Protein S deficiency
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
| Protein S deficiency Classification and external resources | |
| ICD-9 | 289.81 |
|---|---|
| OMIM | 176880 |
| DiseasesDB | 10814 |
| eMedicine | med/1924 |
| MeSH | D018455 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-525-6884
Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Protein S deficiency is a disorder associated with increased risk of venous thrombosis. Protein S, a vitamin K-dependent physiological anticoagulant, acts as a nonenzymatic cofactor to activated protein C in the proteolytic degradation of factor Va and factor VIIIa. Decreased (antigen) levels or impaired function (activity) of protein S, leads to decreased degradation of factor Va and factor VIIIa and an increased propensity to venous thrombosis. Protein S circulates in human plasma in two forms: approximately 60% is bound to complement component C4b β-chain while the remaining 40% is free. Only free protein S has activated protein C cofactor activity.
Types
There are three types of hereditary protein S deficiency:
- Type I - decreased protein S activity: decreased total protein S (=both bound and free protein S) levels AND decreased free protein S levels (quantitative defect)
- Type II - decreased protein S activity: normal free protein S levels AND normal total protein S levels (qualitative defect)
- Type III - decreased protein S activity: decreased free protein S levels AND normal total protein S levels (quantitative defect)
Protein S deficiency can also be acquired due to vitamin K deficiency or treatment with warfarin, systemic sex hormone therapy and pregnancy, liver disease, and certain chronic infections (for example HIV). Vitamin K deficiency or treatment with warfarin generally also impairs the coagulation system itself (factors II, VII, IX and X), and therefore predisposes to bleeding rather than thrombosis. Protein S deficiency is the underlying cause of a small proportion of cases of disseminated intravascular coagulation (DIC), deep venous thrombosis (DVT) and pulmonary embolism (PE).
Hereditary PSD is an autosomal dominant condition, resulting in a 50 percent chance of passing the disease to offspring. Less than half of those diagnosed with PSD will experience thrombosis, and those who do usually are affected only from the age of the late teens onwards. A positive blood test can lead to the loss of health insurance benefits and/or employment, and the social downsides need to be balanced against the actual medical benefit of accurate diagnosis. Screening of young children is usually deferred because early testing is often inaccurate, and it is better to wait until they are old enough to decide for themselves whether they want to be tested.
External links
- Protein S Deficiency And Thrombophilia at protein.org.uk
- Protein S Deficiency -- University of Illinois
Articles
ten Kate M, Mulder R, Platteel M, Brouwer J, van der Steege G, van der Meer J (2006). "Identification of a novel PROS1 c.1113T-->GG frameshift mutation in a family with mixed type I/type III protein S deficiency". Haematologica 91 (8): 1151-2. PMID 16885060.
WikiDoc Research Resources for Protein S deficiency | |
|---|---|
| Articles on Protein S deficiency | Most recent articles on Protein S deficiency • Most cited articles on Protein S deficiency • Review articles on Protein S deficiency • Articles on Protein S deficiency in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Protein S deficiency | Powerpoint slides on Protein S deficiency • Images of Protein S deficiency • Photos of Protein S deficiency • Podcasts & MP3s on Protein S deficiency • Videos on Protein S deficiency |
| Evidence Based Medicine Regarding Protein S deficiency | Cochrane Collaboration on Protein S deficiency • Bandolier on Protein S deficiency • TRIP on Protein S deficiency |
| Cost Effectiveness of Protein S deficiency | Cost Effectiveness of Protein S deficiency |
| Clinical Trials Involving Protein S deficiency | Ongoing Trials on Protein S deficiency at Clinical Trials.gov • Trial results on Protein S deficiency • Clinical Trials on Protein S deficiency at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Protein S deficiency | US National Guidelines Clearinghouse on Protein S deficiency • NICE Guidance on Protein S deficiency • NHS PRODIGY Guidance • FDA on Protein S deficiency • CDC on Protein S deficiency |
| Textbook Information on Protein S deficiency | Books and Textbook Information on Protein S deficiency |
| Pharmacology Resources on Protein S deficiency | Dosing of Protein S deficiency • Drug interactions with Protein S deficiency • Side effects of Protein S deficiency • Allergic reactions to Protein S deficiency • Overdose information on Protein S deficiency • Carcinogenicity information on Protein S deficiency • Protein S deficiency in pregnancy • Pharmacokinetics of Protein S deficiency • |
| Genetics, Pharmacogenomics, and Proteinomics of Protein S deficiency | Genetics of Protein S deficiency • Pharmacogenomics of Protein S deficiency • Proteomics of Protein S deficiency |
| Newstories on Protein S deficiency | Protein S deficiency in the news • Be alerted to news on Protein S deficiency • News trends on Protein S deficiency |
| Commentary on Protein S deficiency | Blogs on Protein S deficiency |
| Patient Resources on Protein S deficiency | Patient resources on Protein S deficiency • Discussion groups on Protein S deficiency • Patient Handouts on Protein S deficiency • Directions to Hospitals Treating Protein S deficiency • Risk calculators and risk factors for Protein S deficiency |
| Healthcare Provider Resources on Protein S deficiency | Symptoms of Protein S deficiency • Causes & Risk Factors for Protein S deficiency • Diagnostic studies for Protein S deficiency • Treatment of Protein S deficiency |
| Continuing Medical Education (CME) Programs on Protein S deficiency | CME Programs on Protein S deficiency |
| International Resources on Protein S deficiency | Protein S deficiency en Espanol • Protein S deficiency en Francais |
| Business Resources on Protein S deficiency | Protein S deficiency in the Marketplace • Patents on Protein S deficiency |
| Informatics Resources on Protein S deficiency | List of terms related to Protein S deficiency |
| ||||
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 .
