JPGN Reports · 14 authors, 6 centres
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This case report describes an 8-month-old infant with TAR syndrome who developed pediatric acute liver failure due to autoimmune hepatitis and underwent liver transplantation, but died from complications. It highlights a previously unreported association between TAR syndrome and autoimmune hepatitis.
This is a single case report of an 8-month-old female with genetically confirmed TAR syndrome who presented with acute liver failure. Extensive evaluation identified autoimmune hepatitis as the cause, indicated by a high titer anti-liver-kidney microsomal-1 antibody (>1:2560) and explant pathology showing lymphoplasmacytic infiltration. She underwent a liver transplant but had a postoperative course complicated by severe T-cell-mediated rejection, antibody-mediated rejection, biliary stricture, and infections. The patient ultimately died from sepsis and cerebral edema before a second transplant could be performed. The report suggests a possible new association between TAR syndrome and autoimmune hepatitis, though it is unclear if TAR syndrome predisposed her to a severe presentation. Limitations include the single-case design and the inability to establish a causal relationship.
