Drug Index



Mechanism :

As an active reducing agent, tiopronin undergoes thiol-disulfide exchange with cystine to form tiopronin-cystine disulfide, which is more water soluble than cystine.

Indication :

• Prevention of kidney stone (cysteine) formation in patients with severe homozygous cystinuria who have urinary cysteine >500 mg/day who are resistant to treatment with high fluid intake, alkali and diet modification, or who have had adverse reactions to penicillamine.

Contraindications :

Prior history of developing agranulocytosis, aplastic anemia, or thrombocytopenia on this medication.

Dosing :

Oral: Children ≥9 years: Initial: 15 mg/kg/day in 3 divided doses. Titrate dose to maintain urinary cysteine <250 mg/L.

Adverse Effect :

Fever, skin complications, hypersensitivity reactions, taste and smell impairment, generalized rash, SLE-like syndrome, hypogeusia, skin wrinkling, jaundice, leukopenia, thrombocytopenia, aplastic anemia, agranulocytosis, Goodpasture’s syndrome, myasthenia gravis.

Interaction :

There are no known significant interactions.

10/23/2019 14:04:43 Tiopronin
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