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Four pediatric patients are presented in whom profound renal failure (hepatorenal syndrome) developed in association with severe end-stage liver disease. All four patients had successful orthotopic liver transplantation. Special emphasis is given to the preoperative and postoperative renal function in the patients, and the criteria used to establish the diagnosis of the hepatorenal syndrome are discussed. In the initial work on liver transplantation and reversal of the hepatorenal syndrome, two of the three patients recovered renal function but died in the perioperative period. The four patients presented in this report have not only had reversal of the hepatorenal syndrome after successful orthotopic liver transplantation but have also survived long term. The four patients have been followed up for periods ranging from 18 months to 4.5 years. Three of the four patients have maintained near normal renal function, whereas the fourth patient (who had a left nephrectomy for obstruction and sepsis) has had a significant decline in renal function.

The Reversal of the Hepatorenal Syndrome in Four Pediatric Patients Following Successful Orthotopic Liver Transplantation