Table 2.

Summary of the findings of nephropathy in the mitochondrial cytopathy and LBW-related nephropathy patients

	Normal	Mitochondrial cytopathy	LBW-related nephropathy
mtDNA mutation	No	Yes	No
FSGS lesion	No	Yes1	Yes
Glomerular hypertrophy	No	Yes or No	Yes
Foot process effacement	No	Yes	Yes
Increased mitochondria in podocyte	No	Yes	No
GSECs in tubules	No	Yes	Yes
COX IV expression in glomeruli	uniform	mosaic pattern	weak (but intense in a few podocytes)
COX IV expression in tubules	uniform	partially mosaic pattern	partially mosaic pattern

¹Although the two patients with mitochondrial cytopathy had no FSGS lesions in the glomeruli, the presence of FSGS lesions is a characteristic glomerular change in mitochondrial cytopathy patients, as reported in many previous reports.