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[Complement terminal fraction deficiency revealed at first invasive meningococcal infection]. / Déficit en fraction terminale du complément révélé dès le premier épisode d'infection invasive à méningocoque.
de Marcellus, C; Taha, M-K; Gaudelus, J; Fremeaux-Bacchi, V; de Pontual, L; Guiddir, T.
Affiliation
  • de Marcellus C; Service de pédiatrie, université Paris 13, Sorbonne Paris Cité, hôpital Jean-Verdier, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France.
  • Taha MK; Centre national de référence des méningocoques, Institut Pasteur, 26-28, rue du Dr-Roux, 75015 Paris, France.
  • Gaudelus J; Service de pédiatrie, université Paris 13, Sorbonne Paris Cité, hôpital Jean-Verdier, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France.
  • Fremeaux-Bacchi V; Laboratoire d'immunologie biologique, université Paris 5, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France.
  • de Pontual L; Service de pédiatrie, université Paris 13, Sorbonne Paris Cité, hôpital Jean-Verdier, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France.
  • Guiddir T; Service de pédiatrie, université Paris 13, Sorbonne Paris Cité, hôpital Jean-Verdier, AP-HP, avenue du 14-Juillet, 93143 Bondy cedex, France. Electronic address: tamazoust.guiddir@jvr.aphp.fr.
Arch Pediatr ; 22(3): 296-9, 2015 Mar.
Article in Fr | MEDLINE | ID: mdl-25534556
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.
Subject(s)

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Complement C6 / Immunologic Deficiency Syndromes / Meningitis, Meningococcal Type of study: Diagnostic_studies / Risk_factors_studies Limits: Child, preschool / Humans / Male Language: Fr Journal: Arch Pediatr Year: 2015 Document type: Article Affiliation country: France

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Complement C6 / Immunologic Deficiency Syndromes / Meningitis, Meningococcal Type of study: Diagnostic_studies / Risk_factors_studies Limits: Child, preschool / Humans / Male Language: Fr Journal: Arch Pediatr Year: 2015 Document type: Article Affiliation country: France