Xanthomatous Biliary Cirrhosis

Abstract

Summary A case of xanthomatous biliary cirrhosis has been presented. Unusual features included xanthomatous deposits in the right cornea and iris, severe demineralization of the vertebrae and bones of the extremities, leading to pain, fractures, and deformity with diminished stature. Serum remained clear despite elevated total cholesterol and total lipids. The phospholipid fraction was extremely high and the neutral fat relatively low. The alkaline phosphatase remained extremely high during the entire illness. Early in the disease albumin-globulin ratio, cephalin-cholesterol flocculation, thymol turbidity and cholesterol ester ratios were variable; late in the disease, these determinations consistently showed abnormality, indicating severe liver impairment. Treatment with a low-fat, cholesterol-free and low caloric diet combined with lipotropic agents resulted in a moderate decrease of the serum cholesterol levels and in disappearance of some of the xanthomatous lesions. This treatment, however, did not prevent increased endogenous production of cholesterol; it did not interfere with production of pericholangiolar fibrosis, and it did not alter the course of the disease sufficiently to prevent a fatal outcome.