Hepato-Lenticular Degeneration (Wilson’s Disease) Treated by Penicillamine

Effect of penicillamine on serum iron.

“…On the other hand all patients showed a progressive and highly significant fall in the serum copper levels, and these observations, taken in conjunction with findings of a fall in the serum caeruloplasmin reported elsewhere (Walshe, 1964), leave little doubt that this treatment is effective in depleting the abnormal body stores ofcopper which are found in this disease. Failure to achieve and maintain such a fall in the serum copper and caeruloplasmin (cf., Richmond et al, 1964) probably means inadequate maintenance dosage of D-penicillamine and re-accumulation of excess copper stores. It is interesting that, as with iron, penicillamine does not appear to be able to deplete the body of copper for normal requirements of caeruloplasmin formation (Walshe, 1964) or in the formation of hair (Gibbs and Walshe, 1965).…”

“…However, there are no published reports to suggest that this precept has been followed, and none of the reports on the use of penicillamine in the treatment of Wilson's disease have included any data on serum iron levels (Sternlieb and * Dose in rats was 300 mg./kg., equivalent to approximately 20 g./day for an adult patient. 651 Scheinberg, 1964;Richmond, Rosenoer, Tompsett, Draper, and Simpson, 1964;Goldstein, Randall, Gross, Rosevear, and McGuckin, 1962;Walshe, 1960). We have therefore investigated the effect of maintenance doses of penicillamine, given to patients with Wilson's disease, on the concentration of serum iron, iron-binding capacity, and haemoglobin formation, and compared these with the changes in the serum copper and clinical status of the patients.…”