Tiopronin
Tiopronin Uses, Dosage, Side Effects, Food Interaction and all others data.
Tiopronin is a prescription thiol drug used primarily in the treatment of severe homozygous cystinuria. Patients with cystinuria excrete high levels of cystine in their urine and are at risk for kidney stone formation. Tiopronin is used as a second-line therapy to control the rate of cystine precipitation and excretion, and prevent kidney stone formation. It is used after a failure of the non-pharmacological first line treatment consisting of increased fluid intake, restriction of sodium and protein, and urinary alkalinization. As cystinuria is a relatively rare disease, tiopronin is classified as an orphan drug and is not patented in the United States. It is similar to d-penicillamine in use and efficacy, but offers the advantage of far less adverse effects. Tiopronin is dosed on an individual basis using close monitoring of urinary cystine concentrations and urinary output.
Tiopronin may also be used to bind metal nanoparticles in Wilson's disease, which is an overload of copper in the body. It has been investigated for use in the treatment of arthritis and as a neuroprotective agent in aneurysmal subarachnoid hemorrhage.
| Trade Name | Tiopronin |
| Availability | Prescription only |
| Generic | Tiopronin |
| Tiopronin Other Names | Tiopronin, Tiopronine, Tiopronino, Tioproninum |
| Related Drugs | captopril, Capoten, penicillamine, Cuprimine, Depen, Thiola |
| Weight | 100mg, 300mg, 100mg |
| Type | Oral delayed release tablet, oral tablet |
| Formula | C5H9NO3S |
| Weight | Average: 163.19 Monoisotopic: 163.030314328 |
| Protein binding | Tiopronin undergoes extensive protein binding in plasma. It is thought that this occurs through the formation of a disulphide bridge to the free thiol group of albumin. |
| Groups | Approved, Investigational |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | United States |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
Tiopronin is a thiol agent indicated for the prevention of kidney stone formation in patients with severe homozygous cystinuria.
Tiopronin is indicated for the prevention of kidney stone formation in patients with severe homozygous cystinuria consisting of a urinary cystine concentration greater than 500 mg/day, and who have failed treatment with non-pharmacological measures of increased fluid intake, decreased sodium and protein intake, and urine alkalinization.
Tiopronin is also used to associated treatment for these conditions: Kidney stone formation
How Tiopronin works
Kidney stones form when the solubility limit is exceeded and urine becomes supersaturated with endogenous cystine. Tiopronin is an active reducing agent which undergoes a thiol-disulfide exchange with cystine to form a water-soluble mixed disulfide complex. Thus, the amount of sparingly soluble cystine is reduced. By reducing urinary cystine concentrations below the solubility limit, tiopronin helps reduce cystine stone formation.
Toxicity
Long-term carcinogenicity and mutagenicity studies have not been performed with tiopronin. In experimental animal studies, high doses of tiopronin were shown to interfere with the maintenance of pregnancy and viability of a fetus. No neural tube defects were detected when tiopronin was given to mice and rats in doses up to 10 times the highest recommended human dose. However, the manufacturer does not rule out the possibility of teratogenicity, as it has been seen with the drug d-penicillamine, which acts with a similar mechanism to tiopronin. Tiopronin is not recommended for use in breastfeeding mothers and has no established safety in children 9 years old or younger. There have been case reports of tiopronin-related nephropathy.
Food Interaction
- Take at the same time every day. Take each dose at the same time every day.
- Take on an empty stomach. Take tiopronin at least 1 hour before or 2 hours after eating food.
Tiopronin Disease Interaction
Volume of Distribution
The volume of distribution of tiopronin is high at 455 L, indicating that a large portion of the drug is bound to tissues outside plasma.
Elimination Route
Tiopronin undergoes slow absorption, reaching peak plasma concentration 3-6 hours after ingestion. In a study of healthy subjects, the bioavailability of total and unbound tiopronin was found to be 63% and 40%, respectively.
Half Life
Tiopronin has a long terminal half life of 53 hours in healthy subjects. However, the unbound drug fraction of tiopronin is eliminated much more rapidly from plasma with a calculated half life of 1.8 hours.
Clearance
Total renal clearance for the total and unbound fractions of tiopronin were found to be 3.3 and 13.3 L/h respectively.
Elimination Route
Tiopronin is 100% excreted in urine.
Innovators Monograph
You find simplified version here Tiopronin
