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Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report

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 specifica...

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Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins, Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435028/
https://www.ncbi.nlm.nih.gov/pubmed/37600606
http://dx.doi.org/10.1097/PG9.0000000000000325
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author 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.
author_facet 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.
author_sort Mercedes, Rebecca
collection PubMed
description 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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spelling pubmed-104350282023-08-18 Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report 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. JPGN Rep Case Report 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. Lippincott Williams & Wilkins, Inc. 2023-06-09 /pmc/articles/PMC10435028/ /pubmed/37600606 http://dx.doi.org/10.1097/PG9.0000000000000325 Text en Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY (https://creativecommons.org/licenses/by/4.0/) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
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.
Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title_full Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title_fullStr Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title_full_unstemmed Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title_short Pediatric Acute Liver Failure Secondary to Autoimmune Hepatitis in an Infant With Thrombocytopenia-Absent Radius (TAR) Syndrome: A Case Report
title_sort pediatric acute liver failure secondary to autoimmune hepatitis in an infant with thrombocytopenia-absent radius (tar) syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435028/
https://www.ncbi.nlm.nih.gov/pubmed/37600606
http://dx.doi.org/10.1097/PG9.0000000000000325
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