title: Protein C and S deficiency in severe infectious purpura of children: a collaborative study of 40 cases.
abstract: We studied, in 40 children (mean age: 52 months) with severe infectious purpura, the relationships between protein C (PC) and protein S (PS) levels, and shock, disseminated intravascular coagulation (DIC) and outcome. We determined, on admission, PC antigen (ELISA) and activity (chromogenic test), and total PS (ELISA). Results were expressed as % of normal adult values. Statistical analysis was performed with SAS. Thirty children were in shock, 20 had DIC. All children with DIC, and 10 without DIC were in shock. Of 20 children who were in shock and had DIC, 7 died and 3 had an amputation. PC antigen was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.05). PC activity was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.005). Total PS was not decreased in shock children, but was significantly decreased in children with DIC (p less than 0.005), and in non-survivors (p less than 0.005). We conclude that PC and PS levels were decreased in our children, and that PC levels were significantly decreased in the presence of shock, DIC, and fatal outcome. PC and antithrombin III (AT III) supplementation, should be evaluated in children with severe infectious purpura with shock and DIC.
authors.affiliation: Department of Pediatric Intensive Care, Hôpital Calmette, Lille, France.
authors.forename: F
authors.initials: F
authors.lastname: Leclerc
authors.forename: J
authors.initials: J
authors.lastname: Hazelzet
authors.forename: B
authors.initials: B
authors.lastname: Jude
authors.forename: W
authors.initials: W
authors.lastname: Hofhuis
authors.forename: V
authors.initials: V
authors.lastname: Hue
authors.forename: A
authors.initials: A
authors.lastname: Martinot
authors.forename: E
authors.initials: E
authors.lastname: Van der Voort
mesh_terms: D000293:Adolescent; D000990:Antithrombin III / Q000032:analysis / Q000627:therapeutic use; D020152:Antithrombin III Deficiency; D001424:Bacterial Infections / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D004211:Disseminated Intravascular Coagulation / Q000097:blood* / Q000150:complications / Q000401:mortality; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D015278:Intensive Care Units, Pediatric; D009426:Netherlands / Q000453:epidemiology; D011379:Prognosis; D011446:Prospective Studies; D011486:Protein C / Q000032:analysis / Q000627:therapeutic use; D020151:Protein C Deficiency; D017293:Protein S / Q000097:blood; D018455:Protein S Deficiency; D011693:Purpura / Q000097:blood* / Q000150:complications; D012772:Shock, Septic / Q000097:blood* / Q000150:complications / Q000401:mortality; D015996:Survival Rate
chemical_list: D011486:Protein C; D017293:Protein S; D000990:Antithrombin III