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We prospectively collected data on 1,429 liver transplant recipients between December 1984 and December 1998. A utoimmune hepatitis (AIH) is a recognized pathological process first described in the 1950s. However, diagnostic criteria have been clearly defined only recently. 1 AIH affects predominantly middle-aged women and often responds to immunosuppressive therapy. When it leads to chronic liver failure, it constitutes an indication for orthotopic liver transplantation (OLT). 1 Because of post-OLT immunosuppressive therapy, recurrence in liver allografts has been believed to be relatively low. 2,3 This has been supported by reports from both American and European centers 4 dating back to the mid-1980s. Although these studies made use of histological and biochemical parameters for their diagnosis of recurrence, precise patterns of histopathologic findings were not described. In addition, hepatitis C virus infection was not completely excluded because a marker of viremia was not yet available. 5 In this study, we reviewed our experience using OLT for the treatment of AIH. We analyzed the incidence of recurrence and possible risk factors associated with recurrence. We also studied the impact of AIH recurrence on patient and graft survival, HLA typing, episodes of rejection, liver function test (LFT) results, and other biochemical and histological parameters. Materials and MethodsData were collected prospectively between December 1984 and December 1998 on a total of 1,429 patients who underwent OLT at Baylor University Medical Center (Dallas, TX). There were 753 men and 676 women. Mean age at OLT was 48 Ϯ 12 (SD) years. The initial diagnosis of AIH was based on: (1) detection of antinuclear antibodies, anti-smooth muscle antibodies, or antiliver/kidney microsomal type 1 antibodies in titers greater than 1:80; (2) total gamma globulin or immunoglobulin G (IgG) levels greater than 1.5 times the upper limit of normal; (3) absence of IgM antibodies to hepatitis A virus; (4) absence of hepatitis B virus surface antigen and antibodies to hepatitis B virus core antigen; (5) absence of antibodies to hepatitis C virus and hepatitis C virus RNA by polymerase chain reaction assay; (6) laboratory findings of abnormal LFT results (alanine aminotransferase [ALT] or aspartate aminotransferase [AST]); (7) absence of liver disease attributable to alcohol or hepatotoxic drugs; and (8) presence of histological findings consistent with interface hepatitis or piecemeal necrosis, concurrent lobular hepatitis, and no biliary lesions or other changes.Biochemical and clinical data were collected preoperatively and at various times after OLT. All transplant recipients

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who ...

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The purpose of our study was to evaluate the outcome of children who underwent liver transplantation as treatment for unresectable hepatoblastoma. We prospectively collected data on 311 consecutive liver transplants performed at Children's Medical Center of Dallas between October 1984 and November 2000. There were nine recipients (five boys, four girls) with a diagnosis of unresectable hepatoblastoma. Postoperative survival of those currently alive ranged from 6 months to 16 years (mean 6.4 years, median 7.7 years). All recipients received preoperative chemotherapy; 67% received postoperative chemotherapy. Mean AFP level prior to transplantation was 1 448 000 ng/mL. Mean age at diagnosis was 0.81 years. Mean age at transplantation was 1.87 years. Only two patients experienced acute cellular rejection in the postoperative period. There was a total of three deaths and one recurrence. The only instance in which AFP levels did not decrease to low or undetectable levels post-transplantation was in the patient with recurrent tumor. Liver transplantation has an established role in the treatment of hepatoblastoma. It accounted for 3% of pediatric liver transplants, and provided the only opportunity for survival in otherwise incurable patients. Early diagnosis and treatment were found to be associated with better results. Response to chemotherapy may be an important factor influencing survival. Rising AFP levels after transplantation are associated with recurrence.