Invasive pneumococcal disease in children can reveal a primary immunodeficiency - PubMed (original) (raw)

. 2014 Jul 15;59(2):244-51.

doi: 10.1093/cid/ciu274. Epub 2014 Apr 23.

Corinne Levy 2, Maya Chrabieh 3, Bertrand Boisson 4, Cécile Bost-Bru 5, Stéphane Dauger 6, François Dubos 7, Philippe Durand 8, Joël Gaudelus 9, Dominique Gendrel 10, Christèle Gras Le Guen 11, Emmanuel Grimprel 12, Gaël Guyon 13, Catherine Jeudy 14, Eric Jeziorski 13, Francis Leclerc 15, Pierre-Louis Léger 16, Fabrice Lesage 17, Mathie Lorrot 18, Isabelle Pellier 14, Didier Pinquier 19, Loïc de Pontual 9, Philippe Sachs 6, Caroline Thomas 20, Pierre Tissières 8, Frédéric V Valla 21, Philippe Desprez 22, Véronique Frémeaux-Bacchi 23, Emmanuelle Varon 24, Xavier Bossuyt 25, Robert Cohen 2, Laurent Abel 26, Jean-Laurent Casanova 27, Anne Puel 3, Capucine Picard 28

Affiliations

Invasive pneumococcal disease in children can reveal a primary immunodeficiency

Jean Gaschignard et al. Clin Infect Dis. 2014.

Abstract

Background: About 10% of pediatric patients with invasive pneumococcal disease (IPD) die from the disease. Some primary immunodeficiencies (PIDs) are known to confer predisposition to IPD. However, a systematic search for these PIDs has never been carried out in children presenting with IPD.

Methods: We prospectively identified pediatric cases of IPD requiring hospitalization between 2005 and 2011 in 28 pediatric wards throughout France. IPD was defined as a positive pneumococcal culture, polymerase chain reaction result, and/or soluble antigen detection at a normally sterile site. The immunological assessment included abdominal ultrasound, whole-blood counts and smears, determinations of plasma immunoglobulin and complement levels, and the evaluation of proinflammatory cytokines.

Results: We included 163 children with IPD (male-to-female ratio, 1.3; median age, 13 months). Seventeen children had recurrent IPD. Meningitis was the most frequent type of infection (87%); other infections included pleuropneumonitis, isolated bloodstream infection, osteomyelitis, endocarditis, and mastoiditis. One patient with recurrent meningitis had a congenital cerebrospinal fluid fistula. The results of immunological explorations were abnormal in 26 children (16%), and a PID was identified in 17 patients (10%), including 1 case of MyD88 deficiency, 3 of complement fraction C2 or C3 deficiencies, 1 of isolated congenital asplenia, and 2 of Bruton disease (X-linked agammaglobulinemia). The proportion of PIDs was much higher in children aged >2 years than in younger children (26% vs 3%; P < .001).

Conclusions: Children with IPD should undergo immunological investigations, particularly those aged >2 years, as PIDs may be discovered in up to 26% of cases.

Keywords: Streptococcus pneumonia; complement deficiency; innate deficiency; primary antibody deficiency; primary immunodeficiency.

© The Author 2014. Published by Oxford University Press on behalf of the Infectious Diseases Society of America. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

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