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fulminant hepatic failure

Tuesday 10 May 2011

Fulminant hepatic failure (FHF) in infants and children is a rare but often fatal condition.

Recognized etiologies include metabolic disease, infection (mostly viral or presumed viral), drugs and toxins, and autoimmune disease.

In particular, viral hepatitis A account for the majority of cases in developing nations, whereas in developed nations approximately 50% of cases remain etiologically unclassified despite extensive investigation.

The histologic correlate of FHF is massive hepatic necrosis or submassive hepatic necrosis in the majority of cases, but necrosis may be absent in FHF associated with certain metabolic conditions.

A few well-recognized patterns may help to narrow the differential diagnosis such as :
- centrilobular coagulative necrosis (seen with acetaminophen toxicity, halothane, or shock),
- severe steatosis (seen in certain toxic/metabolic conditions),
- geographic nonzonal hemorrhagic necrosis (herpes simplex virus and adenovirus),
- subacute necrosis with marked interface activity and plasma cell prominence (suggestive but not specific for autoimmune hepatitis [AIH]).

Diffuse confluent necrosis - However, the vast majority of cases display a nonspecific picture of diffuse confluent necrosis, often panlobular or multilobular, accompanied by a variable, often prominent portal and lobular lymphocytic and histiocytic infiltrate, hemorrhage, and vacant sinusoids. This pattern is associated with the lowest yield in terms of etiologic diagnosis; many cases are ultimately presumed viral (non–A-E hepatitis) unless there is good clinical evidence to suspect a drug/toxin-related injury.

Within this group, a number of features are periodically encountered for which clinicopathologic correlates are not well established. Such features include syncytial giant cell change, central venulitis, cholangiolitis/pericholangiolitis, lymphocytic infiltration of biliary epithelium, prominence of plasma cells, prominence of eosinophils, and minor degrees of steatosis. Whether these findings merely constitute part of the histologic spectrum of (S)MHN or point to specific etiologic processes remains to be determined.

See also

- fulminant hepatitis
- massive hepatic necrosis or submassive hepatic necrosis