Wilson’s disease is a progressive, familial, terminal neurologic disease accompanied by
chronic liver disease leading to cirrhosis.
It is associated with increased storage of copper.
PRIMARY BILIARY CIRRHOSIS
Primary biliary cirrhosis (PBC) is characterized by generalized pruritus, hepatomegaly,
and hyperpigmentation of the skin.
NONALCOHOLIC FATTY LIVER DISEASE
Nonalcoholic fatty liver disease (NAFLD) is a group of disorders that is characterized
by hepatic steatosis (accumulation of fat in the liver) that is not associated with other
causes such as hepatitis, autoimmune disease, or alcohol.
The risk for developing NAFLD is a major complication of obesity. NAFLD can progress
to liver cirrhosis.
NAFLD should be considered in patients with risk factors such as obesity, diabetes,
hypertriglyceridemia, severe weight loss (especially in those whose weight loss was
recent), and syndromes associated with insulin resistance.
Cirrhosis is a chronic progressive disease characterized by extensive degeneration and
destruction of the liver parenchymal cells.
Common causes of cirrhosis include alcohol, malnutrition, hepatitis, biliary obstruction,
and right-sided heart failure. Excessive alcohol ingestion is the single most common
cause of cirrhosis followed by chronic hepatitis (B and C).
Manifestations of cirrhosis include jaundice, skin lesions (spider angiomas),
hematologic problems (thrombocytopenia, leucopenia, anemia, coagulation disorders),
endocrine problems, and peripheral neuropathy.
Major complications of cirrhosis include portal hypertension, esophageal and gastric
varices, peripheral edema and ascites, hepatic encephalopathy, and hepatorenal
o Hepatic encephalopathy is a neuropsychiatric manifestation of liver damage. It
is considered a terminal complication in liver disease.
o A characteristic symptom of hepatic encephalopathy is asterixis (flapping
Diagnostic tests for cirrhosis include elevations in liver enzymes, decreased total protein,
fat metabolism abnormalities, and liver biopsy. There is no specific therapy for cirrhosis. Management of ascites is focused on sodium
restriction, diuretics, and fluid removal.
o Peritoneovenous shunt is a surgical procedure that provides continuous reinfusion
of ascitic fluid into the venous system.
o The main therapeutic goal for esophageal and gastric varices is avoidance of
bleeding and hemorrhage.
o Transjugular intrahepatic portosystemic shunt (TIPS) is a nonsurgical procedure
in which a tract (shunt) between the systemic and portal venous systems is created
to redirect portal blood flow.
o Management of hepatic encephalopathy is focused on reducing ammonia
formation and treating precipitating causes.
An important nursing focus is the prevention and early treatment of cirrhosis.
If the patient has esophageal and/or gastric varices in addition to cirrhosis, the nurse
observes for any signs of bleeding from the varices (e.g., hematemesis, melena).
The focus of nursing care of the patient with hepatic encephalopathy is on maintaining a
safe environment, sustaining life, and assisting with measures to reduce the formation of
Fulminant hepatic failure, or acute liver failure, is a clinical syndrome characterized by
severe impairment of liver function associated with hepatic encephalopathy.
Indications for liver transplant include chronic viral hepatitis, congenital biliary
abnormalities (biliary atresia), inborn errors of metabolism, hepatic malignancy (confined
to the liver), sclerosing cholangitis, fulmin