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Fulminant hepatitis associated with centrilobular hepatic necrosis in young children
E M Alonso1, R J Sokol, J Hart
1Department of Pediatrics, University of Chicago Pritzker School of Medicine, Illinois, USA.
Insights
Fulminant hepatic failure (FHF) in children presented with unique features, including lower bilirubin levels and centrilobular necrosis. This distinct FHF presentation in pediatric patients showed a good prognosis, unlike typical cases.
Area of Science:
- Pediatric Hepatology
- Toxicology
- Viral Hepatitis
Background:
- Fulminant hepatic failure (FHF) in children typically presents with specific clinical and histopathologic features.
- A distinct form of FHF in children was observed, differing from the usual presentation.
Purpose of the Study:
- To describe the clinical and histopathologic features of a unique presentation of fulminant hepatic failure (FHF) in pediatric patients in the United States.
- To differentiate this FHF presentation from typical cases seen in children.
Main Methods:
- Seven young children diagnosed with FHF in early 1994 were analyzed.
- Clinical data including encephalopathy, coagulopathy, elevated aminotransferase levels, and serum bilirubin levels were recorded.
- Histopathologic examination of liver tissue was performed.
- Patient history regarding prodromal viral illness, fasting, and acetaminophen intake was collected.
Main Results:
- Patients presented with encephalopathy, coagulopathy, and elevated aminotransferase levels, but with serum bilirubin levels below 171 mumol/L.
- Histology revealed centrilobular zonal necrosis, characteristic of toxic injury rather than typical viral hepatitis.
- Six out of seven children recovered spontaneously; one patient died from complications.
Conclusions:
- The observed FHF in children exhibits distinct clinical and histopathologic features, differing from typical FHF.
- A viral or environmental insult, potentially exacerbated by therapeutic acetaminophen doses during fasting, is postulated as the cause.
- The prognosis for this specific FHF presentation appears better than for typical pediatric FHF, correlating with the extent of liver necrosis.
Objective:
To describe fulminant hepatic failure (FHF) in children in the United States with clinical and histopathologic features distinctly different from those typical of FHF.
Patients:
Seven young children were seen in early 1994 with encephalopathy, coagulopathy, and elevated aminotransferase levels. Liver failure was preceded by a prodromal viral illness that resulted in a period of fasting without dehydration. Unlike the majority of children with FHF, these patients had serum bilirubin levels < 171 mumol/L (10 mg/dl). All children had received therapeutic doses of acetaminophen during the prodromal illness.
Histopathologic Findings:
Histologic findings included zonal necrosis of hepatocytes in a centrilobular distribution, which is characteristic of toxic liver injury but is atypical for viral hepatitis and sporadic non-A non-B hepatitis.
Outcome:
Six patients recovered spontaneously, and one died of complications of liver failure and fungal sepsis. The cause of this disorder remains unknown, but we postulate a viral or environmental insult that preferentially damages zone 3 hepatocytes. The potential for this injury may have been augmented by ingestion of therapeutic doses of acetaminophen while patients were in a fasted state. The prognosis was good compared with typical FHF in children and correlated with the degree of liver necrosis on histologic examination.