Copper Chelation in Patients with Wilson's Disease<subtitle>A COMPARISION OF PENICILLAMINE AND TRIETHYLENE TETRAMINE DIHYDROCHLORIDE</subtitle>

Abstract

A comparison has been made of the ability of penicillamine and triethylene tetramine 2HCl to mobilize copper from patients with Wilson's disease. Eighteen patients have been studied and the results correlated with their previous therapeutic history. In patients who have had little or no previous treatement with penicillamine both chelating agents will induce a very large cupruresis which is presumably related to the size of the abnormal body pool of the metal. In patients who have been effectively ‘decoppered’ by prolonged treatment, the cupruretic responce is less and this diminution is more marked in the case of triethylene tetramine. These studies have been confirmed by studies with radioactive copper. The cupruresis induced by penicillamine is associated with a fall in the concentration of copper and of radiocopper in the serum. By contrast triethylene tetramine causes the serum copper concentration to rise, only returning to base line at five hours. It is suggested that these two chelating agents may mobilize copper from different body compartments. The size of the cupruresis induced by triethylene tetramine in untreated patients with Wilson's disease suggests that this compound may have a place in the management of this disease, particularly in patients who have developed toxic reactions to penicillamine.