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[The value of plasma exchange during flare-ups of benign recurrent intrahepatic cholestasis]
F Sanderson1, J F Quaranta, E Cassuto-Viguier
1Clinique Néphrologique, Hôpital Pasteur, Nice.
Abstract:
Benign recurrent intrahepatic cholestasis (BRIC) or Summerskill's disease is a rare affection characterized by recurrent episodes of cholestasis of no apparent cause, separated by totally asymptomatic remissions. No specific treatment exists for the disease, and plasma exchange (PE) was proposed for disabling episodes affecting the general condition of two patients: one by two sessions of 4 and 13 exchanges during two episodes respectively (mass exchanged 14 and 35.5 l) and the other by 3 exchanges (total exchanged 4.5 l) during one episode. Improvement in clinical symptomatology and shortening of duration of two cholestatic episodes was noted when PE was carried out at an early stage. Bile acids were purified satisfactorily (42 to 52%), 0.15 to 1.17 mmole being extracted at each acute attack. Although difficult to assess in a rare affection such as this, PE appears to contribute to the symptomatic treatment of BRIC acute episodes if performed early enough, shortening their duration by the probable purification of circulating factors that are cholestatogenic as in primary biliary cirrhosis.