Introduction. Some primary immunodeficiencies (PIDs) confer predisposition to invasive pneumococcal disease (IPD).
Methods. Identification of pediatric patients with IPD (January 2000-February 2017). Clinical and epidemiologic data and immunological explorations.
Results.We identified 209 children who suffered from IPD, of whom 78 patients (mean age 34 months; range, 0 days-13 years) required hospitalization. Sixteen of the 78 children (20.5%) had classical risk factors. Immunological evaluation could be performed to 44 patients. Eight patients suffered from a PID: IRAK-4 deficiency (1 patient), X- linked agammaglobulinemia (1), congenital asplenia (2), Ataxia-telangiectasia (1), DiGeorge Syndrome (1), Charge Syndrome (1), and partial Chromosome 16 trisomy with low numbers of switched-memory B cells and hypogammaglobulinemia (1). Only two patients had recurrent IPD, and 6/8 patients did not have a clinical history suggestive of PID. In 6 of the 8 patients with PID no serious infections were recorded after diagnosis.
Conclusions. Around 18% of pediatric cases hospitalized with IPD may be due to a PID. Prompt diagnosis and treatment after one episode of hospitalization for IPD, even in the absence of previous severe and/or recurrent infections, protect against posterior serious infections in patients with PID.