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Our experience with fulminant hepatic failure in Turkish children: etiology and outcome
Sema Aydoğdu1, Funda Ozgenç, Serap Yurtsever
1Department of Pediatrics, Division of Pediatric Gastroenterology and Nutrition, Ege University, Turkey.
Insights
Fulminant hepatic failure in Turkish children is most commonly caused by Hepatitis A virus (HAV) infection. Mortality remains high, but liver transplantation offers improved survival rates for eligible patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Infectious Diseases
Background:
- Fulminant hepatic failure (FHF) in children is a critical medical emergency.
- Etiology of FHF varies by age and geographic region.
Purpose of the Study:
- To retrospectively investigate the causes and outcomes of FHF in Turkish children.
- To correlate etiological factors, clinical presentation, and biochemical parameters with patient outcomes.
Main Methods:
- Retrospective analysis of 34 children diagnosed with FHF.
- Evaluation of etiological factors, clinical data, and biochemical markers.
- Correlation of various factors with mortality and survival.
Main Results:
- Acute viral hepatitis was the most frequent cause (35.2%), with Hepatitis A virus (HAV) being the most common (26.4%).
- Wilson's disease was identified in 12.5% of cases.
- Cryptogenic causes accounted for 44.1% of FHF cases.
- Overall mortality was high at 67.6%; higher encephalopathy grade and bilirubin levels correlated with increased mortality.
- Liver transplantation was possible for 7 patients, showing a significantly lower mortality rate (28.5%).
Conclusions:
- Fulminant Hepatitis A virus infection is the leading identifiable cause of FHF in Turkish children.
- Early identification of etiological factors and consideration of liver transplantation are crucial for improving outcomes in pediatric FHF.
Abstract:
Fulminant hepatic failure is a rare and devastating event during childhood. The etiology of liver failure is reported to change according to age and geographical location. We aimed to investigate, retrospectively, causes and outcome of fulminant hepatic failure in Turkish children. Thirty-four children with fulminant hepatic failure were analysed by means of etiology and outcome. Etiological factor, clinical presentation, encephalopathy stage and biochemical parameters were correlated with outcome. Acute viral hepatitis was detected in 12 cases (35.2 per cent) and hepatitis A was the most commonly detected cause among cases with fulminant hepatic failure (n = 9, 26.4 per cent). Hepatitis B and non A-E infection were diagnosed in two (5.8 per cent) and one (2.9 per cent) cases, respectively. Wilson's disease was defined in four patients (12.5 per cent). Budd-Chiari syndrome (2.9 per cent), autoimmune hepatitis (2.9 per cent) and mushroom poisoning (2.9 per cent) were other detected causes of fulminant hepatic failure in this group. No viral, metabolic, toxic or anatomic reason could be detected in the remaining 15 (44.1 per cent) patients and they were evaluated as cryptogenic. Mortality was 67.6 per cent (23 cases). Encephalopathy grade, total and indirect bilirubin levels were found to be significantly higher in patients who died (p = 0.004, p = 0.03, p = 0.04). Seven patients could have been transplanted (two cadavaric, five living related) and the mortality of this group was 28.5 per cent (n = 2). It was concluded that fulminant hepatitis A virus (HAV) infection is the most common detectable cause of fulminant hepatic failure in Turkish children.
