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  • 1
    ISSN: 1432-1238
    Keywords: Infectious purpura ; Purpura fulminans ; Disseminated intravascular coagulation ; Protein C ; Protein S
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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〈0.05), in children with DIC (p〈0.0005). and in non-survivors (p〈0.05). PC activity was significantly decreased in shock children (p〈0.05), in children with DIC (p〈0.0005), and in non-survivors (p〈0.005). Total PS was not decreased in shock children, but was significantly decreased in children with DIC (p〈0.005), and in non-survivors (p〈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.
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  • 2
    ISSN: 1432-1076
    Keywords: Key words Disseminated intra-vascular coagulation ; Limb ischaemia ; Purpura fulminans ; Septic shock ; Skin necrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract More than 10% of children surviving septic shock with purpura have skin necrosis or limb ischaemia (SNLI.). Among 44 children consecutively admitted to our pediatric intensive care unit, 35 (80%) survived, 6 of them (17%) developed SNLI (defined as the need of a surgical procedure). Two timed haemostasis measurements included the determination of coagulation factors, protein C (PC), protein S (PS), C4b binding protein (C4bBP), antithrombin (AT), and plasminogen activator inhibitor 1 (PAI-1). Two severity scores and CRP levels were determined at admission. Children with SNLI and without SNLI were compared. On admission, severity scores, and AT, PC, PS, C4bBP levels were similar in both groups with and without SNLI. Prothrombin time (23% vs 34%; P 〈 0.01), factor VII+X (20% vs 31%; P = 0.05) and factor VII (0% vs 19%; P 〈 0.01) were lower in the group with SNLI. The 2nd sample showed no difference between the two groups. Kinetics of haemostatic abnormalities were no different between the two groups. Conclusion In this series, the only difference between the two groups was lower factor VII levels in children with skin necrosis or limb ischaemia. This suggests the benefit of tissue factor pathway inhibitor administration as an adjunctive therapy to prevent skin necrosis or limb ischaemia. Further studies including more children are needed to determine the potential effects of treatments such as protein C, antithrombin, and plasminogen activator inhibitor antibody administration, and to advocate tissue factor pathway inhibitor in preventing skin necrosis or limb ischaemia.
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  • 3
    ISSN: 1432-0584
    Keywords: FEIBA ; Hemophilia B ; Myocardial infarction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of myocardial infarction (MI) in a hemophilia B patient with a factor IX (FIX) inhibitor (6BU) is described. MI occurred after two infusions of FEIBA concentrate. Unexpectedly, these infusions resulted in a neutralization of the inhibitor and a consistent plasma FIX activity which may have increased the thrombotic risks. Four days later, a psoas hematoma was suspected. At that time the inhibitor remained undetectable, allowing a therapy with purified FIX concentrates. No recurrence of thrombotic complication was observed. This is an additional illustration of the thrombotic risks associated with the use of activated prothrombin complex concentrates, especially in patients having pre-existing risk factors for thrombosis. The management of bleeding episodes in hemophilia B patients with inhibitor represents an especially difficult challenge.
    Type of Medium: Electronic Resource
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