Polymorphisms in the Interleukin-1 Gene Cluster in Children and Young Adults with Systemic Meningococcemia (Technical Briefs‪)‬

Fulminant meningococcemia is a life-threatening disease with acute onset, septic shock, and progressive hemorrhagic necrosis of the skin. The mortality rate is variable, ranging from 5% to 20% (1), and is highest for patients with severe septic shock and the clinical syndrome of purpura fulminans (2). One of the early characteristics of patients with severe sepsis syndrome is the overactivation of proinflammatory cytokines, leading to unusually high plasma concentrations and to symptoms of septic shock and multiorgan failure (3). There is a strong genetic component for sensitivity to infectious disease other than exposure to infectious agents. As of this writing, several polymorphisms in candidate genes have been investigated, including the genes for toll-like receptor 4 (4), tumor necrosis factor-a (5), and plasminogen-activator inhibitor-1 (6). Our knowledge of the genetic factors contributing to the genetic susceptibility, however, is limited.