BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS (BRIC): IS IT REALLY AS BENIGN AS ANTICIPATED?

Abstract

We report an 18 year old male patient with a known diagnosis of BRIC who presented with acute renal failure secondary to hyperbilirubinemia In three successive episodes. Renal replacement therapy was required in all three episodes but his renal function recovered to baseline creatinine on discharge. Proposed pathophysiology of ARF in the setting of hyperbilirubinemia includes direct tubulotoxlcity and sequestration of pigment casts within the tubular lumen causing tubular obstruction aggravated by dehydration. We emphasize the importance of vigorous hydration to be started with the impending attack to prevent progression to ARF.
Key Words : Acute Renal Failure, BRIC

References

1. Van Berge Henegouwen OP. Benign recurrent intrahepatic cholestasis and Byler's disease: one gene, two diseases?J Hepatol 1996; 25:395-397.
2. Schapiro RH, isseibacher lJ. Benign recurrent intrahepatic cholestasis. H Eng J Med 1963; 268:708-71 I.
3. Green J, Better OS. Systemic hypotension and renal failure in obstructive jaundice: Mechanistic and therapeutic aspects. J Am Soc Mephrol 1994;4:1 735-1 753.
4. CorateiH P, Passavanti Q. Pathophysiology of renal failure in obstructive jaundice. Miner Electrolyte Metab 1990; 16:61 -65.
5. Griffin MD, Grande JP, Wiesner RH, et al. Prolonged anuria complicating primary sclerosing cholangitis: successful outcome following orthotopic liver transplantation. Am J Kidney Dis 1998;31:360-363.
6. Yuceyar Y, Gumustas K, Erturk S, et al. The role of oxygen free radicals in acute renal failure complicating obstructive jaundice; an experimental study. HPB Surg 1998; 10:387- 393.