Wilson's Disease

Gastroenterology

Overview

Definition

Copper overload disorder.

Epidemiology

Varies globally depending on genetics, environment, or exposure.

Etiology & Risk Factors

  • Autosomal recessive mutation in ATP7B gene.

Clinical Symptoms

  • Liver disease
  • Parkinsonian symptoms
  • psychiatric changes
  • Kayser-Fleischer rings.

Clinical Approach

Diagnosis

  • Low serum ceruloplasmin
  • High 24-hour urine copper.

Management

  • Copper chelators (Penicillamine
  • Trientine)
  • Zinc.

Complications

  • Fulminant hepatic failure
  • severe neurologic damage.