Immunodeficiency,xanthomas and obstructive liver disease

Chronic obstructive liver disease and secondary hyperlipidemia developed in an immunodeficient boy. Sequential addition of cholestyramine and phenobarbital to his medical regimen, following an initial response to bile drainage, resulted in the disappearance of xanthomas and pruritus, and the restoration of normal serum concentrations of liplds and bile acids. This improvement may result from shifting the bile acid pool from the peripheral blood compartment to the enterohepatlc circulation.