Abstract
In all 8 cases of the nephrotic syndrome occurring in patients with hepatolenticular degeneration (Wilson disease), DL-penicillamine was the offending agent. The renal toxicity of DL-penicillamine was reversible, provided it was recognized in time and administration of the drug was discontinued. Two of these patients tolerated penicillamine (D-peniclllamine) well after recovering from their nephrotic syndrome. The mechanism of nephrotoxicity of DL-penicillamine is unknown.