[Complement terminal fraction deficiency revealed at first invasive meningococcal infection]

Arch Pediatr. 2015 Mar;22(3):296-9. doi: 10.1016/j.arcped.2014.11.011. Epub 2014 Dec 19.
[Article in French]

Abstract

Prevalence of complement protein deficiency in the general population is rare and its association with an increased risk of meningococcal infection is well established. However, management of these patients with potentially serious infections and indications warranting a search for such a deficiency have not met with consensus. We report the case of a 3-year-old child with no significant medical history who consulted in an emergency department for a fever after a stay in Senegal. Medical explorations concluded in septicemia and meningococcal W meningitis with a favorable outcome. Secondarily, we highlighted a complete deficiency of complement component C6. We diagnosed the same deficit in his twin sister who presented no infection. A long-term prophylactic antibiotic therapy and a meningococcal conjugate vaccine A,C,Y,W were set up for the twins. Recurrent invasive meningococcal infections and highlighting certain meningococcal serogroups are currently indications for complement protein exploration. We suggest expanding the search criteria for a complement protein deficiency after a single event of invasive meningococcal infection. This is an easy, rapid, and cost-effective screening system by dosage of CH50, C3, C4, and AP50. The arrival of the new meningococcal B vaccine will contribute to improving these patients' care. Family screening is necessary for prophylactic therapy.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Complement C6 / deficiency*
  • Hereditary Complement Deficiency Diseases
  • Humans
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / diagnosis
  • Male
  • Meningitis, Meningococcal / immunology*

Substances

  • Complement C6

Supplementary concepts

  • Complement Component 6 Deficiency