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...

D-Cysteine differs from the antiarthritis drug D-penicillamine by only two methyl groups on the beta-carbon yet inhibits carboxypeptidase A (CPD) by a distinct mechanism: D-cysteine binds tightly to the active site zinc, while D-penicillamine catalyzes metal removal. To investigate the structural basis for this difference, we solved the crystal structure of carboxypeptidase A complexed with D-c...

tricho-dento-osseous syndrome is a rare autosomal dominant disorder. immunosuppressive drugs, though critical to the treatment, have undesirable effects on patient's healing process. this is a case report of a 10-year-old female, suffering from tricho-dento-osseous syndrome who was under treatment with penicillamine. as the dental treatment was sought too late, both central and lateral incisors...

Sodium bis(L-cysteinato)zincate(II) hexahydrate (1) and bis(0-ethvl-L-cysteinato)zinc(II) (2) have been prepared from respectively alkaline (pH > 1 1 ) and neutral aqueous solutions and their structures determined by X-ray structural analysis. N ,S-coordination of the Zn atoms with the formation of two five-membered chelate rings is observed for both com plexes. The deprotonated carboxylate gro...

The aim of this study was to investigate the effectiveness of a high-dose zinc sulfate and low-dose D-penicillamine combination in the treatment of pediatric Wilson's disease (WD). A retropective chart review of 65 patients with WD was conducted. These patients received D-penicillamine (8-10 mg/kg/day) and zinc sulfate as the primary treatment. The pediatric dose of elemental zinc is 68-85 mg/d...

Background and Aim: Long-term D-Penicillamine (D-Pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective study was conducted aim to evaluate incidence relapse following withdrawal D-Pen from Combination (D-Pen + Zinc) maintenance phase previously symptomatic hepatic WD. Methods: Hepatic WD patients <...