Wilson disease: symptomatic liver therapy

Abstract

Wilson disease leads to symptomatic impairment of liver function or liver cirrhosis. Strict adherence to decoppering agents is essential in these patients. Secondary prevention of additional hepatic damage by avoidance of other toxic substances (e.g., alcohol, drugs) and sufficient calorie intake is recommended. Routine examinations in cirrhotic patients include screening for signs of portal hypertension (esophagus varices), development of ascites, and hepatic encephalopathy. Where varices are present, primary or secondary preventive interventions may include treatment with nonselective beta-blockers or variceal ligation, similar to the approach in patients with liver cirrhosis due to other etiologies. For patients presenting with ascites, diuretics are the treatment of choice. Spontaneous bacterial peritonitis can be diagnosed by paracentesis and should be treated with antibiotics. Liver cirrhosis can also lead to accumulation of neurotoxins causing hepatic encephalopathy. It is characterized by unspecific neuropsychiatric impairment and is treated with laxatives and nonresorbable antibiotics. The best prophylaxis is regular defecation. Patients with liver cirrhosis are susceptible for bacterial infections of any cause and sepsis is one of the leading causes of death in these patients. In advanced stages of cirrhosis renal function impairment is a common feature. The hepatorenal syndrome shows a high mortality. Where Wilson disease patients have decompensated liver cirrhosis, liver transplantation should be evaluated.

Keywords: Wilson disease; acute liver failure; ascites; esophageal varices; hepatic encephalopathy; liver cirrhosis.