Neonatal syncytial giant cell hepatitis with paramyxoviral-like inclusions

Syncytial giant cell hepatitis in the neonatal period has been associated w ith many different etiologic agents and may present initially as cholestasi s. 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, rube lla virus, herpes simplex, HHV-6, varicella, coxsackievirus, echovirus, reo virus 3, parvovirus B19, HIV, enteroviruses, paramyxovirus, and hepatitis A , B, or C (rare). Giant cell hepatitis may result in fulminant liver failur e with massive hepatocyte necrosis and severe liver dysfunction leading to death, resolution with severely compromised liver function, or liver transp lantation. The authors report a 6-week-old male who had an unremarkable per inatal period, became jaundiced after developing diarrhea, and subsequently developed liver dysfunction with massively increased liver enzymes and a c oagulopathy. Open wedge and core liver biopsies were performed to determine if the patient should be listed for liver transplantation. Giant cell hepa titis with a significant mixed lymphocytic and neutrophilic infiltrate was present on both the wedge and core biopsies. The residual 60% of hepatocyte s had ballooning degeneration and many possessed pyknotic nuclei. The hepat ocytes were arranged in a pseudoacinar pattern. Electron microscopy showed paramyxoviral-like inclusions in the giant cells, characterized as large in clusions with fine filamentous, beaded substructures (18-20 nm). Paramyxovi ridae are nonsegmented, negative-sense, single-stranded RNA viruses. This f amily is divided into the Paramyxovirinae subfamily containing respirovirus (Sendai virus, parainfluenza virus type 3), rubulavirus (mumps, parainflue nza virus type 2), and morbillivirus genera (measles); and Pneumovirinae su bfamily (pneumovirus genus [respiratory syncytial virus]). Supportive care to determine if hepatic function resolves following the viral episode, live r transplantation with fulminant liver failure, and ongoing evaluation in t hose who recover to assess chronic liver disease are necessary. Ultrastruct ural evaluation may unmask the etiologic agent for hepatitis and direct the rapy.