Massive hepatic necrosis with impaired consciousness occurring within 8 weeks of the onset of illness.
Infections (viral, including HAV, HBV, HCV [rarely], HDV, HEV; bacterial, rickettsial, parasitic), drugs and toxins, ischemia (shock), Budd-Chiari syndrome, idiopathic chronic active hepatitis, acute Wilson's disease, microvesicular fat syndromes (Reye's syndrome, acute fatty liver of pregnancy).
Pts usually present with signs and symptoms of encephalopathy that may evolve to deep coma. The combination of rapidly shrinking liver size, rapidly rising bilirubin level, and marked prolongation of the PT, even as aminotransferase levels fall, together with clinical signs of confusion, disorientation, somnolence, ascites, and edema, indicates that the pt has hepatic failure with encephalopathy. Cerebral edema is common; brainstem compression, gastrointestinal bleeding, sepsis, respiratory failure, cardiovascular collapse, and renal failure are terminal events. The mortality rate is exceedingly high (>80% in pts with deep coma).
TREATMENT | ||
Fuliminant HepatitisThe goal of therapy is to support the pt by maintenance of fluid balance, support of circulation and respiration, control of bleeding, correction of hypoglycemia, and treatment of other complications of the comatose state in anticipation of liver regeneration and repair. Protein intake should be restricted, and oral lactulose or neomycin administered. Meticulous intensive care that includes prophylactic antibiotic coverage is the one factor that does appear to improve survival. Liver transplantation should be considered. |