Treatment of purpura fulminans in meningococcemia with protein C concentrate. 1995

G E Rivard, and M David, and C Farrell, and H P Schwarz
Division of Hematology-Oncology, Hôpital Sainte-Justine, Université de Montréal, Québec, Canada.

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. METHODS Case series (pilot study). METHODS Intensive care unit in a tertiary care pediatric hospital. METHODS Four children (aged 3 months to 15 years) requiring intensive treatment for meningococcemia with shock, disseminated intravascular coagulation, and purpura fulminans. METHODS Intravenous administration of a protein C concentrate (100 IU/kg every 6 hours). METHODS 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.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D008589 Meningococcal Infections Infections with bacteria of the species NEISSERIA MENINGITIDIS. Meningococcal Septicemia,Infections, Meningococcal,Meningococcal Disease,Infection, Meningococcal,Meningococcal Diseases,Meningococcal Infection,Septicemia, Meningococcal
D010865 Pilot Projects Small-scale tests of methods and procedures to be used on a larger scale if the pilot study demonstrates that these methods and procedures can work. Pilot Studies,Pilot Study,Pilot Project,Project, Pilot,Projects, Pilot,Studies, Pilot,Study, Pilot
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D011486 Protein C A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation.
D011693 Purpura Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. When the size of the discolorization is >2-3 cm it is generally called Ecchymoses (ECCHYMOSIS). Petechiae,Purpuras
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004211 Disseminated Intravascular Coagulation A disorder characterized by procoagulant substances entering the general circulation causing a systemic thrombotic process. The activation of the clotting mechanism may arise from any of a number of disorders. A majority of the patients manifest skin lesions, sometimes leading to PURPURA FULMINANS. Consumption Coagulopathy,Coagulation, Disseminated Intravascular,Disseminated Coagulation, Intravascular,Intravascular Coagulation, Disseminated,Intravascular Disseminated Coagulation,Coagulation, Intravascular Disseminated,Coagulations, Disseminated Intravascular,Coagulations, Intravascular Disseminated,Coagulopathies, Consumption,Coagulopathy, Consumption,Consumption Coagulopathies,Disseminated Coagulations, Intravascular,Disseminated Intravascular Coagulations,Intravascular Coagulations, Disseminated,Intravascular Disseminated Coagulations

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