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Fulminant Hepatic Failure in Children: Superior and Durable Outcomes With Liver Transplantation Over 25 Years at a Single Center

Farmer, Douglas G. MD*; Venick, Robert S. MD; McDiarmid, Sue V. MD; Duffy, John P. MD*; Kattan, Omar BS*; Hong, Johnny C. MD*; Vargas, Jorge MD; Yersiz, Hasan MD*; Busuttil, Ronald W. MD, PhD*

doi: 10.1097/SLA.0b013e3181b480ad
Original Articles

Objective(s): Death occurs in half of all children with fulminant hepatic failure (FHF). Although liver transplantation (LT) is potentially life-saving, there are only a few published series with limited experience. The aim was to examine predictors of survival after LT for FHF.

Methods: Between 1984 and 2008, all LT for FHF performed in recipients less than or equal to 18 years of age were analyzed from a prospectively maintained database using 35 demographic, laboratory, and operative variables. Unique calculated variables included creatinine clearance (cCrCl) and Pediatric End-Stage Liver Disease score (PELD). Study end-points were patient and death censored graft survival. Median follow-up was 98 months. Statistical analysis involved the log-rank test and Cox proportional hazards model.

Results: A total of 122 children underwent 159 LTx. Cryptogenic was the primary etiology (70%) and the median age was 53 months. The significant (P < 0.05) univariate predictors of worse graft survival were: recipient age <24 months, cCrCl <60 mL/min/1.73m2, PELD >25 points, and warm ischemia time >60 minutes. The significant (P < 0.05) univariate predictors of worse patient survival were: recipient African-American and Asian race, recipient age <24 months, cCrCl <60 mL/min/1.73m2, and time from onset jaundice to encephalopathy <7 days. On multivariate analysis, survival was significantly impacted by 4 variables: cCrCl <60 mL/min/1.73m2 (GRAFT and PATIENT), PELD >25 points (GRAFT), recipient age <24 months (GRAFT), and time from onset jaundice to encephalopathy <7 days (PATIENT). While overall 5- and 10-year survival was 73% and 72% (GRAFT) and 77% and 73% (PATIENT), these were significantly worse when a combination of multivariate risk-factors were present.

Conclusions: This data from a large, single-center experience demonstrates that LT is the treatment of choice for FHF and results in durable survival. Analysis revealed 4 novel outcome predictors. Young children with rapid onset acute liver failure are a high-risk subpopulation. Unique to this study, cCrCl and PELD accurately predicted the end-points. This analysis identifies patient subpopulations requiring early aggressive intervention with LT.

Few studies address outcome predictors in children undergoing liver transplantation for acute liver failure. This study found 4 variables that statistically impacted patient and graft survival. These variables may be useful in medical decision making for these acutely ill children.

From the *Departments of Surgery and †Pediatrics, David Geffen School of Medicine at UCLA, Los Angeles, CA.

Supported by Joanne Barr Foundation, Dumont Foundation, and George T. Pfleger Foundation.

Reprints: Douglas G. Farmer, MD, Dumont-UCLA Transplant Center, 757 Westwood Plaza, Suite 8501, Los Angeles, CA. E-mail: dgfarmer@mednet.ucla.edu.

© 2009 Lippincott Williams & Wilkins, Inc.