Q J Med 1971; 40: 355-370
© 1971 Association of Physicians
research-article |
The Treatment of Cystinuria with N-acetyl-D-penicillamine, a Comparison with the Results of D-penicillamine Treatment
From the Medical Professorial Unit, Dunn Laboratories St. Bartholomew's Hospital, London, E.C. 1 The Medical Research Council Clinical Research Centre Harrow, Middlesex
1 Present address: Medical Research Council, Clinical Research Centre, Watford Road, Harrow, Middlesex. HAl 3UJ.
Received 21 September 1970 The results of using N-acetyl-D-penicillamine for the treatment of cystinuria for periods of between 7 months and 2·75 years are reported and compared with those obtained when D-penicillamine is employed.
Both drags reduce the urinary excretion of cystine and their effective doses are similar.
The main side effects encountered were an acute hypersensitivity reaction and proteinuria which developed later and which could not be related to the presence of calculi or infection. Only one mild acute hypersensitivity reaction occurred in the nine patients who took N-acetyl-D-penicillamine. Ten of the 19 patients who were treated with D-penicillamine developed more severe acute hypersensitivity reactions. Desensitization to the drug could, however, be accomplished under cover of prednisolone.
Proteinuria occurred with about equal frequency and severity with both drugs and although it may be heavy, it does not appear to make the renal prognosis markedly worse. Some of the patients with heavy proteinuria also developed oedema and hypoalbuminaemia, but all of these manifestations subsided when the drug was discontinued, a short course of corticosteroid treatment being given in some cases. It was possible to continue treatment in patients with a relatively mild degree of proteinuria in whom there was a very strong indication for doing so.