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Pediatric Liver Transplantation for Alpha-1 Antritrypsin Deficiency: A UNOS Database Analysis.

J. Jossen,1 R. Annunziato,2 J. McShane,2 J. Chu,1 R. Arnon.1

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).

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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 9, 2025.

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