Generic Name
Tiopronin
Brand Names
Venxxiva, Thiola
FDA approval date: August 11, 1988
Classification: Reducing and Complexing Thiol
Form: Tablet
What is Venxxiva (Tiopronin)?
Tiopronin Delayed-Release Tablets are indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients 20 kg and greater with severe homozygous cystinuria, who are not responsive to these measures alone. Tiopronin Delayed-Release Tablets are a reducing and complexing thiol indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients 20 kg and greater with severe homozygous cystinuria, who are not responsive to these measures alone.
Approved To Treat
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Brand Information
VENXXIVA (tiopronin)
1INDICATIONS AND USAGE
VENXXIVA is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients 9 years of age and older with severe homozygous cystinuria, who are not responsive to these measures alone.
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.
2DOSAGE FORMS AND STRENGTHS
Tablets for oral use:
3CONTRAINDICATIONS
VENXXIVA is contraindicated in patients with hypersensitivity to tiopronin or any other components of VENXXIVA
4ADVERSE REACTIONS
Thefollowing adverse reactions are discussed in greater detail in othersections of the labeling:
- Proteinuria
- Hypersensitivity
4.1Clinical Trials Experience
Because clinical trials are conducted under widely varying conditions, the adverse reaction rates observed in the clinical trials of the drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice.
Adverse reactions occurring at an incidence of ≥5% in an uncontrolled trial in 66 patients with cystinuria age 9 to 68 years are shown in the table below. Patients in group 1 had previously been treated with d-penicillamine; those in group 2 had not. Of those patients who had stopped taking d-penicillamine due to toxicity (34 out of 49 patients in group 1), 22 were able to continue treatment with tiopronin. In those without prior history of d-penicillamine treatment, 6% developed reactions of sufficient severity to require tiopronin withdrawal.
Table 1 presents adverse reactions ≥5% in either treatment group occurring in this trial.
Table 1: Adverse Reactions Occurring in One or More Patients
Taste Disturbance
A reduction in taste perception may develop. It is believed to be the result of chelation of trace metals by tiopronin. Hypogeusia is often self-limited.
4.2Postmarketing Experience
Adverse reactions have been reported from the literature, as well as during post-approval use of tiopronin. Because the post-approval reactions are reported voluntarily from a population of uncertain size, it is not always possible to reliably estimate their frequency or establish a causal relationship to tiopronin exposure.
Adverse reactions reported during the postmarketing use of tiopronin are listed by body system in
Table 2: Adverse Reactions Reported for Tiopronin Pharmacovigilance by System Organ Class and Preferred Term
5OVERDOSAGE
There is no information on overdosage with tiopronin.
6DESCRIPTION
VENXXIVA (tiopronin) delayed-release tablets are a reducing and cystine-binding thiol drug (CBTD) for oral use. Tiopronin is N‑(2‑Mercaptopropionyl) glycine and has the following structure:
Tiopronin has the empirical formula C5H9NO3S and a molecular weight of 163.19. In this drug product tiopronin exists as a dl racemic mixture.
Tiopronin is a white to off-white color crystalline powder, which is freely soluble in water.
Each VENXXIVA tablet contains 100 or 300 mg of tiopronin. The inactive ingredients in VENXXIVA tablets include lactose monohydrate, low substitute hydroxypropyl cellulose, hydroxypropyl cellulose, magnesium stearate, hypromellose 2910, methacrylic acid: ethyl acrylate copolymer (Eudragit L 100-55), talc, triethyl citrate. The imprinting ink contains shellac, ferrosoferric oxide and propylene glycol.
7HOW SUPPLIED/STORAGE AND HANDLING
100 mg delayed-release, white to off-white, round shaped, enteric-coated tablets imprinted with "1A" with black ink on one side and plain on other side, free from physical defects.
300 mg delayed-release, white to off-white, round shaped, enteric-coated tablets imprinted with "3A" with black ink on one side and plain on other side, free from physical defects.
Store at 25°C (77°F); excursions permitted between 15°C and 30°C (59°F and 86°F)
8PATIENT COUNSELING INFORMATION
Lactation
Advise women that breastfeeding is not recommended during treatment with VENXXIVA
Manufactured for:
Torrent Pharmaceuticals Limited, India
Distributed by:
Cycle Pharmaceuticals Ltd, Cambridge, CB3 0FA, UK
Cycle Pharmaceuticals Ltd, Cambridge, CB3 0FA, UK
Revised: December 2024
9PRINCIPAL DISPLAY PANEL
100 mg - 300 Tablets
300 mg - 90 Tablets