tiopronin (Rx)

Brand and Other Names:Thiola, Tiopronin DR, more...Tiopronin-K DR, Thiola EC
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

tablet, delayed-release (Thiola EC)

  • 100mg
  • 300mg

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

Prevention of Nephrolithiasis

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

Initial: 800 mg/day PO divided TID

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

Consider starting at lower dose with history of severe toxicity to d-penicillamine

Dosing Considerations

Measure urinary cystine 1 month after initiating and every 3 months thereafter

Assess for proteinuria before treatment and every 3-6 months during treatment

Discontinue in patients who develop proteinuria and monitor urinary protein and renal function

Consider restarting treatment at a lower dosage after resolution of proteinuria

Dosage Forms & Strengths

tablet (Thiola)

  • 100mg

tablet, delayed-release (Thiola EC)

  • 100mg
  • 300mg

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

Prevention of Nephrolithiasis

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

<20 kg: Safety and efficacy not established

≥20 kg

  • 15 mg/kg/day PO divided TID; not to exceed 50 mg/kg/day
  • Adjust dose to maintain urinary cystine levels <250 mg/L
  • Consider starting at lower dose with history of severe toxicity to d-penicillamine

Dosing Considerations

Measure urinary cystine 1 month after initiating and every 3 months thereafter

Assess for proteinuria before treatment and every 3-6 months during treatment

Discontinue in patients who develop proteinuria and monitor urinary protein and renal function

Consider restarting treatment at a lower dosage after resolution of proteinuria

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Interactions

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and tiopronin

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            Adverse Effects

            >10%

            Nausea (12-25%)

            Oral ulcers (12-18%)

            Diarrhea/soft stools (6-18%)

            Fatigue (14%)

            Rash (12-14%)

            Arthralgia (12%)

            Weakness (4-12%)

            1-10%

            Emesis (10%)

            Proteinuria (6-10%)

            Fever (8%)

            Anorexia (8%)

            Urticaria (8%)

            Abdominal pain (6%)

            Peripheral edema (6%)

            Chest pain (6%)

            Impotence (6%)

            Cough (6%)

            Ecchymosis (6%)

            Skin wrinkling (6%)

            Pruritus (4-6%)

            Anemia (2-6%)

            Frequency Not Defined

            Reduced taste perception

            Hypogeusia

            Postmarketing Reports

            Cardiac disorders: CHF

            Ear and labyrinth disorders: Vertigo

            Gastrointestinal disorders: Abdominal discomfort, distension. or pain; chapped lips; diarrhea; dry mouth; dyspepsia; eructation; flatulence; gastrointestinal disorder; gastroesophageal reflux disease; nausea; vomiting; jaundice; liver transaminitis

            General disorders and administration site conditions: Asthenia; chest pain; fatigue; malaise; pain; peripheral swelling; pyrexia; swelling

            Investigations: GFR decreased; weight increased

            Metabolism and nutrition disorders: Decreased appetite; dehydration; hypophagia

            Musculoskeletal and connective tissue disorders: Arthralgia; back pain; flank pain; joint swelling; limb discomfort; musculoskeletal discomfort; myalgia; neck pain; pain in extremity

            Nervous system disorders: Ageusia; burning sensation; dizziness; dysgeusia; headache; hypoesthesia

            Renal and urinary disorders: Nephrotic syndrome; proteinuria; renal failure

            Skin and subcutaneous tissue disorders: Dry skin; hyperhidrosis; pemphigus foliaceus; pruritus; rash; rash pruritic; skin irritation; skin texture abnormal; skin wrinkling; urticaria

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            Warnings

            Contraindications

            Hypersensitivity

            Cautions

            Hypersensitivity reactions reported including drug fever, rash, fever, arthralgia, and lymphadenopathy

            Not recommended if breastfeeding

            Proteinuria

            • Proteinuria reported, including nephrotic syndrome and membranous nephropathy
            • Pediatric patients receiving >50 mg/kg/day may be at increased risk
            • Monitor for development of proteinuria and discontinue therapy in patients who develop proteinuria

            Drug interaction overview

            • Thiola EC: Tiopronin is released faster from Thiola EC in the presence of alcohol and the risk for adverse events associated when taken with alcohol is unknown
            • Avoid alcohol consumption 2 hr before and 3 hr after taking Thiola EC
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            Pregnancy & Lactation

            Pregnancy

            Available published case report data with tiopronin have not identified a drug-associated risk for major birth defects, miscarriage, or adverse maternal or fetal outcomes

            Clinical considerations

            • Renal stones in pregnancy may increase risk of adverse pregnancy outcomes (eg, preterm birth, low birth weight)

            Animal data

            • No findings of fetal malformations could be attributed to the drug in reproduction studies in mice and rats at doses up to 2 times the highest recommended human dose of 2 grams/day (based on mg/m²)

            Lactation

            There are no data on the presence of tiopronin in either human or animal milk or on the effects of the breastfed child

            Prescribing information describes a published study suggesting tiopronin may suppress milk production

            Because of the potential for serious adverse reactions, including nephrotic syndrome, advise patients that breastfeeding is not recommended during treatment

            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.

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            Pharmacology

            Mechanism of Action

            Tiopronin is an active reducing agent which undergoes thiol-disulfide exchange with cystine to form a mixed disulfide of tiopronin-cysteine; from this reaction, a water-soluble mixed disulfide is formed and the amount of sparingly soluble cystine is reduced

            Absorption

            Peak plasma time: 1 hr (immediate-release); 3 hr (delayed-release)

            Elimination

            Excretion: Urine 48% (4 hr); 78% (72 hr)

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            Administration

            Oral Administration

            Thiola: Take at least 1 hr before meals or 2 hr after meals

            Thiola EC

            • May take with or without food
            • Swallow tablet whole; do not chew, crush, or split

            Storage

            Store at 25°C (77°F); excursions permitted to 15-30°C (59-86°F)

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            Formulary

            FormularyPatient Discounts

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            The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

            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.
            3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
            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.
            NC NOT COVERED – Drugs that are not covered by the plan.
            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.
            QL Quantity Limits
            Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
            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.
            OR Other Restrictions
            Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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            Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.