[Benign recurrent intrahepatic cholestasis type-II--a rare cause of direct hyperbilirubinemia exacerbations with hepatic fibrosis].

[benign recurrent intrahepatic cholestasis]

Direct hyperbilirubinemia , may result from a variety of pathologies , including structural obstructions with biliary tract occlusions (as in cholelithiasis ), infections (e .g . hepatitis ) and genetic disorders (Rotor 's and Dubin-Johnson 's syndrome ). One of the less common and probably more frequently underdiagnosed causes is benign recurrent intrahepatic cholestasis (BRIC ). First described in 1959 , BRIC was further classified into two subgroups which differ in their pathogenesis and clinical manifestation . Both BRIC types originate from impaired function bile salt excretion from hepatocytes to the canaliculi which is mediated by the bile salt export pump (BSEP ) which is located on the hepatyocyte membrane , unevenly distributed within the membrane lipid bilayer . In BRIC type -I , a mutation impairs the asymmetrical distribution of BSEP . In BRIC type -II , a mutation occurs directly damaging the BSEP . Apart from cholestasis , clinical manifestations of BRIC type -I include extra -hepatic symptoms such as watery diarrhea , pancreatitis and hearing impairment . Patients with BRIC type -II present mainly with hepatobiliary disease such as colelithiasis . In the past , BRIC was conventionally considered to result in no more than canalicular cholestasis , however several reports have associated BRIC with fibrosis and porto -portal septa formation . Disease course may last between weeks and months , more common in females , at any age , and usually resolves spontaneously , although chronic liver disease has also been described . Treatment modalities range from expectant management , medication (cholestyramine , ursolit ) or even surgery (biliary bypass /liver transplantation ). This report describes a patient with BRIC type -II and reviews the relevant literature .

Diseases
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Diseases presenting "chronic liver disease" symptom

benign recurrent intrahepatic cholestasis

erythropoietic protoporphyria

primary effusion lymphoma

typhoid

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