JPGN reports · 2023
Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Mercedes Rebecca, Patel Kalyani, Shiau Henry, Mysore Krupa R, Ruan Wenly, Leung Daniel H, Tessier Mary Elizabeth M, Cerminara Dana, Nicholas Sarah, Fuller Kelby, Faraone Marielle, Galvan N Thao N, Goss John, Banc-Husu Anna M
Аннотация
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.