Overview of the Etiology, Pathology, and Prognosis of Giant Cell Hepatitis
Demet Teker Düztaş1, Hakan Öztürk, Ödül Eğritaş Gürkan
1From the Department of Pediatric Gastroenterology, Gazi University School of Medicine, Ankara, Turkey.
Insights
Giant cell hepatitis in children often presents with jaundice, and its cause remains unknown in many cases. Further research is crucial for understanding this condition and developing targeted treatments.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Pathology
Background:
- Giant cell hepatitis is a significant diagnostic concern in pediatric patients, frequently presenting with jaundice.
- The etiology of giant cell hepatitis is diverse and often challenging to determine in young children.
Purpose of the Study:
- To present the demographic, clinical, and histopathological characteristics of pediatric patients diagnosed with giant cell hepatitis at our center.
- To investigate the etiological factors and outcomes in a cohort of children with giant cell hepatitis.
Main Methods:
- Retrospective evaluation of liver biopsies with giant cell formation between January 2010 and December 2023.
- Analysis of demographic, clinical, and histopathological data from medical records of included pediatric patients.
Main Results:
- The study included 31 pediatric patients with giant cell hepatitis, predominantly male (90.3%) with a median age of 4 months.
- Jaundice was the most common clinical finding (93.5%). Idiopathic giant cell hepatitis accounted for 41.9% of cases, while progressive familial intrahepatic cholestasis, biliary atresia, and autoimmune hepatitis were the most frequent identified etiologies.
- Histopathological findings included canalicular cholestasis (87.1%) and pericellular fibrosis (35.5%). Liver transplantation was required in 32.3% of patients, and 29% died during the follow-up period.
Conclusions:
- Etiology remains unspecified in a significant proportion of pediatric giant cell hepatitis cases, mirroring findings in existing literature.
- Additional research is necessary to elucidate the underlying causes, identify novel etiologies, and develop specific therapeutic strategies for giant cell hepatitis in children.
Objectives:
Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis.
Materials And Methods:
Liver biopsies performed in our hospital between January 2010 and December 2023 were retrospectively evaluated, and biopsies with histological giant cell formation were included in the study. Demographic, clinical, and histopathological characteristics of the patients were obtained from the medical records.
Results:
Our study included 31 cases of giant cell hepatitis. Twenty-four (90.3%) of the patients were male, and the median age was 4 months (range, 1-148 mo). The most common clinical findings were jaundice (93.5%). Among the 31 patients, idiopathic giant cell hepatitis was observed in 13 cases (41.9%), and etiology was elucidated in 18 patients. The most common diagnoses in these patients were progressive familial intrahepatic cholestasis, biliary atresia, and autoimmune hepatitis (22.6%, 19.4%, and 9.7%, respectively). The most common accompanying histopathologic findings were canalicular cholestasis (87.1%), lobular cholestasis (54.8%), and pericellular fibrosis (35.5%). Liver transplant was required in 10 patients (32.3%). Nine patients (29%) died from various reasons. Although follow-up of 13 patients (41.9%) are ongoing, 9 patients (29%) are no longer being followed.
Conclusions:
Few studies in the pediatric age group on giant cell hepatitis have been conducted in the literature. Similar to our series, etiology could not be specified in most of the patients in these reports. Additional research is needed to improve our understanding of the disease process, uncover any other potential causes, and create specific treatment options.
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