The findings presented in this book will broaden readers’ understanding and hopefully spark new discussions leading to the development of new therapies including cell therapy and molecular target therapy, allowing clinicians to use more effective and [books.google.com]

Protein C Deficiency (2013): A comprehensive discussion of Protein C, including pathophysiology, epidemiology, clinical presentation, diagnosis and treatment. [stoptheclot.org]

Different C4bBP isoforms are present in plasma, but only C4bBP-beta binds protein S. Congenital protein S deficiency is an autosomal dominant disorder that is present in 2% to 6% of patients with venous thrombosis. [mayomedicallaboratories.com]

The present study investigates whether large deletions of PROS1 are found in families where mutations in the PROS1 gene have not been found despite sequencing. [diva-portal.org]

Protein S is also synthesized in endothelial cells and present in platelets. [pediatric.testcatalog.org]

• Sepsis

  Early stage sepsis-associated purpura fulminans may be reversible with quick therapeutic intervention. [flipper.diff.org]

  In one patient sepsis accompanied by disseminated intravascular coagulation appeared to be the trigger of purpura fulminans. [cags.org.ae]

  DIC, Inflammation, Sepsis And Activated Protein C (APC). Transfusion Medicine Update, Issue #3 [On-line information]. Available online at http://www.annals.org/issues/v135n5/full/200109040-00013.html. Chapter 132 Thrombotic Disorders, General. [labtestsonline.it]

  Since a person with protein C deficiency is at increased risk for blood clots during (a) surgery, (b) delivery, (c) prolonged immobility, such as acute illness and hospital admission, or (d) overwhelming infection in the blood stream, called sepsis, intravenous [patientblog.clotconnect.org]

  Crossref, PubMed, Google Scholar 13 Kinasewitz GT, et al. 2004 Universal changes in biomarkers of coagulation and inflammation occur in patients with severe sepsis, regardless of causative micro-organism [ISRCTN74215569]. Crit. [rsob.royalsocietypublishing.org]

• Prolonged Immobilization

  Acute deep-vein thrombosis is a common disorder, with an annual incidence of 1 per 1000 in the general population. 1 Over the past decades, several predisposing factors have been recognized, such as recent surgery, prolonged immobilization, and the presence [nejm.org]

• Vascular Disease

  Maternal Vascular Disease It has long been recognized that many patients with placental abruption also exhibit chronic hypertension or PIH. [glowm.com]

  Due to the versatility exemplified, this probe holds great promise for exploring the role of protein S-nitrosylation in the pathophysiological process of a variety of vascular diseases Title: A fluorogenic probe for imaging protein S-nitrosylation in [genscript.com]

  Bonna also suggested that homocysteine could be a marker, but not a cause, of vascular disease. [thrombosisjournal.biomedcentral.com]

  stroke, and vascular thrombosis. [medtextfree.wordpress.com]

  Other conditions, specifically Meniere's disease and structural and vascular brainstem disease, must be excluded (Black, 2006). [aetna.com]

• Ulcer

  Abstract Background Inflammatory bowel diseases (IBD), ulcerative colitis (UC) and Crohn’s disease (CD) show an increased risk of thrombosis. PROS1 is a well-established anticoagulant. [academic.oup.com]

  We report a case of atypical necrotic leg ulcers induced by VKA. [pubfacts.com]

  Postphlebitic syndrome: chronic complication of thrombosis; pain, swelling, and possibly skin ulceration and induration in the leg. Differential diagnosis Other causes of thrombophilia. [patient.info]

  It includes pain, swelling, ulceration of skin and induration in the lower limbs. Diagnosing Protein S Deficiency Being a silent abnormality, the best and the only method for diagnosing the deficiency of Protein S is by a blood test. [inrtracker.com]

  Most common symptoms of SEVERE HEREDITARY THROMBOPHILIA DUE TO CONGENITAL PROTEIN S DEFICIENCY Retinopathy Abnormality of skin pigmentation Thin skin Skin ulcer Purpura More info about SEVERE HEREDITARY THROMBOPHILIA DUE TO CONGENITAL PROTEIN S DEFICIENCY [mendelian.co]

Thrombotic workup included protein C, protein S, antithrombin (AT), APCR, prothrombin mutation, lupus anticoagulant (LA), and anticardiolipin (ACL). Functional assays were carried out in 179 patients. [cags.org.ae]

Clinical Testing and Workup Doctors will run blood tests that will determine the activity of protein C in the blood. These tests are called assays and they measure the amount and activity of specific enzymes in the blood. [rarediseases.org]

Physicians should also verify that available laboratory workups were appropriately performed and interpreted. [nature.com]

Both the Kleihauer-Betke and CA-125 tests are of little clinical value in the workup of patients with placental abruption. 111, 112 Patients with placenta previa often present with a clinical picture similar to abruption. [glowm.com]

Management and treatment Administration of fresh frozen plasma may be required for the initial treatment of neonatal purpura fulminans. Surgical procedures may be required for excision of thrombotic lesions. [orpha.net]

