Copper Storage in Hepatolenticular Degeneration and Allied Diseases

Amino-aciduria and copper metabolism in hepatolenticular degeneration.

Hepatolenticular Degeneration (Wilson's Disease)

A R?eport of five cases, with Commentary WILSON'S disease is a heredo-familial disorder, the essential pathological elements of which consist in a cirrhotic state of the liver comb;ned with degenerative changes in the lenticular nuclei of the brain. The term "hepatolenticular degenera-tion" is therefore aptly precise, but affords little clue to the diversity of the clinical manifestations. A lo...

Wilson's disease; hepatolenticular degeneration.

The inclusion of a rare neurological disease in an issue of this journal devoted to disorders of the liver would seem to warrant some explanation for although cirrhosis of the liver is an integral part of the syndrome of Wilson's disease, it accounts for an insignificant proportion of all patients suffering from hepatic cirrhosis. However, although quantitatively unimportant, its significance r...

Metabolic balances of copper in patients with hepatolenticular degeneration submitted to vegetarian and mixed diets.

Besides the use of chelating agents, aiming to produce a rise in cupruresis, a decrease of the positive copper balance in hepatolenticular degeneration has been attempted by means of lowering copper absorption. To this end cation exchange resins and potassium sulphide have been used. Nevertheless, the effectiveness of these drugs is still discussed, some investigators being sceptical (Denny-Bro...

Studies on copper metabolism. XIV. Radioactive copper studies in normal subjects and in patients with hepatolenticular degeneration.