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Tiopronin Dosage

Medically reviewed by Drugs.com. Last updated on Mar 10, 2020.

Applies to the following strengths: 100 mg; 300 mg

Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Cystinuria

The dose should be based on that amount required to reduce urinary cystine concentration to below its solubility limit (generally less than 250 mg/L):
-Initial dose: 800 mg orally daily in divided doses 3 times a day
-Maintenance dose: 1000 mg orally daily or less in divided doses 3 times a day

Comments:
-Urinary cystine should be measured at 1 month after beginning therapy and every 3 months thereafter. Adjust the dose depending on the urinary cystine value.
-Before beginning therapy with this drug conservative treatment should be attempted first:
---At least 3 liters of fluid (ten 10-ounce glassfuls), including 2 glasses with each meal and at bedtime.
---Patients should urinate during the night and drink 2 more glasses of fluids before returning to bed.
---Additional fluids should be consumed if there is excessive sweating or intestinal fluid loss.
---A minimum of 2 L/day urine output on a consistent basis is desirable.
---Provide alkali (preferably potassium) to maintain urinary pH at 6.5 to 7. If urinary pH increases above 7, calcium phosphate nephrolithiasis may ensue.
---For patients who continue to form cystine stones on the conservative program, this drug may be added.

Use: For the prevention of cystine (kidney) stone formation in patients with severe homozygous cystinuria with urinary cystine greater than 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.

Usual Pediatric Dose for Cystinuria

The dose should be based on that amount required to reduce urinary cystine concentration to below its solubility limit (generally less than 250 mg/L).

9 to 18 years: 15 mg/kg/day orally in divided doses 3 times a day

Comments:
-Urinary cystine should be measured at 1 month after beginning therapy and every 3 months thereafter. Adjust the dose depending on the urinary cystine value.
-Before beginning therapy with this drug conservative treatment should be attempted first:
---At least 3 liters of fluid (ten 10-ounce glassfuls), including 2 glasses with each meal and at bedtime.
---Patients should urinate during the night and drink 2 more glasses of fluids before returning to bed.
---Additional fluids should be consumed if there is excessive sweating or intestinal fluid loss.
---A minimum of 2 L/day urine output on a consistent basis is desirable.
---Provide alkali (preferably potassium) to maintain urinary pH at 6.5 to 7. If urinary pH increases above 7, calcium phosphate nephrolithiasis may ensue.
---For patients who continue to form cystine stones on the conservative program, this drug may be added.

Use: For the prevention of cystine (kidney) stone formation in patients with severe homozygous cystinuria with urinary cystine greater than 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.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

-In patients who have shown toxicity to d-penicillamine, this drug may be started at a lower dosage.
-The dosage of this drug should be adjusted to reduce urinary cystine concentrations below its solubility limit (generally less than 250 mg/L).

Precautions

CONTRAINDICATIONS:
-Patients with a prior history of developing agranulocytosis, aplastic anemia. or thrombocytopenia on this medication
-Pregnancy (except those with severe cystinuria where the anticipated benefit of inhibited stone formation clearly outweighs possible hazards of therapy)
-Nursing mothers

Safety and efficacy have not been established in patients younger than 9 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-This drug should be given in divided doses 3 times per day, at least 1 hour before or 2 hours after meals.

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

Monitoring:
-Peripheral blood counts
-Direct platelet count
-Hemoglobin
-Serum albumin
-Liver function tests
-Twenty-four-hour urinary protein and routine urinalysis at 3- to 6-month intervals during therapy.
-To assess effect on stone disease, urinary cystine analysis should be monitored frequently during the first 6 months when the optimum dose schedule is being determined, and at 6-month intervals thereafter.
-Abdominal roentogenogram (KUB) is advised on a yearly basis to monitor the size and appearance/disappearance of stone(s).

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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