Protein C deficiency
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| Protein C deficiency Classification and external resources | |
| ICD-9 | 289.81 |
|---|---|
| OMIM | 176860 |
| DiseasesDB | 10807 |
| eMedicine | med/1923 |
| MeSH | D020151 |
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Protein C deficiency is a rare genetic trait that predisposes to thrombotic disease. It was first described in 1981. The disease belongs to a group of genetic disorders know as thrombophilias.
The prevalence of protein C deficiency has been estimated to about 0,2% of the general population.
Protein C deficiency is associated with an increased incidence of venous thromboembolism (relative risk 7), whereas no association with arterial thrombotic disease has been found.
Pathophysiology
The main function of protein C is its anticoagulant property as an inhibitor of coagulation factors V and VIII. There are two main types of protein C mutations that lead to protein C deficiency:
- Type I: Quantitative defects of protein C (low production or short protein halflife)
- Type II: Qualitative defects, in which interaction with other molecules is abnormal. Defects in interaction with thrombomodulin, phospholipids, factors V/VIII and others have been described.
Homozygous protein C mutations often causes a severe thrombotic disorder known as purpura fulminans.proteinc defiency has only had 16 caes before 1999
Treatment
Primary phrophylaxis with aspirin, heparin or warfarin should be considered in known familial cases.
Anticoagulant prophylaxis is given to all who develop a venous clot regardless of underlying cause.
Studies have demonstrated an increased risk of recurrent venours thromboembolic events in patients with protein C deficiency. Therefore, long-term anticoagulation therapy with warfarin should be considered in these patients.
Homozygous protein C defect constitutes a potentially life-threatening disease, and warrants the use of supplemental protein C concentrates.
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| Cost Effectiveness of Protein C deficiency | Cost Effectiveness of Protein C deficiency |
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| Genetics, Pharmacogenomics, and Proteinomics of Protein C deficiency | Genetics of Protein C deficiency • Pharmacogenomics of Protein C deficiency • Proteomics of Protein C deficiency |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
