Tiopronindosage

The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day. The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day. Avoid dosages greater than 50 mg/kg per day in pediatric patients. Measure urinary cystine 1 month after initiation of VENXXIVA and every 3 months thereafter. Administer VENXXIVA in 3 divided doses at the same times each day, without food.

2.1 Recommended

Dosage Adults: The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day.

Pediatrics: The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day. Avoid dosages greater than 50 mg/kg per day in pediatric patients. Administer VENXXIVA in 3 divided doses at the same times each day, without food. Consider starting VENXXIVA at a lower dosage in patients with history of severe toxicity to d-penicillamine. Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.

2.3 Monitoring

Measure urinary cystine 1 month after starting VENXXIVA and every 3 months thereafter. Adjust VENXXIVA dosage to maintain urinary cystine concentration less than 250 mg/L. Assess for proteinuria before treatment and every 3 to 6 months during treatment. Discontinue VENXXIVA in patients who develop proteinuria, and monitor urinary protein and renal function. Consider restarting VENXXIVA treatment at a lower dosage after resolution of proteinuria.