Published online Dec 27, 2019. doi: 10.4254/wjh.v11.i12.752
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
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.
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.
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.
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 transplantations.
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.
Core tip: Post-infantile giant cell hepatitis is a rare disorder and very poorly defined in the literature. Our study aimed to present our center’s experience in an attempt to shed more light about the predisposing factors, outcomes and efficacy of proposed therapeutic interventions for post-infantile giant cell hepatitis.