title: Immunodeficiency, xanthomas and obstructive liver disease.
abstract: 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 lipids and bile acids. This improvement may result from shifting the bile acid pool from the peripheral blood compartment to the enterohepatic circulation.
mesh_terms: D001647:Bile Acids and Salts / Q000378:metabolism; D002635:Chenodeoxycholic Acid / Q000378:metabolism; D002648:Child; D002792:Cholestyramine Resin / Q000494:pharmacology; D002793:Cholic Acids / Q000378:metabolism; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications; D006949:Hyperlipidemias / Q000150:complications*; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008107:Liver Diseases / Q000150:complications*; D008297:Male; D010634:Phenobarbital / Q000494:pharmacology; D011537:Pruritus / Q000150:complications; D014973:Xanthomatosis / Q000150:complications*
chemical_list: D001647:Bile Acids and Salts; D002793:Cholic Acids; D002635:Chenodeoxycholic Acid; D002792:Cholestyramine Resin; D010634:Phenobarbital