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Topic: Protein S deficiency

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Protein S

Prothrombin mutation

Coagulation

Factor V Leiden

Antithrombin

Antiphospholipid syndrome

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	eMedicine - Protein S Deficiency : Article Excerpt by: John E Godwin, MD, MS
		Protein S deficiency may be hereditary or acquired, the latter usually is due to hepatic diseases or a vitamin K deficiency.
		Protein S deficiency manifests as an autosomal dominant trait; manifestations of thrombosis are observed in both heterozygous and homozygous genetic deficiencies of protein S. Pathophysiology: To understand how thrombosis occurs in protein S deficiency, its physiological function should be briefly reviewed.
		Protein S and APC are sufficient to inactivate FVa.
	www.emedicine.com /med/byname/protein-s-deficiency.htm (667 words)

	Protein S Deficiency
		Protein S deficiency is a genetic trait that predisposes one to the formation of venous clots.
		The function of protein S is to inactivate factor Va and factor VIIIa (the 'a' denotes the active form).
		Activated protein C then combines with protein S on the surface of a platelet (platelets are the clotting cells that circulate in the blood and provide phospholipids to support that clotting process).
	www-admin.med.uiuc.edu /hematology/PtProtS.htm (987 words)

	Ask Dr. Stephan Moll - Protein S deficiency
		Protein S deficiency is an uncommon clotting disorder: approximately 1 per 500 to 1 per 3,000 people has inherited protein S deficiency.
		When protein S deficiency was first described in the 1980's it appeared that it was a strong risk factor for blood clots.
		Protein S deficiency by itself may not be a very strong risk factor for clots.
	www.fvleiden.org /ask/50.html (1709 words)

	BioMed Central \| Full text \| Testicular infarction secondary to protein S deficiency: a case report
		Protein S deficiency is an inherited cause of thrombophilia.
		Protein S deficiencies are associated with superficial and deep vein thrombosis and pulmonary embolism [2].
		Protein S deficiency has been associated with arterial as well as venous occlusion [5,6] and it is now suggested to be an independent risk factor for peripheral arterial insufficiency [6].
	www.biomedcentral.com /1471-2490/6/17 (1404 words)

	protein S deficiency - General Practice Notebook (Site not responding. Last check: 2007-09-16)
		Protein S deficiency results in a primary hypercoagulable state.
		In the normal individual, 60% of protein S in the plasma is inactive, being bound to the C4b-binding protein.
		Excessive binding of protein S to C4b-binding protein may result in a deficiency of active protein S in the plasma.
	www.gpnotebook.co.uk /cache/362414124.htm (126 words)

	Protein S
		Abstract Protein S is a required cofactor for the anticoagulant activity of protein C. A hereditary deficiency of protein S leads to a hypercoagulable state with an increased risk for venous thrombosis.
		Individuals heterozygous for protein S deficiency have an increased risk for venous thrombosis, and the risk is further increased in the presence of a second risk factor.
		It is speculated that this is because protein S is synthesized in endothelial cells and megakaryocytes in addition to the liver, whereas protein C and antithrombin are synthesized predominantly or exclusively in the liver.
	www.massgeneral.org /pathology/coagbook/co004300.htm (1890 words)

	Protein S deficiency - MayoClinic.com (Site not responding. Last check: 2007-09-16)
		Protein S is an anti-clotting factor in the blood.
		A deficiency of protein S may increase the risk of abnormal blood clots, such as in the lung (pulmonary embolism) or leg (deep vein thrombosis).
		Protein S deficiency is a rare, usually inherited disorder.
	www.mayoclinic.com /health/protein-s-deficiency/AN01124 (223 words)

	Protein S Deficiency
		Protein S is a vitamin K dependent co-factor of protein C. Deficiency of Protein S results in a hypercoagulable state, which is treated with anticoagulation.
		Protein S is a vitamin K dependent co-factor of protein C. Protein C acts by neutralizing activated factors V and VIII, and by exhibiting an inhibitory action of plasminogen activator.
		The frequency of venous thrombo-embolism from protein C and S deficiency ranges from 7 to 17%.
	www.ispub.com /ostia/index.php?xmlFilePath=journals/ija/vol11n1/protein.xml (1146 words)

	Protein C and Protein S: The Test (Site not responding. Last check: 2007-09-16)
		Protein C and Protein S tests are requested when you have had a thrombotic episode, especially when you are relatively young (less than 50 years old) and/or do not have any other obvious reasons for developing a blood clot.
		Protein C and Protein S tests should not, however, be requested for at least 10 days after the episode and they should not be used while you are on anticoagulant therapy.
		Protein C and Protein S concentrations (quantity) may be measured with, or after, function/activity levels to determine whether a sufficient amount of each is being produced, to determine the severity of any deficiencies, and to classify the type of deficiency.
	www.labtestsonline.org.uk /understanding/analytes/protein_c_and_s/test.html (907 words)

