Tiopronin: Difference between revisions

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'''Tiopronin''' is a [[prescription medication]] used primarily in the treatment of [[cystinuria]], a rare inherited disorder that causes a buildup of the amino acid [[cystine]] in the kidneys and bladder, leading to the formation of [[cystine stones]]. Tiopronin, sold under the brand name [[Thiola]], works by reducing the amount of cystine in the urine, thereby preventing the formation of these stones.
{{Short description|A medication used to treat cystinuria}}
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==Pharmacology==
'''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.
Tiopronin is a [[thiol]], a type of organic compound that contains a sulfur-hydrogen group. In the body, tiopronin binds to cystine to form a more soluble compound, which can be excreted more easily in the urine. This reduces the concentration of cystine in the urine and prevents the formation of cystine stones.


==Uses==
==Mechanism of Action==
The primary use of tiopronin is in the treatment of cystinuria. It is typically used when other treatments, such as dietary changes and increased fluid intake, are not sufficient to prevent the formation of cystine stones. In some cases, tiopronin may also be used to dissolve existing cystine stones.
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==
==Side Effects==
Like all medications, tiopronin can cause side effects. The most common side effects include [[nausea]], [[vomiting]], [[diarrhea]], and [[abdominal pain]]. In rare cases, tiopronin can cause more serious side effects, such as [[skin rash]], [[fever]], and [[joint pain]], which may be signs of a severe allergic reaction.
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==
==Precautions==
Before starting treatment with tiopronin, patients should inform their healthcare provider about any other medications they are taking, as tiopronin can interact with other drugs. Patients should also inform their healthcare provider if they have any other medical conditions, especially liver or kidney disease.
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.


==See Also==
==Related pages==
* [[Cystinuria]]
* [[Cystinuria]]
* [[Kidney stones]]
* [[Cystine]]
* [[Prescription medication]]
* [[Chelating agent]]
* [[Thiol]]
* [[D-penicillamine]]


[[Category:Medications]]
[[Category:Thiol drugs]]
[[Category:Thiols]]
[[Category:Urologic drugs]]
[[Category:Prescription medication]]
{{Medicine-stub}}

Latest revision as of 03:54, 13 February 2025

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]