The disease occurs more often in men. The incidence is 1 out of 10,000 people. This disorder is rare in children. Endoscopic balloon dilation of strictures may relieve symptoms. Liver transplantation prior to liver failure is a possibility in selected cases.
Prognosis is poor, most persons survive just a few years after the symptoms develop.
Call your health care provider if persistent jaundice and itching are present. This illustration shows the major organs of the digestive system. Their relative sizes and positioning in the body are also demonstrated.