title: Protein S activity in patients with heredofamilial protein S deficiency and in patients with juvenile venous thrombosis. Results of a functional method.
abstract: Using an ACL 300R coagulometer (Instrumentation Laboratory) we assessed the clinical usefulness of a new method to measure PS activity (PS:Act), based on the prolongation of prothrombin time of a mixture of diluted plasma sample, PS depleted plasma previously incubated with Protac for protein C activation, bovine thromboplastin and calcium ions. The results were compared with those from immunological assays. PS:Act was measured in 42 apparently healthy subjects, in 12 patients with hereditary PS deficiency (HPSD group) diagnosed on the basis of immunologic tests and in 48 patients with episodes of juvenile venous thromboembolism at least three months prior to testing (JVTE group). All the HPSD patients had PS:Act below the normal range (less than 62%). In JVTE group 9 patients (18.7%) showed abnormal results for PS:Act, 4 (8.3%) had low levels of free PS:Ag; all patients had normal total PS:Ag levels. Levels of antiphospholipid antibodies (immunologic test) were normal in the 9 JVTE patients with low PS:Act. When all the results were considered together (n = 102), the correlation coefficient between PS:Act and free PS:Ag was 0.78 (p less than 0.01).
authors.affiliation: Department of Angiology and Blood Coagulation Disease, University Hospital S. Orsola, Bologna, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Maccaferri
authors.forename: C
authors.initials: C
authors.lastname: Legnani
authors.forename: L
authors.initials: L
authors.lastname: Preda
authors.forename: G
authors.initials: G
authors.lastname: Palareti
mesh_terms: D000293:Adolescent; D000328:Adult; D001774:Blood Chemical Analysis / Q000379:methods; D001798:Blood Proteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D006023:Glycoproteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017293:Protein S; D011517:Prothrombin Time; D012016:Reference Values; D015203:Reproducibility of Results; D013924:Thrombophlebitis / Q000097:blood*
chemical_list: D001798:Blood Proteins; D006023:Glycoproteins; D017293:Protein S