Benign recurrent intrahepatic cholestasis in a Chinese girl

A 15 year old Chinese girl presented with features of benign recurrent intrahepatic cholestasis, confirmed by liver biopsies performed during attack and remission. Her only younger brother also has features of this syndrome. Serial biochemistry monitoring during a recent attack demonstrated that a rise in the serum bile acids preceded the clinical onset of symptoms and the rise in serum bilirubin and ductal enzymes by 8 weeks. Whether this earlier rise in serum bile acids is related to the pathogenetic mechanism of this syndrome remains to be elucidated.