Abstract
Acute liver failure (ALF) (sometimes referred to as fulminant hepatic failure) is
a clinical syndrome from a variety of causes resulting from rapid loss in hepatocyte
function, typically associated with coagulopathy and encephalopathy in a patient without
preexisting liver disease or cirrhosis. Cerebral edema is a cardinal feature and may
produce uncal herniation, yielding brain stem compression and death. The typical interval
from onset of symptoms to onset of encephalopathy is 1 to 2 weeks, but cases evolving
more slowly, up to 6 months, may still be included in the definition. ALF is rare,
affecting 2000 patients annually in the United States, and comprises ∼7% of liver
transplants annually. Currently, in the United States, acetaminophen accounts for
∼50% of all cases of ALF, but other etiologies include hepatitis, drug-induced liver
injury, autoimmune hepatitis. Prior to the availability of liver transplantation (LT),
mortality of ALF was extremely high, often exceeding 90%; most common causes of death
were multiorgan failure, hemorrhage, infection, and cerebral edema. Fortunately, survival
has improved considerably in the last 3 decades (overall survival now exceeds 60%).
In large part, this improved survival reflects the option of LT but also reflects
the high frequency of acetaminophen toxicity as a cause of ALF. In fact, most patients
with ALF are not candidates for LT. Critical care of patients with ALF is key to their
survival, and decisions must sometimes be made with inadequate information. We review
standard practices (medical, pharmacological, and LT) and new research initiatives
and findings for this interesting but vexing orphan disease. Particular attention
will be paid to practical matters for clinicians to consider in approaching the ALF
patient.
Keywords
acute liver injury - acetaminophen toxicity - coagulopathy - encephalopathy - hepatic
necrosis
