Venxxiva

VENXXIVA is indicated, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation in adults and pediatric patients 9 years of age and older with severe homozygous cystinuria, who are not responsive to these measures alone.

• The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day. (
  2.1 Recommended Dosage

  Adults

  : The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day.

  Pediatrics

  : The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day.  Avoid dosages greater than 50 mg/kg per day in pediatric patients

  [see Warnings and Precautions , Use in Specific Populations ]

  .

  Administer VENXXIVA in 3 divided doses at the same times each day, without food.

  Consider starting VENXXIVA at a lower dosage in patients with history of severe toxicity to d-penicillamine.

  Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.
  )
• The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day.  Avoid dosages greater than 50 mg/kg per day in pediatric patients. (
  5.1 Proteinuria

  Proteinuria, including nephrotic syndrome, and membranous nephropathy, have been reported with tiopronin use. Pediatric patients receiving greater than 50 mg/kg of tiopronin per day may be at increased risk for proteinuria.

  [see Dosage and Administration , Adverse Reactions , Use in Specific Populations ]

  . Monitor patients for the development of proteinuria and discontinue therapy in patients who develop proteinuria

  [see Dosage and Administration ]

  .
  ,
  8.4 Pediatric Use

  VENXXIVA is indicated in pediatric patients 9 years of age and older with severe homozygous cystinuria, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation who are not responsive to these measures alone. This indication is based on safety and efficacy data from a trial in patients 9 years to 68 years of age and clinical experience.

  Proteinuria, including nephrotic syndrome, has been reported in pediatric patients. Pediatric patients receiving greater than 50 mg/kg tiopronin per day may be at greater risk

  [see Dosage and Administration , Warnings and Precautions and Adverse Reactions ]

  .

  VENXXIVA tablets are not approved for use in pediatric patients weighing less than 20 kg or in pediatric patients unable to swallow tablets

  [see Dosage and Administration ]

  .

  Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.
  )
• Measure urinary cystine 1 month after initiation of VENXXIVA and every 3 months thereafter. (
  2.3 Monitoring

  Measure urinary cystine 1 month after starting VENXXIVA and every 3 months thereafter. Adjust VENXXIVA dosage to maintain urinary cystine concentration less than 250 mg/L.

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

  [see Warnings and Precautions ]

  .

  Discontinue VENXXIVA in patients who develop proteinuria, and monitor urinary protein and renal function. Consider restarting VENXXIVA treatment at a lower dosage after resolution of proteinuria.
  )
• Administer VENXXIVA in 3 divided doses at the same times each day, without food. (
  2.1 Recommended Dosage

  Adults

  : The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day.

  Pediatrics

  : The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day.  Avoid dosages greater than 50 mg/kg per day in pediatric patients

  [see Warnings and Precautions , Use in Specific Populations ]

  .

  Administer VENXXIVA in 3 divided doses at the same times each day, without food.

  Consider starting VENXXIVA at a lower dosage in patients with history of severe toxicity to d-penicillamine.

  Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.
  )

Tablets for oral use:

100 mg tablets: White to off-white, round shaped, enteric coated tablets imprinted with "1A" with black ink on one side and plain on other side, free from physical defects.

300 mg tablets: White to off-white, round shaped, enteric coated tablets imprinted with "3A" with black ink on one side and plain on other side, free from physical defects.

• Lactation: Breastfeeding is not recommended. (
  8.2 Lactation

  Risk Summary

  
  
  There are no data on the presence of tiopronin in either human or animal milk or on the effects of the breastfed child. A published study suggests that 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 with VENXXIVA.
  )
• Geriatric: Choose dose carefully and monitor renal function in the elderly. (
  8.5 Geriatric Use

  This drug is known to be substantially excreted by the kidney, and the risk of adverse reactions to this drug may be greater in patients with impaired renal function. 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.
  )

• Proteinuria, including nephrotic syndrome, and membranous
  nephropathy, has been reported with tiopronin use. Pediatric patients
  receiving greater than 50 mg/kg of tiopronin per day may be at increased
  risk for proteinuria. (
  2.1 Recommended Dosage

  Adults

  : The recommended initial dosage in adult patients is 800 mg/day. In clinical studies, the average dosage was about 1,000 mg/day.

  Pediatrics

  : The recommended initial dosage in pediatric patients 9 years of age and older is 15 mg/kg/day.  Avoid dosages greater than 50 mg/kg per day in pediatric patients

  [see Warnings and Precautions , Use in Specific Populations ]

  .

  Administer VENXXIVA in 3 divided doses at the same times each day, without food.

  Consider starting VENXXIVA at a lower dosage in patients with history of severe toxicity to d-penicillamine.

  Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.
  ,
  5.1 Proteinuria

  Proteinuria, including nephrotic syndrome, and membranous nephropathy, have been reported with tiopronin use. Pediatric patients receiving greater than 50 mg/kg of tiopronin per day may be at increased risk for proteinuria.

  [see Dosage and Administration , Adverse Reactions , Use in Specific Populations ]

  . Monitor patients for the development of proteinuria and discontinue therapy in patients who develop proteinuria

  [see Dosage and Administration ]

  .
  ,
  8.4 Pediatric Use

  VENXXIVA is indicated in pediatric patients 9 years of age and older with severe homozygous cystinuria, in combination with high fluid intake, alkali, and diet modification, for the prevention of cystine stone formation who are not responsive to these measures alone. This indication is based on safety and efficacy data from a trial in patients 9 years to 68 years of age and clinical experience.

  Proteinuria, including nephrotic syndrome, has been reported in pediatric patients. Pediatric patients receiving greater than 50 mg/kg tiopronin per day may be at greater risk

  [see Dosage and Administration , Warnings and Precautions and Adverse Reactions ]

  .

  VENXXIVA tablets are not approved for use in pediatric patients weighing less than 20 kg or in pediatric patients unable to swallow tablets

  [see Dosage and Administration ]

  .

  Additional pediatric use information is approved for Mission Pharmacal Company’s Thiola EC (tiopronin delayed-release) tablets. However, due to Mission Pharmacal Company’s marketing exclusivity rights, this drug product is not labeled with that information.
  )
• Hypersensitivity reactions have been reported during tiopronin
  treatment. (
  4 CONTRAINDICATIONS

  VENXXIVA is contraindicated in patients with hypersensitivity to tiopronin or any other components of VENXXIVA

  [see Warnings and Precautions ]

  .

  • Hypersensitivity to tiopronin or any component of VENXXIVA
  ,
  5.2 Hypersensitivity Reactions

  Hypersensitivity reactions (drug fever, rash, fever, arthralgia and lymphadenopathy) have been reported

  [see Contraindications ]

  .
  )