Table 4.

Biochemical differentiation between various metabolic hepatopathies (respiratory chain disorder vs non respiratory chain disorder comparison)

	Acidosis	Urine ketones	Blood sugar	Serum lactate	Serum ammonia
RCD	++	++	Normal	++++	±
FAOD	++	Nil (non-ketotic)	Low (hypoglycemia)	+	+
OA	+++ (persistent)	++/+++	Low/normal/high	Normal	++
UCD	Normal	Normal	Normal	Normal	++++

FAOD: Fatty acid oxidation defects; OA: Organic acidemias; RCD: Respiratory chain defects; UCD: Urea cycle defects.