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Gastroenterology Research and Practice
Volume 2009, Article ID 765318, 3 pages
Case Report

Mauriac Syndrome in a Child with a Positive Antinuclear Antibody Screen

Department of Pediatric Gastroenterology, Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, UT 84113-1103, USA

Received 22 July 2009; Accepted 24 September 2009

Academic Editor: Vasundhara Tolia

Copyright © 2009 John F. Pohl. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


A 17-year-old male with type 1 diabetes mellitus (T1DM) presented to clinic with elevated transaminases and a positive antinuclear antibody (ANA) screen. Due to concern for autoimmune hepatitis, a liver biopsy was performed which revealed Mauriac syndrome. This case report is the second known description of a child with Mauriac syndrome presenting with positive autoimmune markers.