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World J Clin Pediatr. Jun 9, 2023; 12(3): 77-85
Published online Jun 9, 2023. doi: 10.5409/wjcp.v12.i3.77
Seronegative autoimmune hepatitis in childhood
Ali Islek, Gokhan Tumgor
Ali Islek, Gokhan Tumgor, Department of Pediatric Gastroenterology, Cukurova University School of Medicine, Adana 01320, Turkey
Author contributions: Islek A substantially contributed to the conception and design of the paper and the acquisition, analysis and interpretation of the data; Islek A and Tumgor G drafted the article and made critical revisions related to the intellectual content of the manuscript and approved the final version of the article to be published.
Conflict-of-interest statement: There was no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ali Islek, MD, Associate Professor, Department of Pediatric Gastroenterology, Cukurova University School of Medicine, Sarıcam, Adana 01320, Turkey. islekali@hotmail.com
Received: January 28, 2023
Peer-review started: January 28, 2023
First decision: April 2, 2023
Revised: April 26, 2023
Accepted: May 8, 2023
Article in press: May 8, 2023
Published online: June 9, 2023
Processing time: 130 Days and 15.2 Hours
Abstract

Comprehensive guidelines on seropositive autoimmune hepatitis have been published for both adults and children, although these guidelines comprise only limited knowledge about seronegative autoimmune hepatitis. Autoimmune hepatitis presents as an acute or chronic progressive disease and poor outcomes are inevitable if left untreated. The absence of autoantibody positivity, hypergammaglobulinemia and lack of comprehensive algorithms makes seronegative autoimmune hepatitis a mysterious disease. In general, seronegative autoimmune hepatitis often presents with acute hepatitis, and its treatment and prognosis similar to seropositive autoimmune hepatitis. The present review focuses on the known characteristics of seronegative autoimmune hepatitis in childhood, and those of which current knowledge is vague.

Keywords: Seronegative autoimmune hepatitis; Autoantibody negative; Lymphocytopenia; Aplastic anemia

Core Tip: Seronegative autoimmune hepatitis is a diagnostic dilemma. The absence of autoantibodies and hypergammaglobulinemia which is not uncommon in seronegative autoimmune hepatitis complicates the diagnosis. Seronegative autoimmune hepatitis often presents with acute hepatitis, and its treatment and prognosis similar to seropositive autoimmune hepatitis. This review discusses whether seronegativity is only a matter of laboratory issue or if it implies an entity with different pathogenesis, clinical features, treatment algorithms, and prognosis.