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Utterson, E. C.1; Shepherd, R. W.1; Sokol, R. J.2; Bucuvalas, J. C.3; Rosenthal, P.4; Schneider, B.5; McDiarmid, S. V.6; Anand, R.7; Zeng, L.7The SPLIT Research Group

Journal of Pediatric Gastroenterology and Nutrition: June 2004 - Volume 39 - Issue - p S27
ABSTRACTS: Oral Presentation Abstracts

1Pediatric Gastroenterology and Nutrition, Washington University School of Medicine, St. Louis,2Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Denver,3Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children’s Hospital, Cincinnati,4Pediatric Gastroenterology, Hepatology and Nutrition, University of California, San Francisco,5Pediatric Gastroenterology, Hepatology and Nutrition, Mount Sinai Medical Center, New York,6Pediatric Gastroenterology, Hepatology and Nutrition, University of California-Los Angeles Medical Center, Los Angeles,7The SPLIT Research Group, The EMMES Corporation, Rockville, United States

Submitted by:

Introduction: Biliary Atresia (BA) is a progressive cholangiopathy which, if untreated, leads to death by 2yrs of age. Liver transplant (LT) remains the only ultimate treatment for most patients. Once BA patients have been listed for LT, limited data exist regarding their potential for dying on the waiting list, waiting time, antecedents of early post-LT morbidity and survival.

Methods: We analyzed data from 755 cases of BA listed for first LT (1995–2003) from the Studies of Pediatric Liver Transplantation (SPLIT) database, a 39 center North American consortium.

Results: Most patients were female with 70% <1yr at the time of listing. Of the 755 patients listed, 567 have had LT and 24 have died while waiting. The probability of survival on an intent-to-treat basis from listing was 93% and 86% at 6m and 3yr, respectively. The overall probability of receiving a LT was 44% and 80% at 3 and 12 months post-listing, respectively. Between listing and LT, nutrition and growth declined. Once receiving a LT, patient and graft survival were 92% and 86%, respectively, at 6m. Tacrolimus was superior to cyclosporine in univariate analyses. Multivariate analysis revealed 3 significant risk factors for death, including recipient age<1yr, ht/wt<−2SD and donor age>50yr. Graft failure risks included ht/wt <−2SD, , the use of cadaveric partial donor organs, and donor age >50yr. 86% had a KP prior to transplant; however, KP was not a predictor for survival. Rejection occurred in 34% by 1m and 48% by 6m. Bacterial, viral and fungal infection occurred <30d following LT in 40%, 12% and 9%, respectively.

Conclusion: In conclusion, aggressive pre-LT management of complications of BA should be pursued, particularly in infants. Patient and graft survival improvements may come from enhanced pre-LT nutrition and growth, care with donor selection, use of tacrolimus and prevention or aggressive treatment of infection. Further analysis of the outcomes of specific BA subtypes, the influence of KP timing and the intent-to-treat data is warranted.

© 2004 Lippincott Williams & Wilkins, Inc.