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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Protein C deficiency

*


* Not a rare disease
Other Names for this Disease
  • Hereditary thrombophilia due to protein C deficiency
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Treatment


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How might protein C deficiency be treated?

Most people with mild protein C deficiency never develop abnormal blood clots and thus do not require treatment. However, individuals that have experienced a deep venous thrombosis (DVT) or a pulmonary embolism are usually treated with blood-thinning drugs such as heparin or warfarin, which help to prevent another blood clot from developing in the future.[1] Preventative treatment with these blood-thinning drugs may also be considered in those with a family history of blood clotting as well as in high risk situations such as pregnancy in the postpartum state, surgery, and trauma.[2]

A protein C concentrate (Ceprotin®) was approved by the Food and Drug Administration in 2007 for the treatment of protein C deficiency. High doses of intravenous protein C concentrates can help thin the blood and protect from blood clots. It can also be used a preventative treatment against blood clots during surgery, pregnancy delivery, prolonged immobility, or overwhelming infection in the blood stream (sepsis). Currently, no guidelines exist as to which patients should receive protein C concentrate. It is typically given only at times of increased risk for clotting, or when the blood thinner heparin by itself cannot be safely given because it would lead to an increased risk for bleeding. However, in those with severe protein C who have had severe bleeding complications on long-term blood thinner therapy, protein C concentrate has been used on a regular basis.[3]
Last updated: 9/20/2011

References
  1. Congenital protein C or S deficiency. MedlinePlus. March 2010; http://www.nlm.nih.gov/medlineplus/ency/article/000559.htm. Accessed 9/20/2011.
  2. Cuker A, Pollak ES. Protein C Deficiency. eMedicine Journal. August 2011; http://emedicine.medscape.com/article/205470-overview. Accessed 9/20/2011.
  3. Protein C Deficiency. Clot Connect. June 2011; http://clotconnect.wordpress.com/2011/06/15/protein-c-deficiency/. Accessed 9/20/2011.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Protein C deficiency. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • Orphanet lists clinical trials, research studies, and patient registries enrolling people with this condition. Click on Orphanet to view the list.

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 Protein C concentrate
Trade Name
(Manufacturer Name)
Ceprotin®
(Baxter Healthcare Corporation)
Indication
The FDA has approved this product to be used in this manner.
Prevention and treatment of venous thrombosis and purpura fulminans
More Information about this product Drug Information Portal