Alpha-1-antitrypsin deficiency: outcomes after liver transplantation.

Alpha-1-antitrypsin deficiency (AAT) is the most common inherited metabolic disease leading to liver transplantation (LT) in children and adults. The aim of the study was to determine transplantation trends and survival of LT recipients with AAT. Using the UNOS (United Network for Organ Sharing) database, we identified 567 patients who underwent LT and 3 who received lung and LT from 1995 to 2004. AAT accounted for 1.06% of all adult LTs and 3.51% for pediatric LT. The 1-, 3-, and 5-year patient survival was 89%, 85%, and 83%, respectively, for adults versus 92%, 90%, and 90% for pediatric patients (P = .04), and graft survival was 83%, 79%, and 77% for adults versus 84%, 81%, and 78% for pediatric patients (P = .51). By regression analysis, age was the only predictor for patient survival (P = .04). In conclusion, adult and pediatric LT recipients with AAT are predominantly of Caucasian ethnicity and have an excellent post-LT survival.

Outcomes in adult and pediatric liver transplantation among various ethnic groups.

BACKGROUND: The reported patient and graft survivals among adults post-orthotopic liver transplantation (OLT) are variable, with an apparent discrepancy between ethnic groups. The aim of this study was to evaluate the impact of ethnicity on patient and graft survivals among adult and pediatric patients. METHODS: A retrospective analysis from the UNOS/OPTN databank between January 1995 and December 2006 was performed on adult and pediatric liver transplant recipients. Patients were divided into 4 groups based on ethnicity: African Americans, Hispanic, Caucasians, and other. Kaplan-Meier (KM) analysis was used to calculate patient and graft survival. Log-rank tests were used to compare survival rates between groups. RESULTS: In our study 42,710 OLT patients were included in the analysis, 90% of whom were adults. Of the 38,639 adult recipients, 29,432 (76.1%) were Caucasian, 4369 (11.3%) were Hispanic, 2963 (7.7%) were African American, and the remaining 1875 (4.9%) were of other ethnicities. KM estimates and Cox regression analyses demonstrated that there was a significant ethnic difference in both patient and graft survivals at 1, 3, 5, and 10 years. African Americans showed a lower rate (P<.001). Of the 4341 pediatric recipients, 2461 (56.7%) were Caucasian, 797 (18.4%) were Hispanic, 824 (18.9%) were African American, and the remaining 259 (5.9%) were of other ethnicities. Unlike the adults, there were no significant differences among ethnic groups in terms of patient (P=.31) and graft (P=.33) survival at 1, 3, 5, and 10 years after OLT. CONCLUSION: These results showed that adult African American OLT patients have a reduced transplantation rate and a worse survival rate when compared with other ethnicities in the adult but not in the pediatric population. This information suggests that further studies are indicated to identify the causes of racial differences in transplant access and outcomes in the adult patient population.

Long-term analysis of primary nonfunction in liver transplant recipients.

UNLABELLED: Long-term allograft and patient survival following liver transplantation continues to improve with the development of new surgical techniques and immunosuppressive agents. Complications such as primary nonfunction (PNF) have not been well characterized in terms of long-term allograft and patient survival. The aim of this study was to determine the incidence of PNF in liver transplant recipients and patient and graft survival, in addition to identifying temporal trends in these parameters. METHOD: Data were obtained from the United Network for Organ Sharing/Organ Procurement and Transplant Network for all adults (>18 years old) who received a deceased donor liver transplant between January 1990 and December 2004. RESULTS: Of the 58,576 liver transplant recipients, 2061 had PNF, an overall incidence of 3.5%. There was a 30% annual increase in the incidence of PNF between 1990 and 2000; the incidence of PNF peaked at 7%, and then decreased by 20% annually thereafter. No differences in donor and perioperative variables were identified to account for this variation. One-, 3-, and 5-year patient and graft survival for patients with PNF who underwent retransplant were significantly lower than those with primary liver transplant. In conclusion, there has been decreased incidence of PNF among liver transplant recipients in the last decade.

Analysis of hospitalizations comparing rifaximin versus lactulose in the management of hepatic encephalopathy.

INTRODUCTION: Patients with end-stage liver disease often develop hepatic encephalopathy. The loss in cognitive abilities results in marked economic loss to the patient and health care community. We report hospital admission rates and economic impact of patients with end-stage liver disease suffering from hepatic encephalopathy. METHODS: The medical records were reviewed involving liver transplant patients started on lactulose or rifaximin therapy after presenting with stage 2 hepatic encephalopathy from January 2004 to November 2005. Information collected included demographics, hospitalizations required for hepatic encephalopathy, economic data, and Model for End-stage Liver Disease (MELD) score. RESULTS: Thirty-nine patients met study criteria: 24 patients treated with lactulose (group one) and 15 with rifaximin (group two). Group one included 18 men and six women of mean age 48 (range 39 to 58), average MELD 14 (range 10 to 19). Group two included 10 men and five women of mean age 47 (range 42 to 58), average MELD 15 (range 10 to 19). Group one patients required 19 hospitalizations overall: three patients with three hospitalizations, four patients with two hospitalizations, and two patients required one hospitalization. Total drug cost per month was 50 dollars(group one) and 620 dollars(group two). The average annual cost of hospitalization, emergency room visit, and drug per patient treated was 13,284.96 dollars for a total of 318,839 dollars (range 5005 dollars to 26,255 dollars, including drug cost and hospital care). Group two required three hospitalizations, all three with one visit. The average annual cost of hospitalization, emergency room visit, and drug per patient treated was 7958.13 dollars for a total of 119,372 dollars (range 6005 dollars to 19,255 dollars, including drug cost and hospital care). The total cost of therapy per patient per year was 13,285 dollars (group one) versus 7958 dollars (group two). The average length of stay was shorter in group two [3.5 days (range 3 to 4)] versus group 1 [5.0 days (range 3 to 10); P < .0001]. CONCLUSION: These pilot data demonstrate the marked difference in economic costs for the treatment of hepatic encephalopathy. The results also show that in comparative groups, the economic gains are quickly lost when using lactulose.

The Leros PIKPA asylum. Deinstitutionalisation and rehabilitation project.

A three-year deinstitutionalisation and rehabilitation pilot intervention project was implemented at Leros PIKPA for people with severe learning disabilities. Initial conditions at the asylum were appalling. Residents suffered severe deprivation, extreme institutionalisation, and violation of basic human rights. Intervention involved professionals from different disciplines, and involved residents, their families, care staff, the institution, and the local community. As a result, resident care and adaptive behaviour has started to improve. Communication between residents and families has increased. Owing to training and sensitisation, care staff's poor resident-management practices and negative attitudes toward disabled people have changed. Living and hygienic conditions have been upgraded and building renovation is under way. Asylum administration and the local community have been sensitised to residents' needs. Eleven residents have moved to the project's pilot community home in Athens; two others now live with foster families. The results suggest that deinstitutionalisation and rehabilitation can be successfully initiated even in residential institutions of the severest kind.