Hepatic Failure/GI/Endocrine Emergencies

Typically caused by the Amanita phalloides spp. of mushrooms

ii.

Mushroom poisoning is classified as a hyperacute ALF with an onset of symptoms within

24 hours after ingestion. In addition to hepatotoxicity, patients with mushroom poisoning

present with severe gastrointestinal (GI) symptoms including nausea, vomiting, diarrhea, and

abdominal cramping.

Wilson disease

Rare cause of ALF; implicated in approximately 1% of cases per year, mostly affecting young

people

ii.

Wilson disease is a rare disorder that causes accumulation of copper in the body. Diagnosis is

characterized by an abrupt onset of Coombs-negative hemolytic anemia with serum bilirubin

greater than 20 mg/dL. Other diagnostic laboratory findings include low serum ceruloplasmin,

presence of Kayser-Fleischer rings, and high urinary and hepatic copper concentrations.

iii.

Patients who present with fulminant liver failure secondary to Wilson disease have exceedingly

high mortality rates without transplantation.

Autoimmune hepatitis

Autoimmune hepatitis is considered a chronic inflammatory disease; however, patients can

still be considered to have ALF if they had rapid deterioration of symptoms.

ii.

About 20% of patients with stable disease will have ALF, which is typically instigated by an

environmental trigger.

Budd-Chiari syndrome

Budd-Chiari syndrome is caused by an acute hepatic vein thrombosis. Presenting symptoms

include abdominal pain, ascites, and frank hepatomegaly. Patients who initially present with

ALF require anticoagulation with heparin as initial therapy; in those who do not respond

anticoagulation, transjugular intrahepatic portosystemic shunt (TIPS) may be considered.

ii.

In patients with ALF who do not respond to medical or therapeutic interventions, transplantation

may be required.

Acute fatty liver of pregnancy/hemolysis, elevated liver enzymes, low platelets (HELLP)

Toward the end of pregnancy, a small percentage of women will develop rapidly progressive

hepatic failure that is generally associated with three hallmark signs: jaundice, coagulopathy,

and low platelets, also known as HELLP. Features of pre-eclampsia such as hypertension and

proteinuria are common with HELLP.

ii.

HELLP is associated with increased mortality for both the fetus and the mother.

iii.

Requires emergency delivery of fetus, after which symptoms should resolve. If ALF fails to

improve after delivery of the fetus, liver transplant evaluation is necessary.

ALF affects almost every organ system in the body:

Neurologic

Cerebral edema and elevated intracranial pressures (ICPs) are the most serious complications

of ALF. Uncontrolled edema and elevated ICPs can lead to uncal herniation and are usually

fatal. Cerebral edema may also lead to tissue hypoxia, which may result in long-term neurologic

deficits.

inflammation, and neurotoxins are thought to be the primary causes.