Treatment of purpura fulminans in meningococcemia with protein C concentrate.

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.

Uvulitis and supraglottitis: early manifestations of Kawasaki disease.

Two children with Kawasaki disease initially had upper respiratory tract manifestations. The first was admitted with a diagnosis of uvulitis; in the second the clinical picture was characterized by supraglottic involvement, confirmed by direct laryngoscopic examination.