Faculty Bibliography

Caroli's disease is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts that, when progressive, leads to intrahepatic stones, recurrent cholangitis, portal hypertension, cholangiocarcinoma, and liver failure. Liver transplantation is a promising curative option for advanced Caroli's disease. The aim of this study was to determine the outcomes of liver transplantation in unselected patients with Caroli's disease and recommend an evidence-based therapeutic algorithm for the management of Caroli's disease. Of the 78,124 patients transplanted in the United States between 1987 and 2006, 104 had Caroli's disease; 96 of these underwent liver alone, and 8 underwent combined liver/kidney transplantation. The patient survival and graft survival were analyzed by Kaplan-Meier survival analysis, and risk of death and risk of graft loss were analyzed by Cox proportional hazards regression. The overall 1-, 3-, and 5-year graft (79.9%, 72.4%, and 72.4%) and patient (86.3%, 78.4%, and 77%) survival rates were excellent for patients after liver transplantation. For combined liver/kidney transplantation (n = 8), the 1-year patient survival and graft survival were 100%. Proportional hazards analysis identified Asian ethnicity, elevated bilirubin, requirement of life support or hospitalization prior to transplantation, and a cold ischemia time greater than 12 hours as associated with increased risk of both graft loss and death. A history of prior transplant or prior abdominal surgery was also associated with increased risk of graft loss. In conclusion, liver transplantation is an excellent treatment option for patients with advanced Caroli's disease and should be considered in a timely fashion to prevent worsening complications including refractory cholangitis and cholangiocarcinoma.

Elderly liver donors (ELDs) represent a possible expansion of the donor pool, although there is great reluctance to use ELDs because of reports that increasing donor age predicts graft loss and patient death. The goal of this study was to identify a subgroup of recipients who would be least affected by increased donor age and thus best suited to receive grafts from ELDs. A national registry of deceased donor liver transplants from 2002-2005 was analyzed. ELDs aged 70-92 (n = 1043) were compared with average liver donors (ALDs) aged 18-69 (n = 15,878) and ideal liver donors (ILDs) aged 18-39 (n = 6842). Recipient factors that modified the effect of donor age on outcomes were identified via interaction term analysis. Outcomes in recipient subgroups were compared using Kaplan-Meier survival analysis. Recipients preferred for ELD transplants were determined to be first-time recipients over the age of 45 with body mass index <35, non-status 1 registration, cold ischemic time <8 hours, and either hepatocellular carcinoma or an indication for transplantation other than hepatitis C. In preferred recipients, there were no differences in outcomes when ELD livers were used (3-year graft survival: ELD 75%, ALD 75%, ILD 77%, P > 0.1; 3-year patient survival: ELD 81%, ALD 80%, ILD 81%, P > 0.1). In contrast, there were significantly worse outcomes when ELD livers were used in nonpreferred recipients (3-year graft survival: ELD 50%, ALD 71%, ILD 75%, P < 0.001; 3-year patient survival: ELD 64%, ALD 77%, ILD 80%, P < 0.001). CONCLUSION: The risks of ELDs can be substantially minimized by appropriate recipient selection.

Informatics initiatives, including on-line discussion boards, increase the accessibility of health care information and may strengthen patient and caregiver levels of satisfaction with health care. In 2005, 95% of the living organ donors in the U.S. were living kidney donors. Care of the donor frequently falls to the spouse/companion and has been reported as extremely stressful. The proposed study seeks to better prepare the caregiver through the use of online information- discussion board interaction.