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Low Dose D-penicillamine in cystinuria.
Proceedings of the Royal Society of Medicine
|January 1, 1977
Summary
A single nightly dose of D-penicillamine effectively prevents cystine stone recurrence by maintaining low urinary cystine levels. This regimen is recommended for prevention, not dissolution, of cystine stones.
Area of Science:
- Nephrology
- Urology
- Pharmacology
Background:
- Cystinuria is a genetic disorder leading to cystine kidney stones.
- Preventing cystine stone recurrence is crucial for patient management.
- Optimizing D-penicillamine dosage and timing is key to therapeutic success.
Observation:
- A single 750 mg dose of D-penicillamine at 2200 h, with high fluid intake during the day, maintained urinary cystine below saturation.
- This regimen primarily reduced cystine levels in overnight urine (0200-0800 h).
- Total daily cystine excretion was not significantly reduced by this single-dose regimen.
Findings:
- A single, low-dose (≤750 mg) nightly administration of D-penicillamine is sufficient to prevent urinary cystine supersaturation.
- The therapeutic effect is most pronounced during the overnight urine voiding period.
- This dosing strategy does not substantially alter overall daily cystine excretion.
Implications:
- This D-penicillamine regimen is provisionally recommended for preventing recurrent cystine nephrolithiasis.
- It is not suitable for promoting the dissolution of existing cystine stones.
- Higher D-penicillamine doses in divided schedules remain indicated for cystine stone dissolution.