Date: Sunday, April 30, 2017
Session Name: Poster Session B: Pediatric Liver Transplant - Clinical
Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall D1
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.
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.
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.
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.
|Mean (SD) or %||p value|
|Age, yr||0.7 (.8)||4.3 (.9)||10.1 (3.7)||<.01|
|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)|
|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 January 27, 2021.
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