Cholestasis- benign recurrent intrahepatic 1
Overview
Benign recurrent intrahepatic cholestasis (BRIC) a rare form of hereditary cholestasis syndrome characterized by repeated self limited episodes of pruritus and jaundice. It recurs but occasionally leads to progressive liver disease. Treatment is symptomatic
Diagnosis
Diagnosis of BRIC was made on the basis of clinical and laboratory parameters. He was treated symptomatically and he improved within 4 weeks with normalization of liver function tests. The child was asymptomatic on 1 year follow up.
Prognosis
chronic recurrent illness but no progression to hepatocellular failure or cirrhosis
Treatment
No specific treatment that could either prevent or limit the duration of attacks is known. The key to treatment is therefore relief of severe pruritus and other symptoms until attacks resolve spontaneously.