Abstract
Meningococcal disease, with a high prevalence in our country, might course as a fulminant illness with a high mortality (Purpura fulminans). The Waterhouse-Friderichsen syndrome is the association of Purpura fulminans with suprarenal necrosis and haemorrhage. In order to analyze the profile of the patients who died of meningococcal disease in our Unit, twelve patientss autopsies were reviewed through a thirteen-year period. The epidemiological data and clinical characteristics are described. The mean age was 3.2 years. All of them presented Waterhouse- Friderichsen syndrome. On arrival, the clinical presentation was fever less 24 hours and hypotension. Leucopenia was remarkable. All twelve patients received aggressive hemodynamic support from the beginning and haemofiltration was used in four of them. The evolution was fulminant in all of them, with a mean survival of 20 hours. Severity scores were applied showing a high mortality risk: median PRISM (Pediatric Risk of Mortality) score was 32, Niklasson and Wong scores with more than 80% of predicted mortality, and median NESI score was 5. Due to the outcome and poor prognosis of these patients, the use of unconventional therapies of support should be evaluated and multicentric protocols of treatments should be performed.
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Copyright (c) 2001 Revista Chilena de Pediatría