April 17, 2015
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Recurrent invasive pneumococcal disease predicts pediatric primary immune deficiencies

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Recurrent invasive pneumococcal disease episodes are a significant indicator for the presence of primary immune deficiencies in children, according to results of a 29-year Danish study.

“Recurrent invasive pneumococcal disease occurs mostly in children with an underlying disease, but some cases remain unexplained,” Helene Ingels, MD, of the National Neisseria and Streptococcus Reference Center and the department of microbiological surveillance and research at the Statens Serum Institut in Denmark, and colleagues wrote. “Immunodeficiency has been described in [these children] but the prevalence is unknown.”

Previous results from a nationwide study of pediatric invasive pneumococcal disease cases suggest one-third of children studied did not have any obvious clinical features likely to explain recurrent infection. For the follow-up analysis of this cohort, Ingels and colleagues pooled data from the nationwide registry including cases of laboratory-confirmed recurrent invasive pneumococcal disease among children aged 0 to 15 years during 1980 and 2008.

Study participants had no apparent underlying disease and were screened for immunological parameters including complement function, B-cell function and toll-like receptor signaling.

Among 2,192 cases of invasive pneumococcal disease, researchers identified 68 recurrent cases. After the researchers excluded children with classical risk factors for invasive pneumococcal disease, 15 were eligible for analysis. Twelve children were vaccinated with the 23-valent pneumococcal polysaccharide vaccine.

Forty percent of children were complement C2 deficient, and one child had a severe TLR signaling dysfunction. Moreover, six children had an impaired vaccination response, of which three were C2 deficient, according to the researchers.

“Delayed diagnosis of complement deficiency can be associated with increased morbidity and mortality; therefore it is of clinical importance that persons with C2 deficiency and recurrent infections are identified,” Ingels and colleagues wrote. – by Jennifer Southall

Disclosure: The study was partially funded by a grant from the Oda and Hans Svenningsens Foundation, Vanførefonden, Ydes foundation and Statens Serum Institut.