They usually form after damage to veins or arteries and often resolve without treatment after the wound has healed. The formation of a blood clot is called coagulation. [medicalnewstoday.com]

Standard Therapies Treatment There are no standardized treatment protocols or guidelines for affected individuals. Due to the rarity of the disease, there are no treatment trials that have been tested on a large group of patients. [rarediseases.org]

Medication for anticoagulation is usually part of treatment in order to improve blood flow and reduce thrombosis (clotting). [diseaseinfosearch.org]

Prognosis Prognosis is severe in homozygous or compound heterozygous patients. Prognosis is good for heterozygous patients. With adequate treatment and monitoring, the risk of thromboembolic disease is markedly reduced. [orpha.net]

The prognosis therefore depends on early diagnosis, effective measures to prevent thrombosis and effective management of any thrombosis that does occur. [patient.info]

In case of the acquired Protein S deficiency, the prognosis will depend on how cruel the underlying cause is. [inrtracker.com]

Other tests that may be done include: * Bleeding time * Partial thromboplastin time * Prothrombin time * Thrombin time Prognosis - Protein S deficiency The outcome is usually good with treatment, but symptoms may return. [checkorphan.org]

Etiology Protein S deficiency is caused by mutations in the PROS1 gene (3q11-q11.2). Diagnostic methods Diagnosis is based on the measurement of protein S antigen levels (total protein S or free protein S) and anticoagulant activity. [orpha.net]

The etiology and risk factors that are associated with recurrent abruptions are unknown. [glowm.com]

Etiology of neonatal purpura fulminans Purpura fulminans was first described in a neonate in 1962 and the etiology was presumed an inherited disorder as three siblings had similar skin lesions. [flipper.diff.org]

[…] affected: cardiovascular; hematologic/immunologic; pulmonary Epidemiology Incidence Mean age of first thrombosis: 2nd decade Predominant sex: male = female Prevalence ~0.2% of general population Found in ~1% of persons with venous thrombosis embolism (VTE) Etiology [unboundmedicine.com]

Protein C Deficiency (2013): A comprehensive discussion of Protein C, including pathophysiology, epidemiology, clinical presentation, diagnosis and treatment. [stoptheclot.org]

Summary Epidemiology Prevalence of partial protein S deficiency (heterozygous individuals) is estimated at 0.16-0.21% in the general population. [orpha.net]

[…] recurrent (pro-S deficiency),types I with reduced level of antigen and activity,type II with normal antigen level,reduced activity but excluding most cases of type III Relevant External Links for PROS1 Genetic Association Database (GAD) PROS1 Human Genome Epidemiology [genecards.org]

System(s) affected: cardiovascular; hematologic/immunologic; pulmonary Epidemiology Incidence Mean age of first thrombosis: 2nd decade Predominant sex: male = female Prevalence ~0.2% of general population Found in ~1% of persons with venous thrombosis [unboundmedicine.com]

Nathan and Oski’s is the only comprehensive product on the market that relates pathophysiology in such depth to hematologic and oncologic diseases affecting children. [books.google.com]

Protein C Deficiency (2013): A comprehensive discussion of Protein C, including pathophysiology, epidemiology, clinical presentation, diagnosis and treatment. [stoptheclot.org]

Retrieved 2016-10-16. ^ a b c d e f "Protein S Deficiency: Background, Pathophysiology, Epidemiology". 2016-05-02. ^ "Protein S: Reference Range, Collection and Panels, Interpretation". 2016-06-01. ^ "Congenital protein C or S deficiency: MedlinePlus [en.wikipedia.org]

[…] cardiovascular; hematologic/immunologic; pulmonary Epidemiology Incidence Mean age of first thrombosis: 2nd decade Predominant sex: male = female Prevalence ~0.2% of general population Found in ~1% of persons with venous thrombosis embolism (VTE) Etiology and Pathophysiology [unboundmedicine.com]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Protein S is a vitamin K-dependent plasma glycoprotein and a part of the natural anticoagulant system. [proteinsdeficiency.com]

Prevention If your provider diagnoses you with this disorder, you should be careful to prevent clots from forming. This can occur when the blood moves slowly in the veins, such as from prolonged bed rest during an illness, surgery, or hospital stay. [nicklauschildrens.org]

Prevention - Protein S deficiency If your health care provider diagnoses you with this disorder, you should be careful to prevent clots from forming. [checkorphan.org]

Blood-thinning drugs are used to treat and prevent blood clots. The outcome is usually good with treatment, but symptoms may return, especially if blood-thinning agents are stopped. [medlineplus.gov]

They interfere with the clotting process and can be used to treat or prevent DVT and pulmonary embolism. You may be be prescribed warfarin if you need an anticoagulant to treat a clot and prevent another one occurring. [nhs.uk]

When to see a doctor Staying active may help to prevent blood clots. Every blood clot is a potential medical emergency. [medicalnewstoday.com]