Liver Transplantation for Children With Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Analysis of the UNOS Database
1Department of Pediatrics, Mount Sinai Medical Center, New York, NY
2Fordham University, New York.
Meeting: 2015 American Transplant Congress
Abstract number: 51
Keywords: Liver transplantation, Pediatric
Session Information
Session Name: Concurrent Session: Liver: Pediatrics
Session Type: Concurrent Session
Date: Sunday, May 3, 2015
Session Time: 2:15pm-3:45pm
Presentation Time: 2:51pm-3:03pm
Location: Room 115-C
Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are unknown in the PELD era.
Aim: To characterize the outcome of LT in children with AIH and to compare the outcomes to those of another immune mediated disease, PSC.
Methods: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database. Comparisons were made between patients transplanted for AIH vs. PSC as well as chronic AIH versus fulminant AIH.
Results: 174 children with AIH and 113 with PSC were transplanted in the study period. Characteristics of our sample are shown in Table 1. Forty-four (25.2%) children with AIH had fulminant hepatic failure (FHF). One and five year patient survival rates after LT of children with chronic compared to FHF were 97.7, 88.6 (p=0.013), and 93.8, 84.1 (p =0.46) respectively. One and five year graft survival rates of chronic versus FHF were 92.3, 86.4 and 84.6, 81.1 (p>0.05 for both). The one and five year patient and graft survival were not significantly different between AIH and PSC patients; patient survival: 95.4, 91.4 vs 97.3, 92.9% respectively, graft survival: 90.8, 83.9 vs 91.2, 83.2%. 19% of children with AIH and 17% of children with PSC lost a graft to recurrent disease.
Conclusion: Children with AIH and FHF had significantly lower patient survival rates and similar graft survival rates to children with chronic AIH. Children transplanted for AIH compared to PSC showed no significant differences in 1 and 5 year patient or graft survival. For both AIH and PSC, a significant percentage of children lose their grafts to recurrent disease.
| AIH | PSC | ||
| Mean (SD) or % | Mean (SD) or % | p value | |
| Age (yrs) | 12.7 (4.3) | 13.0 (4.2) | 0.48 |
| Gender (% female) | 69.5 | 43.4 | <0.01 |
| Ethnicity | <0.01 | ||
| White | 47.7 | 73.5 | |
| Hispanic | 17.8 | 8.0 | |
| Black | 32.2 | 12.4 | |
| Other | 2.3 | 6.1 | |
| BMI | 22.5 (5.1) | 20.1 (4.4) | <0.01 |
| Albumin G/L | 2.96 (0.78) | 3.17 (0.66) | 0.02 |
| Bilirubin mg% | 8.7 (9.8) | 6.79 (7.6) | 0.12 |
| INR mg% | 2.03 (0.94) | 1.38 (0.45) | <0.01 |
| Life Support (% yes)) | 12.6 | 2.7 | <0.01 |
| Status 1 | 25.3 | 0.9 | <0.01 |
| Cold Ischemic Time (hrs) | 7.0 (4.2) | 6.0 (2.9) | 0.03 |
| Transplant Type | 0.99 | ||
| Whole | 78.7 | 78.8 | |
| Technical variant | 21.3 | 21.2 | |
| Donor Age (yrs) | 19.8 (12.2) | 19.7 (14.7) | 0.95 |
| Donor type | 0.99 | ||
| Cadaveric | 93.1 | 84.1 | |
| Living | 6.9 | 15.9 |
To cite this abstract in AMA style:
Jossen J, Annunziato R, Chu J, Kim H-S, Sheflin S, Lai J, Arnon R. Liver Transplantation for Children With Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Analysis of the UNOS Database [abstract]. Am J Transplant. 2015; 15 (suppl 3). https://atcmeetingabstracts.com/abstract/liver-transplantation-for-children-with-autoimmune-hepatitis-and-primary-sclerosing-cholangitis-an-analysis-of-the-unos-database/. Accessed December 3, 2024.« Back to 2015 American Transplant Congress