Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.21.69 (APC)
 16,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report 33 cases of venous thrombosis of the limb, in children aged 15 years or less (average age is 10 years old): 22 acute thrombophlebitis have been treated, 11 children shown post-phlebitic disease. The thrombus was found, most of the time, in the iliac and/or femoral vein. Acute complications were seen in 30% of our cases, and 25% treated children reviewed, had post-phlebitic sequelae. Congenital disease of hemostasis (deficiency of antithrombin III, protein C or S) must be detect before anticoagulant start, because such deficiency influence the treatment and the prognosis. There is non indication for preventive treatment, because of the rarity of spontaneous thrombophlebitis by children. Nevertheless, we can draw an "high risk" population: antecedent of phlebitis, antecedent of congenital disease of hemostasis, antecedent of thrombophlebitis by parents below 40 years old, thrombogenic disease (homocystinuria), vertebral arthrodesis.
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PMID:[Thromboembolic complications in pediatric orthopedics. Multicentric collection of 33 case reports]. 262 Mar 85

Gel filtered human platelets contaminated with less than 0.02% of plasma protein S contained 490 ng of protein S antigen per 3 X 10(8) platelets, equivalent to 2.5% of protein S in whole blood. Three patients with heterozygous plasma protein S deficiency, a congenital disorder associated with venous thrombotic disease, had platelet protein S antigen levels that were 40% of the mean platelet level in ten normal volunteers. In immunoblotting analysis, platelet protein S was indistinguishable from plasma protein S. Thrombin stimulation of platelets caused release of 63% of total protein S antigen and this release was abolished when platelets were preincubated with metabolic inhibitors. Thrombin effected limited proteolysis of platelet protein S and this reaction was inhibited by calcium ions. Immunofluorescent staining of platelets using protein S antibodies demonstrated that protein S colocalized with fibrinogen, an established alpha-granule protein. Thus, human platelets contain protein S in alpha granules that can be released by thrombin stimulation. The released protein S may bind to stimulated platelets and thereby promote and localize the anticoagulant activity of activated protein C on the platelet surface.
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PMID:Identification and quantitation of protein S in human platelets. 293 98

The congenital disorder of glycosylation type Ia (CDG-Ia) presents a broad clinical spectrum. Some patients suffer from acute vascular events (thrombosis and bleeding) and stroke-like events. No correlations have been made between the marked hemostasis abnormalities of CDG-Ia and the occurrence of acute vascular events. We report on 6 patients with CDG-Ia presenting vascular events, then we analyze the clinical and hemostasis data of 39 CDG-Ia patients described in the literature, 17 with vascular events (E) and 21 unscathed from any event (EF), to determine the risk factors for acute vascular events in CDG-Ia. Acute vascular events occurred in patients younger than 15 years, especially with fever and prolonged immobilization. Hemostasis and liver cytolysis were statistically abnormal in patients younger than 5 years whatever the occurrence of vascular events and they normalized with time. Higher factors VIII and IX activities were statistically observed in the E cluster (p=0.03) compared to the EF cluster. The activity/antigenicity ratio for protein C (p=0.02) was also higher in the E group. CDG-Ia patients younger than 15 years old are at risk of acute vascular events. The paradoxical results-abnormal VIII and IX factors in EF patients and normal results in E patients, while XI, antithrombin, protein C, ASAT and ALAT are abnormal in both groups, could suggest a disequilibrium between prothrombotic and antithrombotic factors in the E group. Vascular events may also occur in patients where glycoproteins are proportionally more hypoglycosylated, particularly protein C.
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PMID:Risk assessment of acute vascular events in congenital disorder of glycosylation type Ia. 1809 57