8. Inherited Diseases Affecting the Liver

Obligatory	Refer to the entry for the condition. If there is no specific entry, refer to a DCSO.
Discretionary	a) If the donor is well and stable on treatment for Wilson’s Disease, refer to DCSO. b) If the donor has Gilbert’s Syndrome, accept.
See if Relevant	Inherited Diseases
Additional Information	Wilson’s disease is caused by an excessive accumulation of copper in the liver and other organs. e.g. brain. If diagnosed and treated early with chelating agents, such as Penicillamine and Trientine, and avoidance of high copper foods, the prognosis is good and individuals can lead a normal life. If there is uncertainty about the donor’s health or treatment, refer to a Designated Clinical Support Officer. Alpha-1-antitrypsin deficiency can occasionally cause liver disease in adults. This may lead to liver failure and the need for liver transplantation. Gilbert's syndrome is an inherited defect in bilirubin metabolism. It is harmless but can cause jaundice (yellowing of the whites of the eyes).