Hyperchloremic Metabolic Acidosis With Cholestyramine Therapy for Biliary Cholestasis

Cholestyramine is a hydrophilic, insoluble anion-exchange resin that removes substantial amounts of bile acids from the enterohepatic circulation by binding them in a compound that cannot be reabsorbed through the intestine. It has been used without complication in children with biliary cholestasis associated with biliary atresia¹ and in the treatment of intractable diarrhea in infants.^2.3 The case reported here deals with hyperchloremic acidosis as a complication of cholestyramine therapy in a 6-month-old infant with intercurrent infection, and adds to other reports of this finding by Hartline,⁴ Primak and McGurk,⁵ and Kleinman.⁶

Report of a Case.—The patient was first referred to the pediatric consultation service at Mt Zion Hospital in San Francisco at the age of 10 weeks, when a diagnosis of intrahepatic cholestasis secondary to intrahepatic biliary atresia was made and confirmed by open liver biopsy. Cholestyramine therapy was begun six weeks prior to this