Chronic renal failure and portal hypertension - is portosystemic shunt indicated?

We report two girls with histories of recessive polycystic kidney disease. Both were on maintenance hemodialysis. They had undergone surgical distal p ortocaval shunt because of portal hypertension. Later, bilateral nephrectom y was per-formed, and they presented with hepatic encephalopathy (HE) and e volution towards irreversible hepatic coma and death. Portosystemic shunt i s the treatment of choice of portal hypertension. The kidney plays a pivota l role in ammonia disposal during portosystemic shunt. Thus, we stress the risk of HE after portosystemic shunt followed by bilateral nephrectomy in p atients with end-stage renal failure and suggest that combined liver-kidney transplantation should be considered.