Pediatric Liver Transplantation for Alpha-1 Antritrypsin Deficiency: A UNOS Database Analysis.
1Department of Pediatrics, Mount Sinai Hospital, New York, NY
2Psychology, Fordham University, Bronx, NY
Meeting: 2017 American Transplant Congress
Abstract number: B285
Keywords: Liver transplantation, Pediatric
Session Information
Session Name: Poster Session B: Pediatric Liver Transplant - Clinical
Session Type: Poster Session
Date: Sunday, April 30, 2017
Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall D1
Purpose
Alpha-1 Antritypsin Deficiency (ATD) is a heterogeneous disease, and is an important indication for liver transplantation (LT) in children. We examine the ATD population requiring LT and their outcomes in the pediatric end-stage liver disease (PELD) era.
Methods
Children ≤18 years old that had a first isolated LT for ATD were identified from the UNOS database from 3/2002-12/2012. Comparisons were made based on age and gender. Recipient, transplant, and donor characteristics were analyzed.
Results
5,140 children underwent LT in the study period. 113 (2.2%) children underwent LT for ATD. Recipient, transplant and donor characteristics are detailed in Table 1. Bilirubin and INR at transplant were significantly higher in the 0-2 year group than in the older cohorts. This youngest group also had more transplants for status 1; 18.9% versus 0%. Of the 10 patients listed as status 1, 70% were male and 90% were < 1 year old.
Patient survival at 1 year was 86.8%, 100%, and 97.9% for the youngest, middle and oldest groups respectively. 5 year patient survival was 84.9%, 100%, and 97.9%, respectively. Early graft survival (≤14 days) was significantly worse in the youngest group; 81.1% versus 91.7% and 100% in the middle and older groups (p<0.01). Younger donor age was identified as a risk factor for graft loss with thrombosis as the most common cause.
Conclusion
Survival in the PELD era was high for children undergoing LT for ATD. Children age <2 had worse coagulopathy and cholestasis, were more likely to be listed as status 1, and had increased rates of early graft loss.
| Age Group | 0-2(N=53) | 3-5(N=12) | 6-17(N=48) | |
| Mean (SD) or % | p value | |||
| Age, yr | 0.7 (.8) | 4.3 (.9) | 10.1 (3.7) | <.01 |
| Gender, %Female | 47.2 | 25.0 | 43.8 | .38 |
| Albumin, Gr/L | 2.8 (.6) | 2.7 (.7) | 3.0 (.6) | .1 |
| Bilirubin, mg% | 12.0 (15.0) | 7.1 (11.4) | 4.8 (9.6) | .01 |
| INR | 2.4 (1.4) | 1.5 (0.5) | 1.5 (.3) | |
| Status 1 | 18.9 | 0 | 0 | <.01 |
| Time between listing and transplant, days | 88 (101) | 422 (529) | 434 (658) | <.01 |
| Donor Type, %cadaveric | 88.7 | 100 | 85.4 | .3 |
| Donor Age, yr | 8.9 (14.1) | 8.3 (9.9) | 16.2 (11) | .01 |
| Donor Gender, %Female | 34.0 | 33.3 | 50 | .23 |
CITATION INFORMATION: Jossen J, Annunziato R, McShane J, Chu J, Arnon R. Pediatric Liver Transplantation for Alpha-1 Antritrypsin Deficiency: A UNOS Database Analysis. Am J Transplant. 2017;17 (suppl 3).
To cite this abstract in AMA style:
Jossen J, Annunziato R, McShane J, Chu J, Arnon R. Pediatric Liver Transplantation for Alpha-1 Antritrypsin Deficiency: A UNOS Database Analysis. [abstract]. Am J Transplant. 2017; 17 (suppl 3). https://atcmeetingabstracts.com/abstract/pediatric-liver-transplantation-for-alpha-1-antritrypsin-deficiency-a-unos-database-analysis/. Accessed May 29, 2025.« Back to 2017 American Transplant Congress