Benign recurrent intrahepatic cholestasis

Benign recurrent intrahepatic cholestasis (BRIC) is a rare cause of cholestatic jaundice in children and young adults.Jaundice recur throughout life but does not lead to chronic liver disease or cirrhosis.It is an autosomal recessive disorder characterised by profound elevation of alkaline phosphatase with normal gamma glutamyl transferase activity.Gene responsible for BRIC is located in chromosome 18.Genetic studies have demonstrated that this disorder is a result of mutation of ATP8B1,which is involved in the transport of bile in the biliary canaliculi. Treatment is mostly symptomatic. Very few cases have been reported till date.Here we report a 24 year old female ,who had recurrent episodes of jaundice since 2 years of age . Keyword : BRIC,cholestasis,jaundice,cirrhosis Case report 24 years old female presented with history of yellowish discolouration of sclera , intense pruritus and hyperpigmentation of skin all over the body 3 months. Patient had first episode of jaundice at the age of 2 years which lasted for one month.Second episode at the age of 14 years which lasted for two months. During each episode patient was admitted in hospital and discharged .She was asymptomatic in between the episodes. No family history of liver disease.No history of ingestion of toxins or drugs.