Table 1.

Treatment for CD.

Treatment	Target Disease	Concept	References
Low-carbohydrate diet	NICCD CTLN2	Basal therapy	[18,47,48]
MCT + lactose-restricted formula	NICCD CTLN2	Energy supply Correction of metabolic disorder	[53,54,55,56,57,58]
Sodium pyruvate	CTLN2	Energy supply Correction of metabolic disorder	[59,60,61,62]
Ursodeoxycholic acid	NICCD	Hepatoprotection Anti-cholestasis	[18,63,64,65]
Mannitol	NICCD CTLN2	Osmotic agent for treating brain edema due to hyperammonemia	[51]
Liver transplantation *	NICCD CTLN2	Ultimate fundamental therapy	[16,66,67,68,69]

* In Japan, liver transplantation is performed on recipients under 65 years old. CTLN-2, adult-onset type II citrullinemia; NICCD, neonatal intrahepatic cholestasis caused by citrin deficiency; MCT, medium-chain triglycerides.