• A Pettenazzo and T Malusa.
• Terapia Intensiva Pediatrica, Dipartimento di Pediatria, Università degli Studi di Padova, Padova, Italy. a.pettenazzo@unipd.it
• Minerva Anestesiol. 2004 May 1; 70 (5): 357-63.

AbstractThe coagulation disturbance, typical of septic conditions, is associated to a reduction of clotting factors in plasma with an "acquired" deficiency (from consumption) of protein C. As observed with "purpura fulminans" in neonates affected by congenital protein C deficiency, administration of protein C concentrate has proved to reduce thrombotic manifestations and to improve morbidity and mortality of children with septic shock. The Protein C concentrate is presently utilized as a therapy for patients with a congenital deficiency of protein C and several papers in the literature support the efficacy of protein C concentrate in the treatment of children with meningococcus septicemia, with the aim of correcting the acquired protein C deficiency often seen in septic conditions and shown to be strongly correlated to a higher morbidity and mortality. Protein C, given as a plasma concentrate, can exert its therapeutic actions only after activation once in the blood stream: clinical trials with the use of protein C concentrate failed to show any increased risk of bleeding or related disorders. At our PICU 8 children, with sepsis, septic shock and purpura have been treated with protein C concentrate (Ceprotin); because the plasma protein C level was lower than the normal range (mean value 0.32 IU/ml, range 0.11-0.6 IU/ml). Six children have shown a rapid response to all therapeutic efforts and survived without sequelae and two are died. No adverse reaction was observed during and after Ceprotin administration to all patients.

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