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Invasive Pneumococcal Disease in Children Can Reveal a Primary Immunodeficiency

2014; Oxford University Press; Volume: 59; Issue: 2 Linguagem: Inglês

10.1093/cid/ciu274

1537-6591

Jean Gaschignard, Corinne Lévy, Maya Chrabieh, Bertrand Boisson, C. Bost-Bru, Stéphane Dauger, François Dubos, Philippe Durand, J. Gaudelus, D Gendrel, Christèle Gras‐Le Guen, E. Grimprel, G. Guyon, C. Jeudy, Éric Jeziorski, Francis Leclerc, Pierre-Louis Léger, Fabrice Lesage, Mathie Lorrot, Isabelle Pellier, Didier Pinquier, Loïc de Pontual, P. Sachs, Caroline Thomas, Pierre Tissières, Frédéric V. Valla, P Desprez, Véronique Frémeaux‐Bacchi, Emmanuelle Varon, Xavier Bossuyt, Robert Cohen, Laurent Abel, Jean‐Laurent Casanova, Anne Puel, Capucine Pïcard,

Pneumocystis jirovecii pneumonia detection and treatment

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.