tiopronin

Action and Spectrum

Mechanism of action

tiopronin is a medication used primarily for the treatment of cystinuria, a genetic disorder in which excess cystine is excreted in the urine, leading to the formation of kidney stones. The exact mechanism of action of tiopronin in cystinuria has yet to be fully understood. Still, it is believed to work by binding to cystine and forming a more soluble compound, thereby preventing the formation of kidney stones

Spectrum

tiopronin has a relatively narrow spectrum of activity and is primarily used to treat cystinuria. It is ineffective against other kidney stones or urinary tract infections. In addition, tiopronin is not adequate for treating other genetic disorders that involve the build-up of amino acids in the body, such as homocystinuria or phenylketonuria

Drug Interaction

Adverse Reaction

Frequency defined:

>10%

Oral ulcers (12-18%)

Nausea (12-25%)

Weakness (4-12%)

Diarrhea/soft stools (6-18%)

Fatigue (14%)

Arthralgia (12%)

Rash (12-14%)

1-10%

Proteinuria wrinkling edema (6%)

Skin (2-6%)

Anorexia pain (6%)

Peripheral (6-10%)

Fever (6%)

Emesis (10%)

Ecchymosis (8%)

Urticaria (6%)

Impotence (6%)

Cough (8%)

Abdominal (6%)

Chest (4-6%)

Anemia (6%)

Pruritus (10%)

Frequency not defined:

Hypogeusia

Reduced taste perception

Post-marketing reports:

Increased weight

Decreased appetite

Aguesia

Dizziness

Headache

Nephrotic syndrome

Renal failure

Pruritis

Skin irritation

Asthenia

Urticaria

Vomiting

Dyspepsia

Pyrexia

vertigo

Congestive heart failure

Black Box Warning

Contraindication / Caution

Contraindications

• tiopronin is contraindicated in hypersensitive individuals tiopronin or any of its components. It is also contraindicated in individuals with a history of penicillamine-induced myasthenia gravis, or other autoimmune disorders
• tiopronin should not be used during pregnancy, as it has been shown to cross the placenta and may harm the developing fetus. It is also not recommended during breastfeeding, as it is unknown whether tiopronin is excreted in breast milk.

Caution

• tiopronin should be used cautiously in individuals with a history of liver or kidney disease, as it may worsen these conditions. It is important to monitor liver and kidney function regularly during treatment with tiopronin to ensure that these organs function properly
• It may cause gastrointestinal disturbances, such as nausea, vomiting, and diarrhea. These side effects may be reduced by taking tiopronin with food or dividing the dose into smaller, more frequent daily doses. Drinking plenty of fluids while taking tiopronin is essential to prevent dehydration

Pregnancy / Lactation

Pregnancy consideration: It should not be used during pregnancy, as it has been shown to cross the placenta and may harm the developing fetus

Lactation: Excretion of the drug in human breast milk is unknown

Pregnancy category:

Category A: well-controlled and Satisfactory studies show no risk to the fetus in the first or later trimester.

Category B: there was no evidence of risk to the fetus in animal studies, and there were not enough studies on pregnant women.

Category C: there was evidence of risk of adverse effects in animal reproduction studies, and no adequate evidence in human studies must take care of potential risks in pregnant women.

Category D: adequate data with sufficient evidence of human fetal risk from various platforms, but despite the potential risk, and used only in emergency cases for potential benefits.

Category X: Drugs listed in this category outweigh the risks over benefits. Hence these categories of drugs need to be avoided by pregnant women.

Category N: There is no data available for the drug under this category

Pharmacology

Pharmacology

tiopronin is a sulfhydryl-containing medication used primarily for treating cystinuria, a genetic disorder in which excess cystine is excreted in the urine, leading to the formation of kidney stones

The exact mechanism of action of tiopronin in cystinuria is not fully understood. Still, it is believed to work by binding to cystine and forming a more soluble compound, thereby preventing the formation of kidney stones.

Pharmacodynamics

tiopronin is a medication that works primarily by binding to cystine and forming a more soluble compound, thereby preventing the formation of kidney stones in individuals with cystinuria

tiopronin has been shown to reduce urinary cystine levels and increase the solubility of cystine in the urine, which helps prevent kidney stones’ formation. The exact mechanism of action of tiopronin in cystinuria is not fully understood. Still, it is believed to involve the formation of a disulfide bond between cysteine and tiopronin, which makes cysteine more soluble

Pharmacokinetics

Absorption

It is well-absorbed after oral administration and reaches peak plasma concentrations within 1-2 hours

Distribution

It is widely distributed throughout the body, and the active metabolites are primarily excreted in the urine

Metabolism

It is rapidly metabolized in the liver to form active and inactive metabolites

Elimination and Excretion

The active metabolites of tiopronin are primarily excreted in the urine, where they bind to cystine and prevent the formation of kidney stones. The kidneys primarily eliminate tiopronin, and dosage adjustments may be necessary for individuals with renal impairment

Adminstartion

Administration

tiopronin is usually taken orally, as a tablet or capsule. The recommended dose and frequency of tiopronin depend on the patient’s age, weight, medical condition, and response to treatment

Patient Information Leaflet

Patient information leaflet

Generic Name: tiopronin

Pronounced: [ tye-OH-proe-nin ]

Why do we use tiopronin?

tiopronin is a medication primarily used to prevent and treat kidney stones and cystinuria.

• Rheumatoid arthritis: tiopronin has been used as a disease-modifying antirheumatic drug (DMARD) in treating rheumatoid arthritis, a chronic inflammatory disorder that affects the joints
• HIV-associated nephropathy: tiopronin has been investigated as a potential treatment for HIV-associated nephropathy, a kidney disease that affects some people with HIV
• Cysteamine toxicity: tiopronin has been used to treat cysteamine toxicity, a side effect of cysteamine, which is used to treat a rare genetic disorder called cystinosis
• Acetaminophen overdose: tiopronin has been studied as a potential treatment for acetaminophen overdose, a common cause of liver failure
• Wilson’s disease: tiopronin has been used to treat Wilson’s disease, a rare genetic disorder that causes a build-up of copper in the body