Hepatic ultrastructure in congenital total lipodystrophy with special reference to peroxisomes.

The liver of an 8-year-old boy with congenital total lipodystrophy was investigated by means of catalase cytochemistry and morphometry. Comparison was made with eight human control livers. Light microscopy revealed cirrhosis and steatosis. Ultrastructural changes included lipid droplets with lamellae in the periphery, cup-shaped mitochondria, and nuclear pseudoinclusions. Peroxisomes were significantly increased in number but were not enlarged; they displayed various shapes and showed a moderate heterogeneity in catalase activity. A correlation between increased lipids and peroxisomal proliferation is suggested.