	UMDNJ-NJMS Ophthalmology: Case of the Month
		Protein C and protein S deficiency and activated protein C resistance have been associated with retinal vascular occlusions and other thromboembolic phenomena.
		Inherited deficiencies in antithrombin, protein C, or protein S are the most commonly reported causative hematological factors in patients with retinal vascular occlusions.
		Protein S is a cofactor to APC in factor Va degradation, whereas factor VIIIa degradation is potentiated by the synergistic APC-cofactor activity of protein S and factor V. The prevalence of
	www.umdnj.edu /eyeweb/casemon/case1099/proteins.html (350 words)

	Protein S - Wikipedia, the free encyclopedia
		Protein S is a vitamin K-dependent plasma glycoprotein synthesized in the liver.
		The best characterized function of Protein S is its role in the anti coagulation pathway, it functions as a cofactor to Protein C in the inactivation of Factors Va and VIIIa.
		Protein S deficiency is a rare blood disorder which can lead to an increased risk of thrombosis.
	en.wikipedia.org /wiki/Protein_S (274 words)

	Thrombotic Disorders: Hematology and Oncology: Merck Manual Professional
		Protein S is a cofactor for APC-mediated cleavage of factors Va and VIIIa.
		Heterozygous deficiency of plasma protein S predisposes to venous thrombosis and is similar to protein C deficiency in genetic transmission, prevalence, laboratory testing, treatment, and precautions.
		Homozygous deficiency of protein S can cause neonatal purpura fulminans that is clinically indistinguishable from that caused by homozygous deficiency of protein C. Acquired deficiencies of protein S (and protein C) occur during DIC and warfarin therapy and after l-asparaginase administration.
	www.merck.com /mmpe/print/sec11/ch135/ch135a.html (1298 words)

	eMedicine - Protein S Deficiency : Article by John E Godwin, MD, MS
		Protein S is a single-chain glycoprotein, and it is dependent on vitamin K action for posttranslational modification of the protein to a normal functional state.
		The coexistence of type I deficiency and type III deficiency in families with the same protein S mutation has been shown at least in one family to be due to an age-related increase in total protein S level.
		Functional protein S: Assays for functional protein S are indirect and are based on prolongation of blood clotting by the generation of APC and its function in the assay.
	www.emedicine.com /med/topic1924.htm (6104 words)

	Protein C deficiency - Wikipedia, the free encyclopedia
		Protein C deficiency is a rare genetic trait that predisposes to thrombotic disease.
		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.
		The main function of protein C is its anticoagulant property as an inhibitor of coagulation factors V and VIII.
	en.wikipedia.org /wiki/Protein_C_deficiency (304 words)

	Varicella Purpura Fulminans Associated With Heterozygosity for Factor V Leiden and Transient Protein S Deficiency -- ...
		The mechanism of transient protein S activity associated with varicella infection appears to be the induction of anti-protein
		Dominey A, Kettler A, Yiannias J, Tschen JA Purpura fulminans and transient protein C and S deficiency.
		Dahlback B Factor V and protein S as cofactors to activated protein C. Haematologica.
	pediatrics.aappublications.org /cgi/content/full/102/5/1208 (1709 words)

	Protein S Deficiency Profile
		Protein S (PS) is produced by the liver, megakaryocytes, and endothelial cells.
		A deficiency in PS, either congenital or acquired, increases the risk of thromboembolism because of a decrease in the anticoagulant capacity of the blood.
		Congenital protein S deficiency: The prevalence of congenital PS deficiency in the general population experiencing their first venous thrombosis is approximately 1%.
	www.labcorp.com /datasets/labcorp/html/chapter/mono/cf004700.htm (1254 words)

	Trifunctional Protein Deficiency
		Because the diagnosis and therapy of TFP Deficiency is complex, the pediatrician is advised to manage the patient in close collaboration with a consulting pediatric metabolic disease specialist.
		Mild Trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation.
		Molecular characterization of mitochondrial Trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits.
	www.pediatrix.com /body_screening_menu.cfm?id=1563 (686 words)

	About thrombosis: thrombophilia: congenital thrombophilia: Protein S deficiency
		Protein S is one of the natural anticoagulants found in the blood.
		Individuals with Protein S deficiency should also seek medical advice before major surgery, or when they are likely to be immobile for a long period, as this increases the risk of venous thrombosis.
		Testing is recommended for close blood relatives of people with a Protein S deficiency, including brothers and sisters and sons and daughters, as there is a 50:50 chance that they will inherit the condition.
	www.thrombosis-charity.org.uk /aboutthrom_thrombophilia_congenital_proteinS.htm (550 words)

	Protein S deficiency at The Medical Dictionary (Site not responding. Last check: 2007-09-16)
		Serum protein electrophoresis is used to identify patients with multiple myeloma and other serum protein disorders.
		Vitamin K deficiency exists when chronic failure to eat sufficient amounts of vitamin K results in a tendency for spontaneous bleeding or in prolonged and excessive bleeding with trauma or injury.
		Vegetarians should not be overconcerned about the amount of protein they eat as protein deficiency is an uncommon problem in any developed country.
	the-medical-dictionary.com /protein_s_deficiency.htm (570 words)

