Intensive Care Medicine

, Volume 18, Issue 4, pp 202–205 | Cite as

Protein C and S deficiency in severe infectious purpura of children: A collaborative study of 40 cases

  • F. Leclerc
  • J. Hazelzet
  • B. Jude
  • W. Hofhuis
  • V. Hue
  • A. Martinot
  • E. Van der Voort
Originals

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.

Key words

Infectious purpura Purpura fulminans Disseminated intravascular coagulation Protein C Protein S 

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References

  1. 1.
    Clouse LH, Comp PC (1986) The regulation of hemostasis: the protein C system. N Engl J Med 314:1298–1304Google Scholar
  2. 2.
    Leclerc F (1987) Le purpura fulminans de l'enfant. Arch Fr Pediatr 44:137–144Google Scholar
  3. 3.
    Leclerc F, Beuscart R, Guillois B et al. (1985) Prognostic factors of severe infectious purpura in children. Intensive Care Med 11:140–143Google Scholar
  4. 4.
    Marlar RA, Endres-Brooks J, Miller C (1985) Serial studies of protein C and its plasma inhibitor in patients with disseminated intravascular coagulation. Blood 66:59–63Google Scholar
  5. 5.
    Powars DR, Rogers ZR, Patch MJ, McGehee WG, Francis RB (1987) Purpura fulminans in meningococcemia: association with acquired deficiencies of proteins C and S. N Engl J Med 317:571–572Google Scholar
  6. 6.
    Brandtzaeg P, Sandset PM, Joo GB, Ovstebo R, Abildgaard U, Kierulf P (1989) The quantitative association of plasma endotoxin, antithrombin, protein C, extrinsic pathway inhibitor and fibrinopeptide A in systemic meningococcal disease. Thromb Res 55:459–470Google Scholar
  7. 7.
    Madden RM, Cox Gill J, Marlar RA (1990) Protein C and protein S levels in two patients with acquired purpura fulminans. Br J Haematol 75:112–117Google Scholar
  8. 8.
    Marlar RA, Montgomery RR, Broekmans AW and the working party (1989) Diagnosis and treatment of homozygous protein C deficiency: report of the working party on homozygous protein C deficiency of the subcommittee on protein C and protein S, international committee on thrombosis and haemostasis. J Pediatr 114:528–534Google Scholar
  9. 9.
    Mahasandana C, Suvatte V, Marlar RA, Manco-Johnson MJ, Jacobson LJ, Hathaway WE (1990) Neonatal purpurpa fulminans associated with homozygous protein S deficiency. Lancet 335:61–62Google Scholar
  10. 10.
    Haggerty RJ, Maroney MW, Nadas AS (1956) Essential hypertension in infancy and childhood. Am J Dis Child 92:535–549Google Scholar
  11. 11.
    Nardi M, Karpatkin M (1986) Prothrombin and protein C in early childhood: normal adult levels are not achieved until the fourth year of life. J Pediatr 109:843–845Google Scholar
  12. 12.
    Griffin JH, Mosher DF, Zimmerman TS, Kleiss AJ (1982) Protein C, an antithrombotic protein, is reduced in hospitalized patients with intravascular coagulation. Blood 60:261–264Google Scholar
  13. 13.
    Heeb MJ, Mosher D, Griffin JH (1989) Activation and complexation of protein C and cleavage and decrease of protein S in plasma of patients with intravascular coagulation. Blood 73:455–461Google Scholar
  14. 14.
    Fourrier F, Lestavel P, Chopin C, Marey A, Goudemand J, Rime A, Mangaloboyi J (1990) Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates. Intensive Care Med 16: 121–124Google Scholar
  15. 15.
    Blanco A, Guisasola JA, Solis P, Bachiller R, Gonzalez H (1990) Fibronectin in meningococcal sepssis: correlation with antithrombin III and protein C. Acta Paediatr Scand 79:73–76Google Scholar
  16. 16.
    Ozzoylu S, Cengiz B, Karabent A (1988) Purpura fulminans in a case of protein C deficiency. Eur J Pediatr 147:209–210Google Scholar
  17. 17.
    Taylor FB, Chang A, Esmon T, D'Angelo A, Vigano-D'Angelo S, Blick KE (1987) Protein C prevents the coagulopathic and lethal effects of escherichia coli infusion in the baboon. J Clin Invest 79:918–925Google Scholar
  18. 18.
    Okajima K, Koga S, Kaji M, Inoue M, Nakagakit T, Funatsu A, Okabe H, Takatsuki K, Aoki N (1990) Effect of protein C and activated protein C on coagulation and fibrinolysis in normal human subjects. Thromb Haemost 63:48–53Google Scholar
  19. 19.
    Pralong G, Calandra T, Glauser MP, Schellekens J, Verhoef J, Bachmann F, Kruithof EKO (1989) Plasminogen activator inhibitor 1: a new prognostic marker in septic shock. Thromb Haemost 61:459–462Google Scholar
  20. 20.
    Brandtzaeg P, Joo GB, Brusletto B, Kierulf P (1990) Plasminogen activator inhibitor 1 and 2, alpha-2-antiplasmin, plasminogen and endotoxin levels in systemic meningococcal disease. Thromb Res 57:271–278Google Scholar
  21. 21.
    Vukovich T, Auberger K, Well J, Engelmann H, Knöbl P, Hadora HB (1988) Replacement therapy for a homozygous protein C deficiency-state using a concentrate of human protein C and S. Br J Haematol 70:435–440Google Scholar
  22. 22.
    Madden RM, Oppenheimer C, Wydro R, Marlar MA (1990) Recombinant human protein C: comparative functional studies with human plasma protein C. Thromb Res 57:425–435Google Scholar

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • F. Leclerc
    • 1
    • 2
    • 3
    • 4
  • J. Hazelzet
    • 1
    • 2
    • 3
  • B. Jude
    • 1
    • 2
    • 3
  • W. Hofhuis
    • 1
    • 2
    • 3
  • V. Hue
    • 1
    • 2
    • 3
  • A. Martinot
    • 1
    • 2
    • 3
  • E. Van der Voort
    • 1
    • 2
    • 3
  1. 1.Department of Pediatric Intensive CareHôpital CalmetteLilleFrance
  2. 2.Department of HematologyHôpital CalmetteLilleFrance
  3. 3.Sophia Children's HospitalRotterdamThe Netherlands
  4. 4.Service de Réanimation InfantileHôpital CalmetteLilleFrance

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