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Volume 13
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10.3390/diagnostics13172753
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Review

Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome

by
Silvia Nastasio
1,
Antonella Mosca
2,
Tommaso Alterio
2,
Marco Sciveres
3 and
Giuseppe Maggiore
2,*
1
Division of Gastroenterology, Hepatology & Nutrition, Boston Children’s Hospital and Harvard Medical School, Boston, MA 02115, USA
2
Hepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
3
Pediatric Department and Transplantation, ISMETT, 90133 Palermo, Italy
*
Author to whom correspondence should be addressed.
Diagnostics 2023, 13(17), 2753; https://doi.org/10.3390/diagnostics13172753
Submission received: 11 July 2023 / Revised: 11 August 2023 / Accepted: 13 August 2023 / Published: 24 August 2023
(This article belongs to the Special Issue Advances in the Diagnosis and Management of Pediatric Autoimmune Diseases)
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Abstract

Juvenile autoimmune hepatitis (JAIH) is severe immune-mediated necro-inflammatory disease of the liver with spontaneous progression to cirrhosis and liver failure if left untreated. The diagnosis is based on the combination of clinical, laboratory and histological findings. Prothrombin ratio is a useful prognostic factor to identify patients who will most likely require a liver transplant by adolescence or early adulthood. JAIH treatment consists of immune suppression and should be started promptly at diagnosis to halt inflammatory liver damage and ultimately prevent fibrosis and progression to end-stage liver disease. The risk of relapse is high especially in the setting of poor treatment compliance. Recent evidence however suggests that treatment discontinuation is possible after a prolonged period of normal aminotransferase activity without the need for liver biopsy prior to withdrawal.
Keywords: autoimmune hepatitis; acute liver failure; autoimmune liver disease; active chronic hepatitis liver transplantation autoimmune hepatitis; acute liver failure; autoimmune liver disease; active chronic hepatitis liver transplantation

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MDPI and ACS Style

Nastasio, S.; Mosca, A.; Alterio, T.; Sciveres, M.; Maggiore, G. Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome. Diagnostics 2023, 13, 2753. https://doi.org/10.3390/diagnostics13172753

AMA Style

Nastasio S, Mosca A, Alterio T, Sciveres M, Maggiore G. Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome. Diagnostics. 2023; 13(17):2753. https://doi.org/10.3390/diagnostics13172753

Chicago/Turabian Style

Nastasio, Silvia, Antonella Mosca, Tommaso Alterio, Marco Sciveres, and Giuseppe Maggiore. 2023. "Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome" Diagnostics 13, no. 17: 2753. https://doi.org/10.3390/diagnostics13172753

APA Style

Nastasio, S., Mosca, A., Alterio, T., Sciveres, M., & Maggiore, G. (2023). Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome. Diagnostics, 13(17), 2753. https://doi.org/10.3390/diagnostics13172753

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