	Protein S Deficiency Research Survey (PSD1) (Site not responding. Last check: 2007-09-16)
		Our survey asked people with Protein S Deficiency to give basic details of their health, lifestyle and experiences dealing with this rare blood disorder which is a recognised risk factor for venous thrombosis.
		Protein S Deficiency is a rare condition that can lead to deep vein thrombosis, pulmonary embolism or stroke and is often treated with long-term anti-coagulant therapy.
		The feasibility of a screening programme for hereditary PSD might be played down because modern treatment techniques for thrombosis have significantly reduced the mortality rate but this doesn't take into account the avoidable loss of quality of life and non-recoverable aspects such as Post Thrombotic Syndrome.
	www.protein.org.uk /psd1report.php (2074 words)

	Protein S Information on Healthline
		Protein C and protein S are decreased by some anticoagulants (Coumadin or warfarin).
		Measurements of protein C or S may be difficult to interpret in patients taking anticoagulants.
		During a clot-related event like a pulmonary embolism, proteins C and S are reduced anyway, and their measurements may be misleading until the episode is resolved.
	www.healthline.com /adamcontent/protein-s (460 words)

	Mitochondrial trifunctional protein deficiency - Genetics Home Reference
		Mitochondrial trifunctional protein deficiency is a rare condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting).
		Mitochondrial trifunctional protein deficiency is a rare disorder; its incidence is unknown.
		Mutations in the HADHA and HADHB genes cause mitochondrial trifunctional protein deficiency.
	ghr.nlm.nih.gov /condition=mitochondrialtrifunctionalproteindeficiency (754 words)

	Clarification of the Risk for Venous Thrombosis Associated with Hereditary Protein S Deficiency by Investigation of a ...
		with a characterized protein S gene mutation that causes a Gly295
		that encodes protein S [19-21] and by the presence of a pseudogene.
		Val mutation was the cause of protein S deficiency
	www.annals.org /cgi/content/full/128/1/8 (4328 words)

	AEGiS-07IAC: Acquired protein S deficiency: correlation with advanced disease in HIV-infected patients.
		OBJECTIVES: To evaluate the interest of measuring protein S (a vitamin K-dependent plasma protein which is involved in the degradation of clotting factors Va and VIIIa) in patients with HIV-1 infection and to assess the prevalence of free protein S deficiency in this population.
		Plasma protein S values were compared to a matched control group of 24 healthy subjects.
		CONCLUSION: Free protein S deficiency may be an index of severity in HIV-1 infection associated with the development of AIDS.
	www.aegis.org /conferences/iac/1991/MB2322.html (413 words)

	Protein S Deficiency Presenting as an Acute Postoperative Arterial Thrombosis in a Four-Year-Old Child -- Zimmerman et ...
		Hereditary protein S deficiency is inherited as an autosomal
		Protein S deficiency is inherited as an autosomal dominant trait.
		Regulation of activated protein C by protein S: the role of phospholipid in factor Va inactivation.
	www.anesthesia-analgesia.org /cgi/content/full/88/3/535 (1825 words)

	Identification of Two Novel Point Mutations in the Human Protein S Gene Associated With Familial Protein S Deficiency ... (Site not responding. Last check: 2007-09-16)
		Gandrille S, Borgel D, Eschwege-Gufflet V, Aillaud MF, Dreyfus M, Matheron C, Gaussem P, Abgrall JF, Jude B, Sie P, Toulon P, Aiach M. Identification of 15 different candidate causal point mutations and three polymorphisms in 19 patients with protein S deficiency using a scanning method for the analysis of the protein S active gene.
		Another protein S functional assay is sensitive to resistance to activated protein C. Thromb Haemost.
		Binding of protein S to C4b-binding protein: mutagenesis of protein S. J Biol Chem.
	atvb.ahajournals.org /cgi/content/full/16/12/1407 (4394 words)

	Association of Protein S Deficiency with Thrombosis in a Kindred with Increased Levels of Plasminogen Activator ...
		Functional protein S activity and free protein S antigen levels measured by enzyme-linked immunosorbent assay in 45 healthy volunteers (23 men and 22 women) and in 14 patients unrelated to this pedigree with protein S deficiency (10 men and 4 women).
		The conversion factor for protein S to SI units is 0.01.
		the concentration of protein S is 346 nmol/L [22].
	www.annals.org /cgi/content/full/119/8/779 (3959 words)

	Hypercoagulability: Too Many Tests, Too Much Conflicting Data -- Bauer et al. 2002 (1): 353 -- Hematology
		S were the first genetic causes of venous thrombosis to be discovered.
		Homozygous protein S deficiency in an infant with purpura fulminans.
		Lindmarker P, Schulman S, Sten-Linder M, Wiman B, Egberg N, Johnson H. The risk of recurrent venous thromboembolism in carriers and non-carriers of the G1691A allele in the coagulation factor V gene and the G20210A allele in the prothrombin gene.
	www.asheducationbook.org /cgi/content/full/2002/1/353 (8763 words)

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