Tiopronin

A medication used to treat cystinuria

Tiopronin
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Tiopronin is a medication primarily used in the treatment of cystinuria, a condition characterized by the excessive excretion of cystine in the urine, leading to the formation of cystine stones. It is a thiol compound that acts as a chelating agent, helping to reduce the concentration of cystine in the urine.

Mechanism of Action

Tiopronin works by forming a disulfide bond with cystine, converting it into a more soluble compound called cysteine-tiopronin disulfide. This transformation reduces the likelihood of cystine stone formation by decreasing the concentration of free cystine in the urine.

Medical Uses

Tiopronin is primarily indicated for patients with severe cystinuria who are unable to control their condition through dietary modifications and increased fluid intake alone. It is often used when other treatments, such as D-penicillamine, are not effective or cause adverse effects.

Administration

Tiopronin is administered orally, and the dosage is typically adjusted based on the patient's urinary cystine levels. Regular monitoring of these levels is essential to ensure the effectiveness of the treatment and to adjust the dosage as needed.

Side Effects

Common side effects of tiopronin include gastrointestinal disturbances, such as nausea and vomiting, as well as rash and fatigue. More serious side effects can include hematological abnormalities and liver function changes, necessitating regular monitoring of blood counts and liver enzymes during treatment.

Precautions

Patients taking tiopronin should be monitored for potential side effects, and regular blood tests are recommended to check for any adverse effects on the liver and blood cells. It is also important to maintain adequate hydration to help prevent the formation of cystine stones.

Related pages

• Cystinuria
• Cystine
• Chelating agent
• D-penicillamine