Characterization of urinary bile acids in a pediatric BRIC-1 patient: Effect of rifampicin treatment

Successful treatment with 4‐phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption

“…However, a reduction in the number and duration of the attacks is highly desirable, because intractable itching accompanied by cholestasis disrupts patients’ activities of daily living and their ability to sleep, making the patients disabled and depressed, and consequently decreases their quality of life and that of their families. There are case studies of medical therapy for cholestatic attacks in BRIC patients with rifampicin, cholestyramine and corticosteroids, and of invasive therapy with nasobiliary drainage (NBD) . However, all of these treatments are symptomatic therapy only, because of the lack of understanding of the molecular mechanism underlying intrahepatic cholestasis in BRIC, and inconsistent results have been reported for each treatment …”

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report

“…It also induces UGT1A1 and ABCC2, which conjugate and excrete bilirubin [17]. In episodes of BRIC, rifampicin therapy leads to reduced symptoms, improved biochemical parameters, and termination of episodes and hence is considered first-line therapy [4,10,[18][19][20].…”

Progressive Familial Intrahepatic Cholestasis

“…Some specimens show hepatocellular cholestasis and cholate injury, mostly centrilobular. During the asymptomatic period, all parameters (clinical, laboratory and liver histology) are normal [49].…”