IS AN ACUTE DISTURBANCE IN HEPATIC TRANSPORT OF BILE-ACIDS THE PRIMARY CAUSE OF CHOLESTASIS IN BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS?

Serum and urinary bile-acids were measured during three episodes of cholestasis in an 8-year-old girl with benign recurrent intrahepatic cholestasis. In the very early phase of cholestasis, serum bile-acid levels were extremely high, while the serum-bilirubin level was still normal. The early phase was accompanied by very severe pruritus. The discrepancy between serum-bile-acids and serum-bilirubin suggests an acute interruption of the enterohepatic circulation of bile-acids; it also indicates that a deficiency of an enzyme involved in the transport of bile-acids by the liver cell might possibly be the primary cause of the cholestasis.