Serum bile acid concentrations in the course of benign recurrent intrahepatic cholestasis

The patient reported here represents the classic features of the syndrome of benign recurrent intrahepatic cholestasis (BRIC). She had conjugated hyperbilirubinaemia during the neonatal period and early infancy. The concentrations of serum primary bile acids, cholic acid (CA) and chenodeoxycholic acid (CDCA) were determined by radioimmunoassay and were continuously extremely raised during the icteric, recovery, and also the anicteric phases of the disease. The ratio of CA to CDCA was always within the normal range. The secondary serum bile acid, deoxycholic acid (DCA), was only slightly raised. The oral cholate tolerance test was abnormal in the patient during the anicteric phase. These observations support the suggestion that a disturbance of the hepatocellular bile acid transport may be the primary defect in BRIC.