Thiola

Thiola Uses, Dosage, Side Effects, Food Interaction and all others data.

Thiola 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. Thiola 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. Thiola is dosed on an individual basis using close monitoring of urinary cystine concentrations and urinary output.

Thiola 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.

Thiola
Uses
Dosage
Side Effect
Precautions
Interactions
Uses during Pregnancy
Uses during Breastfeeding
Accute Overdose
Food Interaction
Half Life
Volume of Distribution
Clearance
Interaction With other Medicine
Contradiction
Storage

Trade Name	Thiola
Availability	Prescription only
Generic	Tiopronin
Tiopronin Other Names	Tiopronin, Tiopronine, Tiopronino, Tioproninum
Related Drugs	captopril, Capoten, penicillamine, Cuprimine, Depen, Thiola
Weight	100mg, 300mg,
Type	Tablet, Sugar Coated, 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

Thiola is a thiol agent indicated for the prevention of kidney stone formation in patients with severe homozygous cystinuria.

Thiola 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.

Thiola is also used to associated treatment for these conditions: Kidney stone formation

How Thiola works

Kidney stones form when the solubility limit is exceeded and urine becomes supersaturated with endogenous cystine. Thiola 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. Thiola 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.

Thiola Disease Interaction

Moderate: liver disease, renal dysfunction

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

Thiola 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

Thiola 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.