Retrospective Study
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Dec 27, 2019; 11(12): 752-760
Published online Dec 27, 2019. doi: 10.4254/wjh.v11.i12.752
Post-infantile giant cell hepatitis: A single center’s experience over 25 years
Bassem Matta, Ricardo Cabello, Mordechai Rabinovitz, Marta Minervini, Shahid Malik
Bassem Matta, Mordechai Rabinovitz, Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, Pittsburgh, PA 15213, United States
Ricardo Cabello, Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States
Marta Minervini, Department of Pathology, University of Pittsburgh, Pittsburgh, PA 15213, United States
Shahid Malik, Department of Medicine, University of Pittsburgh, Division of Gastroenterology Hepatology and Nutrition, Pittsburgh, PA 15213, United States
Author contributions: Matta B and Malik SM designed the research study, obtained and analyzed data and wrote manuscript; Cabello R, Rabinovitz M and Minervini M contributed with data interpretation and critical review of manuscript for intellectual content; All authors have read and approve the final manuscript.
Institutional review board statement: This study was reviewed and approved by the University of Pittsburgh Medical Center Institution Review Board PRO12030073.
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous data that were obtained after each patient agreed to treatment by written consent.
Conflict-of-interest statement: There were no conflict of interests to disclose.
Data sharing statement: All data requests should be submitted to the corresponding author for consideration. Access to anonymized data may be granted following review.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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:
Corresponding author: Shahid Malik, MD, Department of Medicine, University of Pittsburgh, Division of Gastroenterology, Hepatology and Nutrition, 3471 5th Ave 916 Lillian Kaufman Building, Pittsburgh, PA 15213, United States.
Telephone: +1-412-6474932 Fax: +1-412-6479268
Received: May 15, 2019
Peer-review started: May 17, 2019
First decision: July 4, 2019
Revised: September 9, 2019
Accepted: October 18, 2019
Article in press: October 18, 2019
Published online: December 27, 2019
Research background

Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades. Pathological analysis is characterized by the presence of giant multinucleated syncytial hepatocytes. The clinical course of patients with giant cells on histology is widely variable, ranging from minimal symptoms without major clinical implications to acute liver failure that is often times fatal despite standard clinical care.

Research objectives

Our primary objective was to present our center’s experience in an attempt to learn about the predisposing factors, outcomes and efficacy of proposed therapeutic interventions for giant cell hepatitis.

Research methods

A retrospective chart review was conducted through the electronic records of the University of Pittsburgh Medical Center. We queried 36726 liver biopsy reports from January 1, 1991 to December 6, 2016. Our search yielded 50 patients who were identified as carrying a definite diagnosis of post-infantile giant cell hepatitis (PIGCH) by pathology. The data collected included demographic information, laboratory data (liver function tests, autoimmune markers) and transplant status. In order to better analyze patient characteristics and outcomes, subjects were separated into a non-transplant (native) liver group and a post-liver transplant (allograft) group.

Research results

The incidence of PIGCH was approximately 0.14% of all biopsies queried in the 25-year period. The mean age was 48 years with 66% females. Liver function tests were classified as 38.2% cholestatic, 35.3% hepatocellular and 26.5% mixed. Autoimmune hepatitis was found to be the most prevalent predisposing factor leading to PIGCH constituting 32% of cases. Management consisted mainly of immunosuppression, viral targeted therapy, supportive care and in six cases liver transplantation.

Research conclusions

The diagnosis of PIGCH remains clinically challenging and requires a high index of suspicion as well as a thorough history, physical examination, serological workup and liver biopsy. Treatment of the underlying cause can result in clinical stability in a large number of cases.

Research perspectives

This study reports our center’s experience with PIGCH and the importance of thorough history, physical examination, serologic work up and liver biopsy in its diagnosis. Further research should aim at recognizing risk factors for progression from PIGCH to liver failure and further evaluation of therapeutic interventions (immunosuppression vs viral targeted therapy vs liver transplantation).