THE USE OF D-PENICILLAMINE IN CYSTINURIA - EFFICACY AND UNTOWARD REACTIONS

  • 1 January 1981
    • journal article
    • research article
    • Vol. 54  (6) , 439-446
Abstract
A retrospective study was conducted to assess the efficacy of D-penicillamine in the management of cystinuria, as well as to define the frequency and nature of untoward reactions to this drug. Individuals (56) were identified who, by stone analysis and/or biochemical studies, met the accepted diagnostic criteria for phenotypic cystinuria. The majority of these patients presented in the 2nd decade of life with evidence of stone formation: renal colic, hematuria and/or stone passage. Thirty-five individuals were considered to have clinically advanced cystinuria because they had required at least 1 urinary tract lithotomy. In these advanced cases, frequency of subsequent lithotomies and episodes of renal colic per 100 patient-yr of observation were used as indices to measure the efficacy of D-penicillamine treatment. By both measurements, D-penicillamine significantly improved the clinical course of patients. The incidence of acute drug sensitivity reactions (rash, fever and/or arthropathy) was in excess of 40%. Delayed drug-induced proteinuria occurred in 34% of treated patients. Evidently D-penicillamine is useful in the treatment of cystinuria. Because of the significant number of untoward drug reactions, the drug should be instituted only in selected high-risk patients.