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What is Tiopronin
Tiopronin is an oral agent, also known as alpha-mercaptopropionylglycine (alpha-MPG), that is used to prevent cystine kidney stones.
It is used for the prevention of nephrolithiasis due to severe homozygous cystinuria in combination with high fluid intake, alkali, and diet modification in adult and pediatric patients who are not responsive to these measures alone.
Similar to d-penicillamine, tiopronin forms a more water-soluble complex with cystine, resulting in a decrease in the amount of sparingly soluble cystine in the urine.
Compared to d-penicillamine, tiopronin is associated with fewer and less severe adverse reactions.
However, serious adverse reactions and toxicity related to tiopronin are more likely to appear if the patient has a history of toxicity to d-penicillamine.
Brand Name
- Thiola
- Thiola EC
Indications
- cystinuria
- nephrolithiasis
For the prevention of cystine nephrolithiasis due to severe homozygous cystinuria, in combination with high fluid intake, alkali, and diet modification in patients who are not responsive to these measures alone
Side Effects
- abdominal pain
- anemia
- anorexia
- arthralgia
- asthenia
- back pain
- chest pain (unspecified)
- cough
- dehydration
- diarrhea
- dizziness
- dysgeusia
- dyspepsia
- dysphagia
- ecchymosis
- elevated hepatic enzymes
- eructation
- fatigue
- fever
- flank pain
- flatulence
- gastroesophageal reflux
- glomerulonephritis
- headache
- heart failure
- hyperhidrosis
- hypoesthesia
- impotence (erectile dysfunction)
- jaundice
- lymphadenopathy
- malaise
- musculoskeletal pain
- myalgia
- nausea
- nephrotic syndrome
- oral ulceration
- pemphigus
- peripheral edema
- proteinuria
- pruritus
- rash
- renal failure (unspecified)
- skin irritation
- urticaria
- vertigo
- vomiting
- weakness
- weight gain
- xerosis
- xerostomia
Monitoring Parameters
- CBC with differential
- LFTs
- serum creatinine/BUN
- urinalysis
- urinary cystine concentrations
Contraindications
- breast-feeding
- children
- infants
- neonates
- pregnancy
- proteinuria
Interactions
- Ethanol
Ethanol: (Moderate) Avoid alcohol consumption 2 hours before and 3 hours after taking tiopronin EC. Tiopronin is released faster from tiopronin EC in the presence of alcohol; the risk for adverse events associated with tiopronin EC when taken with alcohol is unknown.
