Tiopronin (5 brand names, 2 dosage forms, 1 route)
Available dosage forms: TABLET, TABLET, DELAYED RELEASE.
Routes of administration: ORAL.
Brand Names
- THIOLA
- THIOLA EC
- TIOCYSTIN
- TIOPRONIN
- VENXXIVA
Indications 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.
Contraindications
VENXXIVA is contraindicated in patients with hypersensitivity to tiopronin or any other components of VENXXIVA [see Warnings and Precautions (5.2)].
Adverse Reactions
The following adverse reactions are discussed in greater detail in other sections of the labeling: Proteinuria [see Warnings and Precautions (5.1)] Hypersensitivity [see Warnings and Precautions (5.2)]
Mechanism of Action
The goal of therapy is to reduce urinary cystine concentration below its solubility limit. Tiopronin is an active reducing agent which undergoes thiol-disulfide exchange with cystine to form a mixed disulfide of tiopronin-cysteine. From this reaction, a water-soluble mixed disulfide is formed and the amount of sparingly soluble cystine is reduced.
Overdosage
There is no information on overdosage with tiopronin.