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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cystinuria


Other Names for this Disease
  • CSNU
  • Cystinuria - lysinuria
  • Cystinuria-lysinuria
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

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How might cystinuria be treated?

Treatment of cystinuria is focused on relieving symptoms and preventing the formation of additional stones. A more conservative approach is typically tried first. This may include increasing fluid intake, regular monitoring of urinary pH, dietary restrictions (i.e. eating less salt) and increasing the pH of urine with potassium citrate supplements. If these strategies do not prevent the formation of stones, medications may be added to help dissolve the cystine crystals.[1][2][3]

Treatment for cystinuria-related stones varies depending on the size and location of the stone, but may include:[1][2][3]

Medscape Reference's Web site offers more specific information regarding the treatment and management of cystinuria. Please click on the link to access this resource.

Last updated: 5/12/2015

References
  1. Cystinuria. MedlinePlus. October 2013; http://www.nlm.nih.gov/medlineplus/ency/article/000346.htm.
  2. Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU. Cystinuria. Medscape Reference. April 2015; http://emedicine.medscape.com/article/435678-overview.
  3. Saravakos P, Kokkinou V, Giannatos E. Cystinuria: current diagnosis and management. Urology. April 2014; 83(4):693-699.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Cystinuria. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Medical Products

The medication(s) listed in the table(s) below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table(s) below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search for all FDA approved drugs, visit Drugs@FDA. You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.


Generic Name Tiopronin
Trade Name
(Manufacturer Name)
Thiola®
(Retrophin, Inc.)
Indication
The FDA has approved this product to be used in this manner.
Prevention of cystine nephrolithiasis in patients with homozygous cystinuria.
More Information about this product Drug Information Portal

Other Names for this Disease
  • CSNU
  • Cystinuria - lysinuria
  • Cystinuria-lysinuria
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.