Mitochondrial Disease as a Cause of Neonatal Hemophagocytic Lymphohistiocytosis

Table 2

Enzyme assay of the mitochondrial respiratory chain (I–IV).

(a) Liver

%	Co I	Co II	Co III	Co IV	CS
Patient
% of normal	15	31	78	100	108
CS ratio (%)	13	28	68	88
Co II ratio (%)	46		242	252

(b) Muscle

%	Co I	Co II	Co II + III	Co III	Co IV	CS
Patient
% of normal	17	54	57	51	35	63
CS ratio (%)	26	86	89	83	52
Co II ratio (%)	30		100	94	57

(c) Heart

%	Co I	Co II	Co II + III	Co III	Co IV	CS
Patient
% of normal	15	89	53	159	63	112
CS ratio (%)	14	79	46	142	57
Co II ratio (%)	26		76	220	46

This table shows the activity of the mitochondrial respiratory chain enzyme (I–IV) in the liver, muscle, and heart.
Co I: complex. Co II: complex II. Co III: complex III. Co IV: complex IV. CS: citrate synthase.