Case Report

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A 53-year-old woman with a 10-year history of primary biliary cirrhosis presented with pruritus and stage I primary biliary cirrhosis as defined by Scheuer's histologic criteria. She had been treated for 7 years with various therapies, including cholestyramine, ursodeoxycholic acid, penicillamine, and colchicine. In June 1992, her total bilirubin level was 4.275 µmol/L (0.25 mg/dL). She had been treated unsuccessfully with methotrexate or rifampin or both and had developed clinical evidence of the hepatorenal syndrome with increasing ascites and hepatic encephalopathy. According to the Mayo model for predicting survival of patients with primary biliary cirrhosis, the patient's prognostic index was 9.0 in April 1993, 9.5 in July 1993, and 9.9 in October 1993 [6]. She was believed to be in the terminal stage of her disease; thus, after she and her family gave informed consent, an LRD liver transplantation was done using the left lobe of the liver of her 25-year-old son. The normal liver volume for a person of the patient's size was calculated to be 970 mL [7]. Volumetric analysis of a computed tomographic scan of the son's liver showed that the left lobe had a volume of only 434 mL; this was only 45% of the ideal liver volume estimated for the recipient [8]. The orthotopic LRD liver transplantation was done on 2 November 1993 at Shinsyu University Hospital.

Clinical Course

The resected left lobe of the donor weighed 404 g, or 42% of the ideal mass estimated for the recipient. In the intensive care unit, the patient had high blood glucose levels, due in part to the administration of FK506 and steroids, and severe ascites due to lymphorrhea from the donor liver. After 2 weeks in the intensive care unit, the patient's hepatic encephalopathy was alleviated and her liver function had improved dramatically (at day 7, the total bilirubin level was 124.83 µmol/L [7.3 mg/dL] and the aspartate aminotransferase (AST) level was 0.50 µkatal/L (30 IU/L); at day 14, the total bilirubin level was 104.31 µmol/L (6.1 mg/dL) and the AST level was 0.63 µkatal/L (38 IU/L); and at day 30, the total bilirubin level was 25.65 µmol/L (1.5 mg/dL) and the AST level was 0.52 µkatal/L [31 IU/L]), and she had no surgical complications. After implementation of an immunosuppression regimen based on cyclosporine, marked regeneration of the donor liver occurred as each of these problems resolved. Three months after liver transplantation, the patient's body weight was the same as it had been before the disease. The donor had no postoperative complications and was discharged from the hospital after 10 days; he leads a normal life and has the same physical conditions he had before the operation.

Serial computed tomographic scans have shown adequate hepatic regeneration in both the donor and the recipient. The liver placed in the recipient had a volume of 900 mL after 17 days, a volume of 1150 mL after 30 days, and a volume of 1250 mL after 3 months; the volume then remained stable at 1250 mL for 6 months (Figure 1). In contrast, the recipient's splenic volume decreased progressively from 779 mL before the transplantation to 773 mL at 17 days, 370 mL at 3 months, and 350 mL at 6 months after LRD liver transplantation. Plasma levels of hepatocyte growth factor did not increase during the period of hepatic regeneration.

View larger version (141K): [in this window] [in a new window]   Figure 1. Computed tomographic scans of donor and recipient after living related-donor liver transplantation. 1) Donor's liver, 404 mg of left lobe; 2) 900-mL liver volume 17 days after transplantation; 3) 1150-mL liver volume 1 month after transplantation; and 4) 1250-mL liver volume 3 months after liver transplantation.

The HLA haplotype of the recipient was HLA-A2, A33, B46, B17, Cw1, Cw9, DR8.2, DR13, DQ1; the haplotype of the donor was HLA-A26, A33, B17, B62, Cw9, DR13, DQ1. These data indicate complete HLA class II matching between donor and recipient. A minor episode of rejection seen 1 month after transplantation responded immediately to a pulse of steroid therapy consisting of 1 g of methylprednisolone.

Before transplantation, the recipient had an antimitochondrial antibody titer of 1280 and was positive for anti-pyruvate dehydrogenase complex antibody (1.680, x 1000 dilution), and for anti-branched-chain ketoacid dehydrogenase complex antibody (0.637, x 1000 dilution). For 6 months after liver transplantation, all antibody markers in the recipient's serum were negative.

Discussion

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Data on clinical liver transplantation survival indicate that 6 months after liver transplantation, the survival curve of most recipients is identical to that of a normal, age-matched population and that it remains identical for 5 years [9]. In other words, most deaths that occur after liver transplantation occur within 6 months of the procedure. Six months after the transplantation reported here, donor and recipient have both completely recovered. We document that it is possible to successfully use either a cadaver or a living related donor for adult-to-adult liver transplantation in persons with end-stage disease and probably also in persons with fulminant hepatic failure.

It has been shown that the donor liver regenerates from a volume of approximately 40% to a mass sufficient for the recipient [8, 10]. This is important because it suggests that a partial liver can regenerate 2.5 times its volume after LRD liver transplantation with no special provisions other than the use of either FK506 or cyclosporine. From our results and those from a study of livers resected because of liver malignancy [5], we could speculate that at least one third or 40% of the ideal liver volume will support hepatic function in a recipient.

Conclusion

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We report a successful LRD liver transplantation from a 25-year-old donor to the donor's 53-year-old mother for primary biliary cirrhosis. Until now, the indications for LRD liver transplantation were reserved solely for children. We suggest that LRD liver transplantation can be done in adults with irreversible liver disease.