Extracorporal albumin dialysis (MARS) improves cholestasis and normalizes low apo A-I levels in a patient with benign recurrent intrahepatic cholestasis (BRIC).

[benign recurrent intrahepatic cholestasis]

The familial cholestatic diseases Benign Recurrent Intrahepatic Cholestasis (BRIC ) and Progessive Familial Intrahepatic Cholestasis type 1 (PFIC 1 ) are characterized by intermittent or permanently elevated plasma bile salt levels , therapy-resistant extreme pruritus and peculiar biochemical abnormalities including low apolipoprotein apo A-I . Previously , symptomatic improvement has been demonstrated in BRIC patients after extracorporal albumin dialysis (MARS ). We hypothesized that MARS improves cholestasis , induces changes in the bile salt profile and normalizes apo A-I serum levels in BRIC . A 17 -year -old-female patient with BRIC experienced an episode of cholestasis lasting for more than 6 months with extreme pruritus and diarrhoea not responding to standard therapy . During a period of five days the patient was treated 3 x 8 h with MARS . The procedures were well tolerated and resulted in reduction of plasma bile salts by 58 %. The plasma bile salt profile changed into a more hydrophilic composition after MARS . Diarrhoea discontinued and the pruritus improved significantly from 9 to 4 on a subjective scale . These effects lasted 4 months until a relapse occurred . Low plasma apo A-I levels (0 .52 g /l ) normalized after MARS (0 .98 g /l ). The procedures were well tolerated . Fatigue was noted as the only transient side-effect . In conclusion , MARS may induce a long -term symptomatic improvement and decrease of cholestatic markers in BRIC . Further studies evaluating efficacy and mechanism of MARS in patients with BRIC are needed .

Diseases
Validation

Diseases presenting "reduction of plasma bile salts" symptom

benign recurrent intrahepatic cholestasis

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