Evaluating Clinical Presentations and Diagnostic Procedures in Pediatric Autoimmune Hepatitis

Bita Heshmati; Parisa Rahmani; Hosein Alimadadi; Jeyran  Zebardast

doi:10.18502/jimc.v7i2.15039

Bita Heshmati Department of Pediatrics, Iran Children’s Medical Center, Center of Pediatric Excellence School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Parisa Rahmani Department of Pediatrics, Iran Children’s Medical Center, Center of Pediatric Excellence School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Hosein Alimadadi Department of Pediatrics, Iran Children’s Medical Center, Center of Pediatric Excellence School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Jeyran Zebardast Department of Cognitive Linguistics, Institute for Cognitive Science Studies (ICSS), Tehran, Iran

DOI: https://doi.org/10.18502/jimc.v7i2.15039

Keywords: Autoimmune hepatitis, Child, Liver transplantation, MUSK protein, Human

Abstract

Background: The aim of the current research was to assess the clinical manifestations and diagnostic methods used in juvenile cases of Autoimmune Hepatitis (AIH).

Methods: This study employed a retrospective cross-sectional design to investigate pediatric patients diagnosed with AIH at Children’s Medical Center Hospital, which is affiliated to Tehran University of Medical Sciences (TUMS), Tehran, Iran. The study included patients who received routine examinations, treatments, and follow-ups during the period from 2018 to 2021.

Results: The present investigation encompassed the evaluation of 52 pediatric patients, mostly female, with a mean age of 7.76 years. The vast majority of patients have encountered the occurrence of acute AIH. Positive findings for the Anti-Smooth Muscle Antibodies (ASMA) test were seen in 50% of the patients. The mean score for fibrosis in the observed individuals was 2.56, whereas the mean value for the Hepatitis Activity Index (HAI) in a subset of 29 patients was found to be 7.34. One patient succumbed to the condition, one case underwent transplantation, and another individual was identified as a candidate for liver transplantation.

Conclusion: Patients with AIH saw a decrease in long-term survival. There was no observed disparity in prognosis based on gender; nevertheless, it was noted that males had a shorter lifespan, perhaps attributable to an earlier beginning of the illness. The presence of cirrhosis at the time of diagnosis constituted a significant risk factor for unfavorable prognosis, as it was associated with an elevated overall risk of mortality owing to liver dysfunction.