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Neonatal syncytial giant cell hepatitis with paramyxoviral-like inclusions
1Department of Pathology, Texas Children's Hospital, Houston 77030-2399, USA. mjhicks@texaschildrenhospital.org
Abstract:
Syncytial giant cell hepatitis in the neonatal period has been associated with many different etiologic agents and may present initially as cholestasis. Infectious causes are most common and include: (1 ) generalized bacterial sepsis, (2) viral agents, (3) toxoplasmosis, (4) syphilis, (5) listeriosis, and (6) tuberculosis. Viral hepatitis may be due to cytomegalovirus, rubella virus, herpes simplex, HHV-6, varicella, coxsackievirus, echovirus, reovirus 3, parvovirus B19, HIV, enteroviruses, paramyxovirus, and hepatitis A, B, or C (rare). Giant cell hepatitis may result in fulminant liver failure with massive hepatocyte necrosis and severe liver dysfunction leading to death, resolution with severely compromised liver function, or liver transplantation. The authors report a 6-week-old male who had an unremarkable perinatal period, became jaundiced after developing diarrhea, and subsequently developed liver dysfunction with massively increased liver enzymes and a coagulopathy. Open wedge and core liver biopsies were performed to determine if the patient should be listed for liver transplantation. Giant cell hepatitis with a significant mixed lymphocytic and neutrophilic infiltrate was present on both the wedge and core biopsies. The residual 60% of hepatocytes had ballooning degeneration and many possessed pyknotic nuclei. The hepatocytes were arranged in a pseudoacinar pattern. Electron microscopy showed paramyxoviral-like inclusions in the giant cells, characterized as large inclusions with fine filamentous, beaded substructures (18-20 nm). Paramyxoviridae are nonsegmented, negative-sense, single-stranded RNA viruses. This family is divided into the Paramyxovirinae subfamily containing respirovirus (Sendai virus, parainfluenza virus type 3), rubulavirus (mumps, parainfluenza virus type 2), and morbillivirus genera (measles); and Pneumovirinae subfamily (pneumovirus genus [respiratory syncytial virus]). Supportive care to determine if hepatic function resolves following the viral episode, liver transplantation with fulminant liver failure, and ongoing evaluation in those who recover to assess chronic liver disease are necessary. Ultrastructural evaluation may unmask the etiologic agent for hepatitis and direct therapy.
Insights
Neonatal giant cell hepatitis can stem from various infections. This case highlights paramyxovirus as a cause, identified via electron microscopy, guiding potential treatment strategies.
Area of Science:
- Hepatology
- Virology
- Pediatric Gastroenterology
Background:
- Neonatal giant cell hepatitis presents with cholestasis and can have diverse etiologies.
- Infectious agents, particularly viruses, are common causes of this condition.
- Giant cell hepatitis can lead to severe liver dysfunction, liver failure, or necessitate transplantation.
Observation:
- A 6-week-old infant developed jaundice, diarrhea, and liver dysfunction with elevated enzymes and coagulopathy.
- Liver biopsies revealed giant cell hepatitis with significant inflammation and hepatocyte damage.
- Electron microscopy identified paramyxoviral-like inclusions within giant cells.
Findings:
- The presence of paramyxoviral inclusions in giant cells suggests a viral etiology for the hepatitis.
- Paramyxoviridae are RNA viruses including genera like respirovirus, rubulavirus, and morbillivirus.
- Ultrastructural analysis is crucial for identifying the causative agent in hepatitis cases.
Implications:
- Identifying the specific viral agent, such as paramyxovirus, can inform supportive care and management strategies.
- Timely diagnosis is essential for determining the need for liver transplantation in fulminant liver failure.
- Long-term monitoring is necessary for patients recovering from giant cell hepatitis to assess for chronic liver disease.