)	18	LDLT	No	Carnitine, metronidazole (stop 2 mo post-LT)	20	No obvious improvement.

DDLT: Decreased donor liver transplantation; N/A: Not available; LT: Liver transplantation; LDLT: Living donor liver transplantation; OLT: Orthotopic liver transplantation.