Penicillamine and Cystinuria

Further Observations on Use of D-penicillamine in Cystinuria.

It was shown in a previous communication (Crawhall et al., 1963) that the abnormally high level of cystine excretion found in homozygotes for cystinuria could be reduced for short periods by the administration of D-penicillamine. This paper reports the results of administering the drug continuously to a group of cystinuric patients for periods of five months to one year. We have used the D-isom...

The use of D-penicillamine in cystinuria: efficacy and untoward reactions.

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. Fifty-six individuals 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 sec...

New Agent in the Treatment of Cystinuria: N-acetyl-D-penicillamine.

Cystinuria and renal transplantation.

Cystinuria—Diagnosis and Management

Cystinuria is an autosomal recessive disorder of cystine and dibasic amino acid transport across the luminal membrane of proximal tubule and small intestine. Two responsible genes have been identified: mutations in the SLC3A1 gene, located on the chromosome 2p, cause cystinuria type I, while variants in SLC7A9 have been demonstrated in nontype I cystinuria. The poor urinary solubility of cystin...