tiopronin (Rx)Brand and Other Names:Thiola, Tiopronin DR, more...Tiopronin-K DR

 
 
 

Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

capsule for customizable compounding (Tiopropin DR)

  • 200mg
  • 250mg

capsule for customizable compounding plus potassium citrate (Tiopropin-K DR)

  • 150mg/2mEq
  • 150mg/4mEq
  • 250mg/2mEq
  • 250mg/4mEq
more...

Prevention of Nephrolithiasis

Indicated for the prevention of cystine kidney stone formation in patients with severe homozygous cystinuria with urinary cystine >500 mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine

Initial: 800 mg/day PO divided three times daily

Titrate dose to maintain urinary cystine <250 mg/L (average daily dose is ~1000 mg/day)

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

capsule for customizable compounding (Tiopropin DR)

  • 200mg
  • 250mg

capsule for customizable compounding plus potassium citrate (Tiopropin-K DR)

  • 150mg/2mEq
  • 150mg/4mEq
  • 250mg/2mEq
  • 250mg/4mEq
more...

Prevention of Nephrolithiasis

Indicated for the prevention of cystine kidney stone formation in patients with severe homozygous cystinuria with urinary cystine >500 mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine

<9 years: Safety and efficacy have not been established

≥9 years

  • Initial: 5 mg/kg PO TID
  • Titrate dose to maintain urinary cystine <250 mg/L
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Adverse Effects

Frequency Not Defined

Fever

Skin complications

Hypersensitivity reactions

Taste and smell impairment

Generalized rash

GI symptoms

SLE-like syndrome

Hypogeusia

Skin wrinkling

Jaundice

Leukopenia

Thrombocytopenia

Aplastic anemia

Agranulocytosis

Goodpasture's syndrome

Myasthenia gravis

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Warnings

Contraindications

Hypersensitivity, pregnancy (except if benefit outweighs risk), breastfeeding

History of developing tiopronin-induced agraulocystosis, aplastic anemia, thrombocytopenia (except in severe cysteinuria where benefit of inhibited stond formation outweighs risk of treatment breast feeding

Breast feeding

Cautions

Check urinary cystine frequently for first 6 months of treatment to assess efficacy, and every 6 months thereafter

Monitor CBC, Hgb, serum albumin, LFTs, 24 hr urinary protein, routine UA q3-6months

Discontinue if WBC <3500/mm³ or Platelets <100 k/mm³

ADRs are more likely to occur in patients who have shown toxicity to d-penicillamine

Risk of Goodpasture's syndrome possible (discontinue if it occurs)

Risk of myesthenia gravis possible (discontinue if it occurs)

Discontinue if pemphigus-type reaction occurs

Monitor for membranous glomerulopathy (proteinuria may develop)

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Pregnancy & Lactation

Pregnancy Category: C

Lactation: Excretion in breast milk unknown; contraindicated

Pregnancy Categories

A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA:Information not available.

more...
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Pharmacology

Mechanism of Action

Undergoes thiol-disulfide exchange with cystine to form a mixed disulfide of tiopronin-cysteine (more water-soluble), which in turn reduces formation of cycteine calculi

Reduces urinary cystine by 250-350 mg per 1 g drug

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Images

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Formulary

FormularyPatient Discounts

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Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
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4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
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Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
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ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
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Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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