Publication Date
8-1-2023
Journal
JPGN Reports
DOI
10.1097/PG9.0000000000000325
PMID
37600606
PMCID
PMC10435028
PubMedCentral® Posted Date
6-9-2023
PubMedCentral® Full Text Version
Post-print
Published Open-Access
yes
Keywords
liver transplant, liver-kidney microsomal antibody, antibody-mediated rejection
Abstract
Thrombocytopenia absent radius (TAR) syndrome is a rare genetic disorder that has been associated with food protein-induced allergic proctocolitis and transient leukemoid reactions, among other manifestations. There has been no prior reports of its association with autoimmune disease, more specifically, autoimmune hepatitis (AIH) or the development of pediatric acute liver failure (PALF). We present a case of an 8-month-old infant with TAR syndrome who presented with PALF, secondary to AIH with elevated liver-kidney microsomal antibody (>1:2560). She received a liver transplant and had a very complicated postoperative course including severe T-cell-mediated rejection, infection, biliary stricture, persistently elevated liver-kidney microsomal antibodies, and antibody-mediated rejection. Ultimately, these complications led to graft failure, severe sepsis, and death. This case highlights a new association of TAR syndrome with AIH and PALF and a potentially aggressive nature of AIH both pre- and post-transplant.
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