The debut of benign recurrent intrahepatic cholestasis in acute hepatitis A

Abstract

Background. Benign recurrent intrahepatic cholestasis is a rare inherited disorder characterized by recurrent episodes of severe hyperbilirubinemia and pruritus that resolve spontaneously. However, attacks of cholestasis may persist for several months and in some cases be associated with frequent recurrences, which may be grounds for liver transplantation.Objective. To present a clinical case of debut benign recurrent intrahepatic cholestasis following acute hepatitis A.Results. A 30-year-old patient was admitted at the Liver Transplantation Center of Moscow Regional Research and Clinical Institute n.a. M.F. Vladimirskiy for a prolonged episode of intrahepatic cholestasis with severe coagulopathy after acute hepatitis A. Total bilirubin was elevated up to 835 µmol/L and INR was 3.6. The manifestations of vitamin K-associated coagulopathy were controlled after the first dose of parenteral menadione sodium bisulfite. Glucocorticosteroids, ursodeoxycholic acid and plasmapheresis turned ineffective in the treatment of hyperbilirubinemia. Due to long-persisting cholestasis resistant to conservative therapy, the patient was considered for inclusion to the liver transplant waiting list. However, spontaneous resolution of the cholestatic episode was achieved at 5 months after the onset of manifestations. Benign recurrent intrahepatic cholestasis type 2 was diagnosed on the basis of the specific clinical signs, laboratory blood tests and genetic testing.Conclusion. The present Case Report shows a long-lasting episode of cholestasis with severe coagulopathy in acute hepatitis A in a patient with benign recurrent intrahepatic cholestasis with subsequent spontaneous resolution of the clinical symptoms at 5 months after their manifestation onset. Therefore, the differential diagnosis of benign recurrent intrahepatic cholestasis should be considered prior to liver transplantation in patients with intrahepatic cholestasis.