Treatment of purpura fulminans in meningococcemia with protein C concentrate

• Published in: The Journal of pediatrics 1995-04, Vol.126 (4), p.646-652
• Main Authors: Rivard, Georges E., David, Michèle, Farrell, Catherine, Schwarz, Hans Peter
• Format: Article
• Language: English
• Subjects: Adolescent; Bacteremia - complications; Bacteremia - drug therapy; Bacterial diseases; Biological and medical sciences; Child; Child, Preschool; Disseminated Intravascular Coagulation - drug therapy; Disseminated Intravascular Coagulation - etiology; Drug Administration Schedule; Female; Human bacterial diseases; Humans; Infant; Infectious diseases; Male; Medical sciences; Meningococcal Infections - complications; Meningococcal Infections - drug therapy; Pilot Projects; Prospective Studies; Protein C - administration & dosage; Protein C - therapeutic use; Purpura - drug therapy; Purpura - etiology; Staphylococcal infections, streptococcal infections, pneumococcal infections
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/S0022-3476(95)70369-1

Objective: To evaluate the clinical and laboratory effects of protein C concentrate as an adjunct to conventional therapy in the treatment of meningococcemia with purpura fulminans. Design: Case series (pilot study). Setting: Intensive care unit in a tertiary care pediatric hospital. Patients: Four children (aged 3 months to 15 years) requiring intensive treatment for meningococcemia with shock, disseminated intravascular coagulation, and purpura fulminans. Intervention: Intravenous administration of a protein C concentrate (100 IU/kg every 6 hours). Main outcome measures: Plasma protein C amidolytic activity, fibrinogen, and D-dimers; evolution of skin and limb lesions. Results: Treatment with protein C concentrate led to a rise in plasma protein C activity levels to within normal limits in all patients, associated with an increase in plasma fibrinogen and a bimodal decrease in D-dimers. No adverse effects were noted. All patients had reversal of organ dysfunction despite the severity of the initial illness. Two patients recovered completely with no sequelae; two required amputations. Conclusions: These encouraging clinical and laboratory results and the absence of side effects warrant the initiation of a double-blind, randomized controlled multicenter trial to determine the role of protein C replacement in the treatment of meningococcemia-associated purpura fulminans. ( J PEDIATR 1995; 126:646-52)