Indications Indications

For your patients managing recurring cystine stone events, consider new THIOLA EC

If your patient is still experiencing recurring stones under his or her current treatment plan, THIOLA EC may help.

INDICATIONS & USAGE: THIOLA EC is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients ≥20 kg with severe homozygous cystinuria, who are not responsive to these measures alone.

Efficacy Efficacy

71% of patients achieved stone prevention with tiopronin in a multi-center clinical trial1

Efficacy results in patients with cystinuria1

Naïve to d‑penicillamine
(n=14)

Stopped new stone formation

71%

Reduced rate of new stone formation

94%

 

In patients previously treated with d-penicillamine (n=43)1:

  • 62% stopped forming new stones
  • 81% reduced the rate of new stone formation

Study design: Sixty-six cystine stone-formers (mean age 32.0±13.3 years), with 1 or more stone episodes (spontaneous passage, surgical removal, or appearance on X-ray) in the prior 2 years, were enrolled at 12 study sites. Forty-nine patients had been previously treated with d-penicillamine (at a mean dosage of 1125±640 mg/day for a mean of 2.81±3.94 years) and 17 patients had no prior d-penicillamine therapy. All patients maintained ongoing dietary and fluid regimens, and 45/66 patients continued ongoing alkali therapy. Patients received tiopronin for between 4 months and 4 years at a mean dosage of 1193±450 mg/day, in 3 to 4 divided doses ≥1 hour before or 2 hours after meals.1

 

Mechanism of action Mechanism of action

THIOLA EC may help prevent stones by binding to cystine2

Prevention of stones requires removal of excess cystine

Tiopronin increases solubility of cystine

Tiopronin undergoes thiol-disulfide exchange with cystine, forming a more soluble complex.

Tiopronin-cysteine complex is excreted in the urine, reducing the possibility of stone formation.

Safety Safety

Adverse reactions (≥5%) occurring in a multi-center trial of 66 cystine stone-formers treated with THIOLA1,2

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Postmarketing adverse reactions reported2

organ class
THIOLA dosing

Routinely monitor cystine levels with 24-hour urine tests to find the right dosage for your patients.1,5

THIOLA EC dosing

References:  1. Pak et al. J Urol. 1986;136(5):1003-1008.  2. THIOLA EC [package insert]. San Antonio, TX: Mission Pharmacal Company.  3. Pareek et al. J Urol. 2005;174(6):2250-2252.  4. Pearle et al. J Urol. 2014;192(2):316-324.  5. Knoll et al. Pediatr Nephrol. 2005;20(1):19-24.

INDICATIONS AND USAGE: THIOLA® EC (tiopronin) delayed-release tablets is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients ≥20 kg with severe homozygous cystinuria, who are not responsive to these measures alone.

Important Safety Information

CONTRAINDICATIONS: THIOLA EC is contraindicated in patients with hypersensitivity to tiopronin or any other components of THIOLA EC.

WARNINGS AND PRECAUTIONS:

  • Proteinuria: Proteinuria, including nephrotic syndrome, and membranous nephropathy, has been reported with tiopronin use. Pediatric patients receiving >50 mg/kg of tiopronin per day may be at increased risk for proteinuria. Monitor patients for the development of proteinuria and discontinue therapy in patients who develop proteinuria.
  • Hypersensitivity Reactions: Hypersensitivity reactions (drug fever, rash, fever, arthralgia and lymphadenopathy) have been reported.

ADVERSE REACTIONS: The most common adverse reactions (≥10%) are nausea, diarrhea or soft stools, oral ulcers, rash, fatigue, fever, arthralgia, proteinuria, and emesis.

DRUG INTERACTIONS: Avoid alcohol consumption 2 hours before and 3 hours after taking THIOLA EC as THIOLA EC is released faster in the presence of alcohol.

SPECIFIC POPULATIONS:

  • Lactation: Breastfeeding is not recommended during treatment with THIOLA EC.
  • Geriatric Use: Because elderly patients are more likely to have decreased renal function, care should be taken in dose selection, and it may be useful to monitor renal function.

You may report negative side effects to Retrophin® Medical Information at 1-877-659-5518, or to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see full THIOLA EC Prescribing Information.