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 evidencebased 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 ϭ 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.

The reported mortality rates in patients with cirrhosis undergoing various non-transplant surgical procedures range from 8.3% to 25%. This wide range of mortality rates is related to severity of liver disease, type of surgery, demographics of patient population, expertise of the surgical, anesthesia and intensive care unit team and finally, reporting bias. In this article, we will review the pathophysiology, morbidity and mortality associated with non-hepatic surgery in patients with cirrhosis, and then recommend an algorithm for risk assessment and evidence based management strategy to optimize postsurgical outcomes. © 2007 WJG . All rights reserved.Key words: P r e -ope r at ive r is k as s e s s me nt ; Ris k stratification; Cirrhosis; Model for end-stage liver disease; Non-transplant surgery; Outcomes Millwala F, Nguyen GC, Thuluvath PJ. Outcomes of patients with cirrhosis undergoing non-hepatic surgery: Risk assessment and management. World J Gastroenterol 2007; 13(30): 4056-4063

Previous studies have documented that sustained virologic response (SVR) is significantly reduced in African Americans with chronic HCV genotype 1 following treatment with interferon and ribavirin when compared with Caucasians. The specific aim of the present retrospective study was to assess virologic response to interferon and ribavirin in African Americans with HCV genotypes 2 and 3. A review of our database identified 42 African Americans and 334 Caucasians with HCV genotypes 2 and 3. Patients coinfected with hepatitis B or human immunodeficiency virus, chronic renal failure, and recipients of an organ transplant were excluded. Thirty of the African Americans were treated with either standard interferon or peginterferon and ribavirin as initial treatment for chronic HCV. Ninety of the 334 Caucasians were matched to the African Americans with regards to genotype, cirrhosis, treatment regimen, sex, age, and body weight for comparison of virologic response. The proportion of patients with HCV genotype 2 was significantly greater (P < 0.001) in African Americans compared with Caucasians (81%vs 52%). End-of-treatment virologic response was observed in 94% of Caucasians compared with 80% in African Americans (P= 0.036). SVR was observed in 82% and 57% of Caucasians and African Americans, respectively (P= 0.012). Similar results were observed when patients who had been treated with only peginterferon and ribavirin were assessed. These results suggest that African Americans have a global defect in their ability to eradicate HCV infection following treatment with interferon and ribavirin which transcends across all genotypes.